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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (6)transporters (1) Show 7 proteins XML

enzymes (6)transporters (1) Show 7 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2025-05-29 18:10:01 UTC

HMDB ID

HMDB0000172

Secondary Accession Numbers

HMDB00172

Metabolite Identification

Common Name

Isoleucine

Description

Isoleucine (Ile) or L-isoleucine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid. L-isolecuine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Isoleucine is found in all organisms ranging from bacteria to plants to animals. It is classified as a non-polar, uncharged (at physiological pH) aliphatic amino acid. Isoleucine is an essential amino acid in humans, meaning the body cannot synthesize it and that it must be obtained from the diet. In plants and microorganisms, isoleucine is synthesized starting from pyruvate and alpha-ketobutyrate. Isoleucine is classified as a branched chain amino acid (BCAA). BCAAs include three amino acids: isoleucine, leucine and valine. They are alpha amino acids whose carbon structure is marked by a beta branch point. Despite their structural similarities, BCAAs have different metabolic routes, with valine going solely to carbohydrates (glucogenic), leucine solely to fats (ketogenic) and isoleucine being both a glucogenic and a ketogenic amino acid. Isoleucine is catabolized via with alpha-ketoglutarate where upon it is oxidized and split into propionyl-CoA and acetyl-CoA. Propionyl-CoA is converted into succinyl-CoA, a TCA cycle intermediate which can be converted into oxaloacetate for gluconeogenesis (hence glucogenic). The acetyl-CoA can be fed into the TCA cycle by condensing with oxaloacetate to form citrate or used in the synthesis of ketone bodies or fatty acids. The different metabolism of BCAAs accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine are required respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAAs are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia. An inability to break down isoleucine, along with other amino acids, is associated with maple syrup urine disease (MSUD) (PMID: 34125801 ). Isoleucine, like other BCAAs, is associated with insulin resistance. In particular, higher levels of isoleucine are observed in the blood of diabetic mice, rats, and humans (PMID 25287287 ). Mice fed an isoleucine deprivation diet for one day have improved insulin sensitivity, and feeding of an isoleucine deprivation diet for one week significantly decreases blood glucose levels (PMID: 24684822 ).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(2S,3S)-2-Amino-3-methylpentanoic acid	ChEBI
2-Amino-3-methylvaleric acid	ChEBI
alpha-Amino-beta-methylvaleric acid	ChEBI
I	ChEBI
Ile	ChEBI
ISOLEUCINE	ChEBI
(2S,3S)-2-Amino-3-methylpentanoate	Generator
2-Amino-3-methylvalerate	Generator
a-Amino-b-methylvalerate	Generator
a-Amino-b-methylvaleric acid	Generator
alpha-Amino-beta-methylvalerate	Generator
Α-amino-β-methylvalerate	Generator
Α-amino-β-methylvaleric acid	Generator
(2S,3S)-2-Amino-3-methyl-pentanoate	HMDB
(2S,3S)-2-Amino-3-methyl-pentanoic acid	HMDB
(2S,3S)-a-Amino-b-methyl-N-valerate	HMDB
(2S,3S)-a-Amino-b-methyl-N-valeric acid	HMDB
(2S,3S)-a-Amino-b-methylvalerate	HMDB
(2S,3S)-a-Amino-b-methylvaleric acid	HMDB
(2S,3S)-Alph-amino-beta-methylvalerate	HMDB
(2S,3S)-Alph-amino-beta-methylvaleric acid	HMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valerate	HMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valeric acid	HMDB
(2S,3S)-alpha-Amino-beta-merthylvalerate	HMDB
(2S,3S)-alpha-Amino-beta-merthylvaleric acid	HMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valerate	HMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valeric acid	HMDB
(2S,3S)-alpha-Amino-beta-methylvalerate	HMDB
(2S,3S)-alpha-Amino-beta-methylvaleric acid	HMDB
(S)-Isoleucine	HMDB
(S,S)-Isoleucine	HMDB
2-Amino-3-methylpentanoate	HMDB
2-Amino-3-methylpentanoic acid	HMDB
2S,3S-Isoleucine	HMDB
Erythro-L-isoleucine	HMDB
Iso-leucine	HMDB
L-(+)-Isoleucine	HMDB
L-Ile	HMDB
[S-(R,r)]-2-amino-3-methylpentanoate	HMDB
[S-(R,r)]-2-amino-3-methylpentanoic acid	HMDB
Isoleucine, L-isomer	HMDB
Alloisoleucine	HMDB
Isoleucine, L isomer	HMDB
L-Isomer isoleucine	HMDB
2S-Amino-3S-methylpentanoate	HMDB
L-Isoleucine	KEGG

Chemical Formula

C₆H₁₃NO₂

Average Molecular Weight

131.1729

Monoisotopic Molecular Weight

131.094628665

IUPAC Name

(2S,3S)-2-amino-3-methylpentanoic acid

Traditional Name

L-isoleucine

CAS Registry Number

73-32-5

SMILES

CC[C@H](C)[C@H](N)C(O)=O

InChI Identifier

InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1

InChI Key

AGPKZVBTJJNPAG-WHFBIAKZSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Isoleucine and derivatives

Alternative Parents

Substituents

Isoleucine or derivatives
Alpha-amino acid
L-alpha-amino acid
Branched fatty acid
Methyl-branched fatty acid
Fatty acid
Fatty acyl
Amino acid
Monocarboxylic acid or derivatives
Carboxylic acid
Organic oxide
Organopnictogen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Carbonyl group
Organic oxygen compound
Amine
Organic nitrogen compound
Hydrocarbon derivative
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

aspartate family amino acid (CHEBI:17191 )
proteinogenic amino acid (CHEBI:17191 )
L-alpha-amino acid (CHEBI:17191 )
isoleucine (CHEBI:17191 )
Common amino acids (C00407 )
Amino fatty acids (LMFA01100047 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	285.5 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	35 mg/mL	Human Metabolome Project
LogP	-1.70	HANSCH,C ET AL. (1995)

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	Astarita_neg	129.0	30932474
[M-H]-	Baker	132.62	30932474
[M-H]-	McLean	129.9	30932474
[M-H]-	MetCCS_test_neg	126.7	30932474
[M+H]+	Baker	134.8	30932474
[M+H]+	McLean	133.0	30932474
[M+H]+	Astarita_pos	128.7	30932474
[M-H]-	Not Available	130.8	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078
[M+H]+	Not Available	136.967	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078

Predicted Molecular Properties

Property	Value	Source
Water Solubility	114 g/L	ALOGPS
logP	-1.7	ALOGPS
logP	-1.5	ChemAxon
logS	-0.06	ALOGPS
pKa (Strongest Acidic)	2.79	ChemAxon
pKa (Strongest Basic)	9.59	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	63.32 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	34.09 m³·mol⁻¹	ChemAxon
Polarizability	14.11 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	130.723	31661259
DarkChem	[M-H]-	127.123	31661259
AllCCS	[M+H]+	132.342	32859911
AllCCS	[M-H]-	129.058	32859911
DeepCCS	[M+H]+	131.217	30932474
DeepCCS	[M-H]-	127.39	30932474
DeepCCS	[M-2H]-	164.848	30932474
DeepCCS	[M+Na]+	140.386	30932474
AllCCS	[M+H]+	132.3	32859911
AllCCS	[M+H-H2O]+	128.2	32859911
AllCCS	[M+NH4]+	136.2	32859911
AllCCS	[M+Na]+	137.3	32859911
AllCCS	[M-H]-	129.1	32859911
AllCCS	[M+Na-2H]-	131.9	32859911
AllCCS	[M+HCOO]-	135.0	32859911

Predicted Retention Times

Underivatized

Chromatographic Method	Retention Time	Reference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.	1.25 minutes	32390414
Predicted by Siyang on May 30, 2022	9.9376 minutes	33406817
Predicted by Siyang using ReTip algorithm on June 8, 2022	6.56 minutes	32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid	317.8 seconds	40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid	692.6 seconds	40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid	326.9 seconds	40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid	74.6 seconds	40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid	181.5 seconds	40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	59.7 seconds	40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid	305.5 seconds	40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid	277.3 seconds	40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)	549.9 seconds	40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid	663.4 seconds	40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid	149.4 seconds	40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid	778.9 seconds	40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	180.2 seconds	40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	238.5 seconds	40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate	490.4 seconds	40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA	393.3 seconds	40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water	252.5 seconds	40023050

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
L-Isoleucine	CC[C@H](C)[C@H](N)C(O)=O	1985.0	Standard polar	33892256
L-Isoleucine	CC[C@H](C)[C@H](N)C(O)=O	1164.3	Standard non polar	33892256
L-Isoleucine	CC[C@H](C)[C@H](N)C(O)=O	1292.7	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
L-Isoleucine,1TMS,isomer #1	CC[C@H](C)[C@H](N)C(=O)O[Si](C)(C)C	1180.4	Semi standard non polar	33892256
L-Isoleucine,1TMS,isomer #2	CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O	1292.1	Semi standard non polar	33892256
L-Isoleucine,2TMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C	1316.6	Semi standard non polar	33892256
L-Isoleucine,2TMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C	1308.4	Standard non polar	33892256
L-Isoleucine,2TMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C	1419.5	Standard polar	33892256
L-Isoleucine,2TMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1480.8	Semi standard non polar	33892256
L-Isoleucine,2TMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1322.9	Standard non polar	33892256
L-Isoleucine,2TMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1511.7	Standard polar	33892256
L-Isoleucine,3TMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1501.3	Semi standard non polar	33892256
L-Isoleucine,3TMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1434.9	Standard non polar	33892256
L-Isoleucine,3TMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1401.2	Standard polar	33892256
L-Isoleucine,1TBDMS,isomer #1	CC[C@H](C)[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C	1396.9	Semi standard non polar	33892256
L-Isoleucine,1TBDMS,isomer #2	CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O	1519.3	Semi standard non polar	33892256
L-Isoleucine,2TBDMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1749.9	Semi standard non polar	33892256
L-Isoleucine,2TBDMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1724.7	Standard non polar	33892256
L-Isoleucine,2TBDMS,isomer #1	CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1722.9	Standard polar	33892256
L-Isoleucine,2TBDMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1878.2	Semi standard non polar	33892256
L-Isoleucine,2TBDMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1758.8	Standard non polar	33892256
L-Isoleucine,2TBDMS,isomer #2	CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1750.4	Standard polar	33892256
L-Isoleucine,3TBDMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2161.4	Semi standard non polar	33892256
L-Isoleucine,3TBDMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2055.1	Standard non polar	33892256
L-Isoleucine,3TBDMS,isomer #1	CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1828.2	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0a4i-0930000000-e78f845bb2a8d4736476	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0a4i-0910000000-de9162d149073d0e2a37	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0a4i-0920000000-599e61f8ccdb6525c7a9	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0a4i-0910000000-742e44c426c0d6c2a9ac	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-05fr-8910000000-dbb33e0f02ac2ca5fedd	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine EI-B (Non-derivatized)	splash10-0a4i-0920000000-37690f426455ac41e2c2	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-0a4i-0930000000-e78f845bb2a8d4736476	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-de9162d149073d0e2a37	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-0a4i-0920000000-599e61f8ccdb6525c7a9	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-742e44c426c0d6c2a9ac	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-QQ (Non-derivatized)	splash10-0udi-2392000000-74cca1eb265b8e3d7f43	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-05fr-8910000000-dbb33e0f02ac2ca5fedd	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-000i-9100000000-27891dff695c5db53796	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-43ec1890f6dbd6aea657	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (Non-derivatized) - 70eV, Positive	splash10-00b9-9100000000-40bc9ef43da5f18be883	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (1 TMS) - 70eV, Positive	splash10-000i-9200000000-ae8694c0f3e4dcc608f9	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isoleucine GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-004r-9000000000-34d4d4cb7042da231eb4	2014-09-20	Not Available	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-000i-9300000000-8a23b3e62231eb65f80f	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-00ko-9000000000-ac7e71578d7e1d0e8ce9	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-052f-9000000000-2586d4a089921dd977ed	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-001i-0900000000-5b11521ff6a631376d2b	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-000i-9000000000-3c352f229e4067fcd489	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-000i-9000000000-8a7ef48fc0c1b6f845c2	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-0002-0920000000-2e11aa1c7a5defc386db	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-001i-0900000000-b63ac9cf06eda4c54a81	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-000i-9000000000-e5b0c8c6b09f541d6dbf	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-000i-9000000000-d89d16d5c2242e44ec63	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-000i-9000000000-5de583142c7bdb381c8f	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOF	splash10-001i-0900000000-8c75bde35c4a4073ee65	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOF	splash10-001i-0900000000-233a9862f616afea2d17	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOF	splash10-001i-1900000000-375f35065b82b13e6d6e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOF	splash10-00dl-9000000000-d0d4a9f90fe2aca74483	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOF	splash10-0006-9000000000-0018f47571feaf232ff8	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOF	splash10-001r-7900000000-278dc67396114be331ba	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOF	splash10-000i-9000000000-e26c042aa6231eeca071	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOF	splash10-014r-9000000000-b6c1752fd3fbccb3d1c5	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOF	splash10-05mo-9000000000-0569c3162621252ed0a8	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOF	splash10-052f-9000000000-3d52b5d56d3fab45276e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isoleucine CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive-QTOF	splash10-001i-0900000000-720554d58264a9cfdb67	2012-08-31	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isoleucine 10V, Positive-QTOF	splash10-0019-9500000000-00268b2694cde281641b	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isoleucine 20V, Positive-QTOF	splash10-000i-9000000000-4014793e17e0290790a4	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isoleucine 40V, Positive-QTOF	splash10-0a4i-9000000000-eb9a97a14461e524e76e	2016-09-12	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Experimental 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, experimental)	2021-10-10	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)	2022-02-11	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Mitochondria

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

All Tissues
Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Valine, leucine and isoleucine degradation	Not Available
Transcription/Translation	SMP00019	Not Available
Beta-Ketothiolase Deficiency		Not Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency		Not Available
Propionic Acidemia		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	62.0 (48.0-76.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	40.0 +/- 20.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	69.0 +/- 13.0 uM	Children (1-13 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	64.0 +/- 13.0 uM	Children (1-13 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	84.0 +/- 18.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	44.0 +/- 6.0 uM	Children (1-13 years old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	81.0 +/- 18.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	56.0 +/- 12.0 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	60.7 +/- 18.6 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	20-90 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	25-87 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	25-100 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	35-100 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21505807	details
Blood	Detected and Quantified	68 +/- 11 uM	Adult (>18 years old)	Male	Normal	2415198	details
Blood	Detected and Quantified	53 +/- 7 uM	Adult (>18 years old)	Female	Normal	2415198	details
Blood	Detected and Quantified	77.5 +/- 15.4 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	40.00-100.0 uM	Adult (>18 years old)	Both	Normal	The Metabolo...	details
Blood	Detected and Quantified	39 +/- 8 uM	Newborn (0-30 days old)	Not Specified	Normal	6422161	details
Blood	Detected and Quantified	76.5 +/- 18.0 uM	Children (1 - 13 years old)	Not Specified	Normal	18088602	details
Blood	Detected and Quantified	64 +/- 13 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	84 +/- 18 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	28-84 uM	Children (1-13 years old)	Both	Normal	5018656	details
Blood	Detected and Quantified	51.1(44.6-60.2) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	92.1 (71.4-116) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	83.1 (72.1-96.5) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	38-94 uM	Adolescent (13-18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	22-107 uM	Children (1-13 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	22-107 uM	Children (1 - 18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	30-108 uM	Adult (>18 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	31-83 uM	Children (1-13 years old)	Not Specified	Normal	22956686	details
Blood	Detected and Quantified	37-39 uM	Infant (0-1 year old)	Not Specified	Normal	24341803	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	73.62 ± 17.62 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	81.73 ± 21.62 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	62.17 ± 15.9 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	72.68 ± 14.03 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	77.95 ± 15.83 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	59.97 ± 12.93 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	73.01 ± 18.87 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	75.89 ± 19.92 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Blood	Detected and Quantified	54.55 ± 11.73 uM	Adult (>18 years old)	Both	Normal	Quantitative comp...	details
Breast Milk	Detected and Quantified	7.9 +/- 3.3 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	3.9 +/- 1.0 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	5.77 (3.30-8.24) uM	Adult (>18 years old)	Both	Normal	12834252	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	13.5 (8.8-18.2) uM	Adult (>18 years old)	Both	Normal	12834252	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	8.4 +/- 2.6 uM	Adult (>18 years old)	Not Specified	Normal	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	8.4 +/- 2.5 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.3 +/- 3.4 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	7.0 +/- 5.0 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	5.5 +/- 0.64 uM	Adult (>18 years old)	Both	Normal	7595563	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	1.2-7.5 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.3-9.3 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.9 +/- 0.6 uM	Newborn (0-30 days old)	Not Specified	Normal	6422161	details
Feces	Detected and Quantified	24.853 +/- 27.140 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected and Quantified	1470 +/- 620 nmol/g wet feces	Adult (>18 years old)	Female	Normal	23997757	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24029555	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	24375085	details
Feces	Detected and Quantified	59209.384 (23912.460-94506.309) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	41395.746 (17686.580-65104.911) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	60810.325 (32222.103-89398.547) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25670064	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	26709303	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Both	Normal	25486321	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	17269711	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27107423	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected and Quantified	1090 +/- 650 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	160 +/- 160 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	3733 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected and Quantified	3644 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected and Quantified	4171 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	details
Saliva	Detected and Quantified	25.53 +/- 17.89 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21190195	details
Saliva	Detected and Quantified	5.4 +/- 5.0 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	9.0 +/- 3.5 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	2.3 +/- 3.4 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	6.0 +/- 5.2 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	2.36 +/- 2.74 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	0.0890 +/- 0.330 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	6.60 +/- 9.47 uM	Adult (>18 years old)	Male	Normal	Sugimoto et al. (...	details
Sweat	Detected and Quantified	40-260 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	40 uM	Adult (40 years old)	Male	Normal	22194922	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected and Quantified	3.75 +/- 2.75 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	1.579 (0.789-2.368) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Not Available	details
Urine	Detected and Quantified	0.94 +/- 0.67 umol/mmol creatinine	Children (1-13 years old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	1.8 +/- 1.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	3.0 +/- 1.5 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	1.64 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	1.41 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	3.05-16.78 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	3.55 +/- 1.85 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	0.9 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	1.3 (0.5-2.7) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	1.3 (0.4-2.6) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22932811	details
Urine	Detected and Quantified	0.0086 - 0.0339 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	3.7308 +/- 1.4276 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	3.93 +/- 3.136 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	0 - 6.11 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 5.09 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 6.11 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 6.11 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 16.62 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 6.11 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 4.07 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 4.07 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0.78-1.4 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	0.71-1.1 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	< 1.0 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	3168222	details
Urine	Detected and Quantified	<1.0 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	3168222	details
Urine	Detected and Quantified	<1.0 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	3168222	details
Urine	Detected and Quantified	35.39 +/- 33.92 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	46.81 +/- 29.40 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	48.16 +/- 29.40 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	0.43-14.07 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	25536744	details
Urine	Detected and Quantified	0.86-16.19 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	25536744	details
Urine	Detected and Quantified	4.82 +/- 4.26 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	25536744	details
Urine	Detected and Quantified	5.68 +/- 4.59 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	25536744	details
Urine	Detected and Quantified	6.62 +/- 4.81 umol/mmol creatinine	Children (0-2 years old)	Female	Normal	25536744	details
Urine	Detected and Quantified	6.83 +/- 4.78 umol/mmol creatinine	Children (0-2 years old)	Male	Normal	25536744	details
Urine	Detected and Quantified	0.29-7.30 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	2.73 +/- 1.52 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	2.93 +/- 1.42 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected and Quantified	<9.73 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<8.82 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<7.013 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<3.85 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<3.17 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<2.49 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected and Quantified	2.35 (1.32-4.29) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	477 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	20156336	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Pancreatic cancer	21505807	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oesophageal cancer	22291914	details
Blood	Detected and Quantified	77 +/- 14 uM	Adult (>18 years old)	Male	Schizophrenia	2415198	details
Blood	Detected and Quantified	64 +/- 15 uM	Adult (>18 years old)	Female	Schizophrenia	2415198	details
Blood	Detected and Quantified	37.6 (9.6) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	46.2 (19.3) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected and Quantified	41.1 +/- 10.7 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	38.6 +/- 16.6 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	43.24 (16.72) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	40.9 (11.7) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	42.7 (38.1-47.3) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	492 uM	Newborn (0-30 days old)	Female	Maple syrup urine disease	6422161	details
Blood	Detected and Quantified	237-794 uM	Newborn (0-30 days old)	Both	Maple syrup urine disease	23430924	details
Blood	Detected and Quantified	99.0 +/- 65.3 uM	Children (1 - 13 years old)	Not Specified	Maple syrup urine disease	18088602	details
Blood	Detected and Quantified	103 uM	Children (1-13 years old)	Female	Hyperlysinemia II or Saccharopinuria	5018656	details
Blood	Detected and Quantified	58.6(49.2-70.0) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	282.0705 uM	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	6688766	details
Blood	Detected and Quantified	10-23 uM	Adolescent (13-18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	10-26 uM	Adult (>18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	9-57 uM	Children (1-13 years old)	Both	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	39.5 (7-51) uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Pancreatic cancer	22613268	details
Blood	Detected and Quantified	40.3 (38.0-42.6) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	44.6 +/- 21.5 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected and Quantified	6100.00 (200.00-12000.00) uM	Children (1-13 years old)	Both	Maple syrup urine disease (MSUD)	MetaGene: Metabol...	details
Blood	Detected and Quantified	1495.2 +/- 373.0 uM	Elderly (>65 years old)	Both	Alzheimer's disease	17031479	details
Blood	Detected and Quantified	166.0 +/- 5.0 uM	Adult (>18 years old)	Both	Heart failure	16789974	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	2.4 +/- 0.65 uM	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	338 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	216 uM	Newborn (0-30 days old)	Female	Maple syrup urine disease	6422161	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.0 +/- 0.65 uM	Adult (>18 years old)	Both	Schizophrenia	7595563	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	5.9 +/- 4.0 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	7.1 +/- 4.2 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Cryptosporidium infection	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Myalgic encephalomyelitis/chronic fatigue syndrome	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative Colitis	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn’s Disease	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable Bowel Syndrome	21494186	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn's disease	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27107423	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohns disease	25598765	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	17269711	details
Saliva	Detected and Quantified	0.83 +/- 0.21 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Missing teeth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral squamous cell carcinoma (OSCC)	21190195	details
Saliva	Detected and Quantified	1.54 +/- 0.98 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Saliva	Detected and Quantified	1.22 +/- 0.30 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral leukoplakia (OLK)	21190195	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Breast cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Pancreatic cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Periodontal diseases	20300169	details
Urine	Detected and Quantified	0.13 +/- 0.04 umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details
Urine	Detected and Quantified	0.0053 - 0.0143 umol/mmol creatinine	Adult (>18 years old)	Both	ADPKD	21389975	details
Urine	Detected and Quantified	4.211 +/- 5.162 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	2.8234 +/- 1.7792 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details
Urine	Detected and Quantified	3.0408 +/- 2.1926 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details

Associated Disorders and Diseases

Disease References

Alzheimer's disease
Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ] Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Heart failure
Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21. [PubMed:16789974 ]
Maple syrup urine disease
Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ] Yunus ZM, Kamaludin DA, Mamat M, Choy YS, Ngu L: Clinical and biochemical profiles of maple syrup urine disease in malaysian children. JIMD Rep. 2012;5:99-107. doi: 10.1007/8904_2011_105. Epub 2011 Dec 11. [PubMed:23430924 ] Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Lipoyltransferase 1 Deficiency
Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Dihydrolipoamide Dehydrogenase Deficiency
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Saccharopinuria
Simell O, Visakorpi JK, Donner M: Saccharopinuria. Arch Dis Child. 1972 Feb;47(251):52-5. [PubMed:5018656 ]
Pregnancy
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ] Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ] Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Early preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Epilepsy
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Colorectal cancer
Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ] Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ] Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ] Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Leukemia
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Schizophrenia
Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ] Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ] Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Ulcerative colitis
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Autism
De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Irritable bowel syndrome
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
Crohn's disease
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Periodontal disease
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ] OuYang D, Xu J, Huang H, Chen Z: Metabolomic profiling of serum from human pancreatic cancer patients using 1H NMR spectroscopy and principal component analysis. Appl Biochem Biotechnol. 2011 Sep;165(1):148-54. doi: 10.1007/s12010-011-9240-0. Epub 2011 Apr 20. [PubMed:21505807 ] Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Perillyl alcohol administration for cancer treatment
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Attachment loss
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Autosomal dominant polycystic kidney disease
Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]

Associated OMIM IDs

104300 (Alzheimer's disease)
248600 (Maple syrup urine disease)
114500 (Colorectal cancer)
268700 (Saccharopinuria)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)
614923 (Branched-chain Keto Acid Dehydrogenase Kinase Deficiency)
616299 (Lipoyltransferase 1 Deficiency)
181500 (Schizophrenia)
266600 (Crohn's disease)
209850 (Autism)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
600274 (Frontotemporal dementia)
601313 (Autosomal dominant polycystic kidney disease)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

DB00167

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

PDB ID

Not Available

ChEBI ID

17191

Food Biomarker Ontology

Not Available

VMH ID

ILE_L

MarkerDB ID

MDB00000083

Good Scents ID

Not Available

References

Synthesis Reference

Marvel, C. S. L-Isoleucine. Organic Syntheses (1941), 21 60-4.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
He XY, Yang SY: Roles of type 10 17beta-hydroxysteroid dehydrogenase in intracrinology and metabolism of isoleucine and fatty acids. Endocr Metab Immune Disord Drug Targets. 2006 Mar;6(1):95-102. [PubMed:16611167 ]
Vaalasti A, Suomalainen H, Kuokkanen K, Rechardt L: Neuropeptides in cutaneous neurofibromas of von Recklinghausen's disease. J Cutan Pathol. 1990 Dec;17(6):371-3. [PubMed:1981573 ]
Eriste E, Norberg A, Bonetto V, Nepomuceno D, Lovenberg TW, Sillard R, Jornvall H: A C-terminally elongated form of PHI from porcine intestine. Cell Mol Life Sci. 1999 Nov 15;56(7-8):709-13. [PubMed:11212317 ]
Jalan R, Olde Damink SW, Lui HF, Glabus M, Deutz NE, Hayes PC, Ebmeier K: Oral amino acid load mimicking hemoglobin results in reduced regional cerebral perfusion and deterioration in memory tests in patients with cirrhosis of the liver. Metab Brain Dis. 2003 Mar;18(1):37-49. [PubMed:12603081 ]
Hervieu G, Segretain D, Nahon JL: Developmental and stage-dependent expression of melanin-concentrating hormone in mammalian germ cells. Biol Reprod. 1996 Jun;54(6):1161-72. [PubMed:8724342 ]
Suk FM, Lin MH, Newman M, Pan S, Chen SH, Liu JD, Shih C: Replication advantage and host factor-independent phenotypes attributable to a common naturally occurring capsid mutation (I97L) in human hepatitis B virus. J Virol. 2002 Dec;76(23):12069-77. [PubMed:12414948 ]
De Miranda J, Panizzutti R, Foltyn VN, Wolosker H: Cofactors of serine racemase that physiologically stimulate the synthesis of the N-methyl-D-aspartate (NMDA) receptor coagonist D-serine. Proc Natl Acad Sci U S A. 2002 Oct 29;99(22):14542-7. Epub 2002 Oct 22. [PubMed:12393813 ]
Blomstrand E, Eliasson J, Karlsson HK, Kohnke R: Branched-chain amino acids activate key enzymes in protein synthesis after physical exercise. J Nutr. 2006 Jan;136(1 Suppl):269S-73S. [PubMed:16365096 ]
Sato T, Shimada Y, Nagasawa N, Nakanishi S, Jingami H: Amino acid mutagenesis of the ligand binding site and the dimer interface of the metabotropic glutamate receptor 1. Identification of crucial residues for setting the activated state. J Biol Chem. 2003 Feb 7;278(6):4314-21. Epub 2002 Nov 19. [PubMed:12444084 ]
Edvinsson L: Innervation and effects of dilatory neuropeptides on cerebral vessels. New aspects. Blood Vessels. 1991;28(1-3):35-45. [PubMed:2001478 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
Allahwala A, Ahmed S, Afroze B: Maple syrup urine disease: magnetic resonance imaging findings in three patients. J Pak Med Assoc. 2021 Apr;71(4):1309-1313. doi: 10.47391/JPMA.1341. [PubMed:34125801 ]
Lynch CJ, Adams SH: Branched-chain amino acids in metabolic signalling and insulin resistance. Nat Rev Endocrinol. 2014 Dec;10(12):723-36. doi: 10.1038/nrendo.2014.171. Epub 2014 Oct 7. [PubMed:25287287 ]
Xiao F, Yu J, Guo Y, Deng J, Li K, Du Y, Chen S, Zhu J, Sheng H, Guo F: Effects of individual branched-chain amino acids deprivation on insulin sensitivity and glucose metabolism in mice. Metabolism. 2014 Jun;63(6):841-50. doi: 10.1016/j.metabol.2014.03.006. Epub 2014 Mar 15. [PubMed:24684822 ]

Enzymes

Enzyme Details

1. Branched-chain-amino-acid aminotransferase, cytosolic

General function:: Involved in catalytic activity
Specific function:: Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:: BCAT1
Uniprot ID:: P54687
Molecular weight:: 38644.77

Reactions

Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic acid	details

References

Goto M, Miyahara I, Hayashi H, Kagamiyama H, Hirotsu K: Crystal structures of branched-chain amino acid aminotransferase complexed with glutamate and glutarate: true reaction intermediate and double substrate recognition of the enzyme. Biochemistry. 2003 Apr 8;42(13):3725-33. [PubMed:12667063 ]
Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. [PubMed:17554170 ]
Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. [PubMed:15667998 ]
Thage BV, Rattray FP, Laustsen MW, Ardo Y, Barkholt V, Houlberg U: Purification and characterization of a branched-chain amino acid aminotransferase from Lactobacillus paracasei subsp. paracasei CHCC 2115. J Appl Microbiol. 2004;96(3):593-602. [PubMed:14962140 ]
Madsen SM, Beck HC, Ravn P, Vrang A, Hansen AM, Israelsen H: Cloning and inactivation of a branched-chain-amino-acid aminotransferase gene from Staphylococcus carnosus and characterization of the enzyme. Appl Environ Microbiol. 2002 Aug;68(8):4007-14. [PubMed:12147502 ]

Enzyme Details

2. Branched-chain-amino-acid aminotransferase, mitochondrial

General function:: Involved in catalytic activity
Specific function:: Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:: BCAT2
Uniprot ID:: O15382
Molecular weight:: 33776.315

Reactions

Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic acid	details

References

Berger BJ, English S, Chan G, Knodel MH: Methionine regeneration and aminotransferases in Bacillus subtilis, Bacillus cereus, and Bacillus anthracis. J Bacteriol. 2003 Apr;185(8):2418-31. [PubMed:12670965 ]

Enzyme Details

3. Isoleucine--tRNA ligase, cytoplasmic

General function:: Involved in nucleotide binding
Specific function:: Not Available
Gene Name:: IARS
Uniprot ID:: P41252
Molecular weight:: 144496.915

Reactions

Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)	details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)	details

References

Crasto CF, Forrest AK, Karoli T, March DR, Mensah L, O'Hanlon PJ, Nairn MR, Oldham MD, Yue W, Banwell MG, Easton CJ: Synthesis and activity of analogues of the isoleucyl tRNA synthetase inhibitor SB-203207. Bioorg Med Chem. 2003 Jul 3;11(13):2687-94. [PubMed:12788342 ]
Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
Mock ML, Michon T, van Hest JC, Tirrell DA: Stereoselective incorporation of an unsaturated isoleucine analogue into a protein expressed in E. coli. Chembiochem. 2006 Jan;7(1):83-7. [PubMed:16397872 ]
Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
Pezo V, Metzgar D, Hendrickson TL, Waas WF, Hazebrouck S, Doring V, Marliere P, Schimmel P, De Crecy-Lagard V: Artificially ambiguous genetic code confers growth yield advantage. Proc Natl Acad Sci U S A. 2004 Jun 8;101(23):8593-7. Epub 2004 May 26. [PubMed:15163798 ]

Enzyme Details

4. Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

General function:: Involved in acyl-CoA dehydrogenase activity
Specific function:: Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Gene Name:: ACADSB
Uniprot ID:: P45954
Molecular weight:: 47485.035

References

Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
Madsen PP, Kibaek M, Roca X, Sachidanandam R, Krainer AR, Christensen E, Steiner RD, Gibson KM, Corydon TJ, Knudsen I, Wanders RJ, Ruiter JP, Gregersen N, Andresen BS: Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping. Hum Genet. 2006 Feb;118(6):680-90. Epub 2005 Nov 30. [PubMed:16317551 ]
Korman SH: Inborn errors of isoleucine degradation: a review. Mol Genet Metab. 2006 Dec;89(4):289-99. Epub 2006 Sep 6. [PubMed:16950638 ]

Enzyme Details

5. L-amino-acid oxidase

General function:: Involved in oxidoreductase activity
Specific function:: Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:: IL4I1
Uniprot ID:: Q96RQ9
Molecular weight:: 65327.26

Reactions

Isoleucine + Water + Oxygen → 3-Methyl-2-oxovaleric acid + Ammonia + Hydrogen peroxide	details

Enzyme Details

6. Isoleucine--tRNA ligase, mitochondrial

General function:: Involved in nucleotide binding
Specific function:: Not Available
Gene Name:: IARS2
Uniprot ID:: Q9NSE4
Molecular weight:: 113790.565

Reactions

Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)	details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)	details

References

Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. [PubMed:17095543 ]
Fukunaga R, Yokoyama S: Crystal structure of leucyl-tRNA synthetase from the archaeon Pyrococcus horikoshii reveals a novel editing domain orientation. J Mol Biol. 2005 Feb 11;346(1):57-71. Epub 2004 Dec 19. [PubMed:15663927 ]
Fukunaga R, Yokoyama S: Structural basis for substrate recognition by the editing domain of isoleucyl-tRNA synthetase. J Mol Biol. 2006 Jun 16;359(4):901-12. Epub 2006 Apr 25. [PubMed:16697013 ]

Transporters

Enzyme Details

1. Monocarboxylate transporter 10

General function:: Involved in transmembrane transport
Specific function:: Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:: SLC16A10
Uniprot ID:: Q8TF71
Molecular weight:: 55492.1

References

Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]

Showing metabocard for Isoleucine (HMDB0000172)

MOL for HMDB0000172 (Isoleucine)

3D MOL for HMDB0000172 (Isoleucine)

3D SDF for HMDB0000172 (Isoleucine)

3D-SDF for HMDB0000172 (Isoleucine)

PDB for HMDB0000172 (Isoleucine)

3D PDB for HMDB0000172 (Isoleucine)

SMILES for HMDB0000172 (Isoleucine)

INCHI for HMDB0000172 (Isoleucine)

Structure for HMDB0000172 (Isoleucine)

3D Structure for HMDB0000172 (Isoleucine)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Retention Times

Underivatized

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

Reactions

References

Reactions

References

Reactions

References

References

Reactions

Reactions

References

Transporters

References