Hmdb loader
Identification
HMDB Protein ID HMDBP08256
Secondary Accession Numbers
  • 13968
Name Protein SCO1 homolog, mitochondrial
Synonyms Not Available
Gene Name SCO1
Protein Type Unknown
Biological Properties
General Function Involved in copper ion binding
Specific Function Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX
Pathways Not Available
Reactions Not Available
GO Classification
Component
mitochondrial inner membrane
organelle inner membrane
organelle membrane
cell part
membrane
Function
cation binding
copper ion binding
ion binding
transition metal ion binding
binding
metal ion binding
Process
respiratory chain complex iv assembly
copper ion transport
cellular protein complex assembly
cellular copper ion homeostasis
macromolecular complex assembly
cellular component assembly
cellular component organization
cellular component organization or biogenesis
cellular di-, tri-valent inorganic cation homeostasis
cellular cation homeostasis
cellular ion homeostasis
ion homeostasis
chemical homeostasis
homeostatic process
transition metal ion transport
metal ion transport
cation transport
ion transport
regulation of biological quality
transport
establishment of localization
protein complex assembly
biological regulation
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location Chromosome:1
Locus 17p13.1
SNPs SCO1
Gene Sequence
>906 bp
ATGGCGATGCTGGTCCTAGTACCCGGACGAGTTATGCGGCCTCTGGGTGGCCAACTTTGG
CGCTTCTTGCCTCGCGGACTCGAGTTTTGGGGCCCAGCCGAGGGGACTGCGAGAGTCTTG
CTGAGGCAGTTCTGCGCGCGGCAAGCGGAGGCGTGGCGTGCCTCGGGGCGCCCTGGCTAT
TGCCTGGGAACCCGGCCCCTCAGCACTGCGAGGCCGCCACCCCCGTGGTCGCAGAAGGGC
CCCGGAGACTCCACGCGCCCCTCGAAGCCCGGGCCTGTTTCCTGGAAGTCTTTAGCAATC
ACATTTGCTATTGGAGGAGCTTTACTGGCTGGAATGAAGCACGTCAAGAAAGAAAAGGCA
GAGAAGTTAGAGAAGGAACGGCAGCGACACATCGGCAAGCCTTTACTTGGGGGACCGTTT
TCCCTCACAACTCATACTGGGGAGCGTAAAACTGACAAGGACTACTTGGGTCAGTGGTTA
TTGATTTATTTTGGCTTCACTCATTGCCCTGATGTCTGTCCAGAAGAACTAGAAAAGATG
ATTCAAGTCGTGGATGAAATAGATAGCATTACAACTCTGCCAGATCTAACTCCACTTTTC
ATCAGCATTGACCCAGAGAGGGACACAAAAGAAGCCATCGCAAATTATGTGAAAGAATTT
TCTCCCAAACTGGTTGGCTTGACTGGCACGAGAGAAGAGGTCGATCAAGTGGCCAGAGCA
TACAGAGTGTATTACAGCCCTGGCCCCAAGGACGAAGATGAAGACTACATAGTGGATCAC
ACAATAATAATGTACTTGATTGGACCAGATGGTGAGTTTCTAGATTATTTTGGCCAGAAC
AAGAGGAAGGGAGAAATAGCTGCTTCAATTGCCACACACATGAGGCCATACAGAAAAAAG
AGCTAG
Protein Properties
Number of Residues 301
Molecular Weight 33813.7
Theoretical pI 9.22
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>Protein SCO1 homolog, mitochondrial
MAMLVLVPGRVMRPLGGQLWRFLPRGLEFWGPAEGTARVLLRQFCARQAEAWRASGRPGY
CLGTRPLSTARPPPPWSQKGPGDSTRPSKPGPVSWKSLAITFAIGGALLAGMKHVKKEKA
EKLEKERQRHIGKPLLGGPFSLTTHTGERKTDKDYLGQWLLIYFGFTHCPDVCPEELEKM
IQVVDEIDSITTLPDLTPLFISIDPERDTKEAIANYVKEFSPKLVGLTGTREEVDQVARA
YRVYYSPGPKDEDEDYIVDHTIIMYLIGPDGEFLDYFGQNKRKGEIAASIATHMRPYRKK
S
GenBank ID Protein 3599966
UniProtKB/Swiss-Prot ID O75880
UniProtKB/Swiss-Prot Entry Name SCO1_HUMAN
PDB IDs
GenBank Gene ID AF026852
GeneCard ID SCO1
GenAtlas ID SCO1
HGNC ID HGNC:10603
References
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  2. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  3. Petruzzella V, Tiranti V, Fernandez P, Ianna P, Carrozzo R, Zeviani M: Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain. Genomics. 1998 Dec 15;54(3):494-504. [PubMed:9878253 ]
  4. Horvath R, Lochmuller H, Stucka R, Yao J, Shoubridge EA, Kim SH, Gerbitz KD, Jaksch M: Characterization of human SCO1 and COX17 genes in mitochondrial cytochrome-c-oxidase deficiency. Biochem Biophys Res Commun. 2000 Sep 24;276(2):530-3. [PubMed:11027508 ]
  5. Leary SC, Cobine PA, Kaufman BA, Guercin GH, Mattman A, Palaty J, Lockitch G, Winge DR, Rustin P, Horvath R, Shoubridge EA: The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis. Cell Metab. 2007 Jan;5(1):9-20. [PubMed:17189203 ]
  6. Williams JC, Sue C, Banting GS, Yang H, Glerum DM, Hendrickson WA, Schon EA: Crystal structure of human SCO1: implications for redox signaling by a mitochondrial cytochrome c oxidase "assembly" protein. J Biol Chem. 2005 Apr 15;280(15):15202-11. Epub 2005 Jan 19. [PubMed:15659396 ]
  7. Banci L, Bertini I, Calderone V, Ciofi-Baffoni S, Mangani S, Martinelli M, Palumaa P, Wang S: A hint for the function of human Sco1 from different structures. Proc Natl Acad Sci U S A. 2006 Jun 6;103(23):8595-600. Epub 2006 May 30. [PubMed:16735468 ]
  8. Shoubridge EA: Cytochrome c oxidase deficiency. Am J Med Genet. 2001 Spring;106(1):46-52. [PubMed:11579424 ]
  9. Valnot I, Osmond S, Gigarel N, Mehaye B, Amiel J, Cormier-Daire V, Munnich A, Bonnefont JP, Rustin P, Rotig A: Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy. Am J Hum Genet. 2000 Nov;67(5):1104-9. Epub 2000 Sep 28. [PubMed:11013136 ]