| Identification |
|---|
| HMDB Protein ID
| HMDBP12570 |
| Secondary Accession Numbers
| None |
| Name
| Ornithine transcarbamylase, mitochondrial |
| Synonyms
|
- OTCase
- Ornithine carbamoyltransferase, mitochondrial
|
| Gene Name
| OTC |
| Protein Type
| Unknown |
| Biological Properties |
|---|
| General Function
| Not Available |
| Specific Function
| Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:2290837). The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2290837). |
| Pathways
|
- Arginine biosynthesis
- Biosynthesis of amino acids
- urea cycle
|
| Reactions
| Not Available |
| GO Classification
|
| Biological Process |
| arginine biosynthetic process via ornithine |
| ornithine catabolic process |
| ammonium homeostasis |
| citrulline biosynthetic process |
| anion homeostasis |
| response to nutrient levels |
| response to biotin |
| response to insulin stimulus |
| urea cycle |
| midgut development |
| ornithine metabolic process |
| response to drug |
| liver development |
| response to zinc ion |
| Cellular Component |
| intracellular membrane-bounded organelle |
| mitochondrial inner membrane |
| mitochondrion |
| cytoplasm |
| mitochondrial matrix |
| Molecular Function |
| amino acid binding |
| phospholipid binding |
| identical protein binding |
| phosphate ion binding |
| ornithine carbamoyltransferase activity |
|
| Cellular Location
|
Not Available
|
| Gene Properties |
|---|
| Chromosome Location
| Not Available |
| Locus
| Not Available |
| SNPs
| Not Available |
| Gene Sequence
|
Not Available
|
| Protein Properties |
|---|
| Number of Residues
| 354 |
| Molecular Weight
| 39885.775 |
| Theoretical pI
| 9.103 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
Not Available
|
| External Links |
|---|
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| P00481 |
| UniProtKB/Swiss-Prot Entry Name
| OTC_RAT |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| Not Available |
| GeneCard ID
| Not Available |
| GenAtlas ID
| Not Available |
| HGNC ID
| Not Available |
| References |
|---|
| General References
| - Takiguchi M, Miura S, Mori M, Tatibana M, Nagata S, Kaziro Y: Molecular cloning and nucleotide sequence of cDNA for rat ornithine carbamoyltransferase precursor. Proc Natl Acad Sci U S A. 1984 Dec;81(23):7412-6. doi: 10.1073/pnas.81.23.7412. [PubMed:6095294 ]
- Takiguchi M, Murakami T, Miura S, Mori M: Structure of the rat ornithine carbamoyltransferase gene, a large, X chromosome-linked gene with an atypical promoter. Proc Natl Acad Sci U S A. 1987 Sep;84(17):6136-40. doi: 10.1073/pnas.84.17.6136. [PubMed:3476935 ]
- Kraus JP, Hodges PE, Williamson CL, Horwich AL, Kalousek F, Williams KR, Rosenberg LE: A cDNA clone for the precursor of rat mitochondrial ornithine transcarbamylase: comparison of rat and human leader sequences and conservation of catalytic sites. Nucleic Acids Res. 1985 Feb 11;13(3):943-52. doi: 10.1093/nar/13.3.943. [PubMed:3839075 ]
- McIntyre P, Graf L, Mercer JF, Wake SA, Hudson P, Hoogenraad N: The primary structure of the imported mitochondrial protein, ornithine transcarbamylase from rat liver: mRNA levels during ontogeny. DNA. 1985 Apr;4(2):147-56. doi: 10.1089/dna.1985.4.147. [PubMed:3838931 ]
- McIntyre P, Graf L, Mercer J, Peterson G, Hudson P, Hoogenraad N: A highly basic N-terminal extension of the mitochondrial matrix enzyme ornithine transcarbamylase from rat liver. FEBS Lett. 1984 Nov 5;177(1):41-6. doi: 10.1016/0014-5793(84)80977-1. [PubMed:6548714 ]
- Aoki Y, Sunaga H, Suzuki KT: A cadmium-binding protein in rat liver identified as ornithine carbamoyltransferase. Biochem J. 1988 Mar 15;250(3):735-42. doi: 10.1042/bj2500735. [PubMed:3390141 ]
- McDowall S, van Heeswijck R, Hoogenraad N: Site-directed mutagenesis of Arg60 and Cys271 in ornithine transcarbamylase from rat liver. Protein Eng. 1990 Oct;4(1):73-7. doi: 10.1093/protein/4.1.73. [PubMed:2290837 ]
|
