Names & Taxonomy
- Uniprot ID:
- P00480
- Entry Name:
- OTC_HUMAN
- Status:
- reviewed
- Protein Names:
- Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)
- Gene Names:
- OTC
- Gene Names Primary:
- OTC
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 354
- Sequence:
- MLFNLRILLNNAAFRNGHNFMVRNFRCGQPLQNKVQLKGRDLLTLKNFTGEEIKYMLWLSADLKFRIKQKGEYLPLLQGKSLGMIFEKRSTRTRLSTETGFALLGGHPCFLTTQDIHLGVNESLTDTARVLSSMADAVLARVYKQSDLDTLAKEASIPIINGLSDLYHPIQILADYLTLQEHYSSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQAATPKGYEPDASVTKLAEQYAKENGTKLLLTNDPLEAAHGGNVLITDTWISMGQEEEKKKRLQAFQGYQVTMKTAKVAASDWTFLHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPQLQKPKF
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- Pathway:
- Nitrogen metabolism; urea cycle; L-citrulline from L-ornithine and carbamoyl phosphate: step 1/1.
- Catalytic Activity:
- Carbamoyl phosphate + L-ornithine = phosphate + L-citrulline.
- Enzyme Regulation:
- ENZYME REGULATION: Negatively regulated by lysine acetylation.
- Active Site:
- ACT_SITE 303 303
- Cross Reference Drug Bank:
- DB00155 DB00129
- Gene Ontology Go:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion
amino acid binding
ornithine carbamoyltransferase activity
phosphate ion binding
phospholipid binding
ammonia homeostasis
arginine biosynthetic process via ornithine
cellular nitrogen compound metabolic process
citrulline biosynthetic process
liver development
midgut development
ornithine catabolic process
polyamine metabolic process
protein homotrimerization
response to biotin
response to drug
response to insulin
response to zinc ion
small molecule metabolic process
urea cycle - Gene Ontology Biological Process:
- ammonia homeostasis
arginine biosynthetic process via ornithine
cellular nitrogen compound metabolic process
citrulline biosynthetic process
liver development
midgut development
ornithine catabolic process
polyamine metabolic process
protein homotrimerization
response to biotin
response to drug
response to insulin
response to zinc ion
small molecule metabolic process
urea cycle - Gene Ontology Molecular Function:
- amino acid binding
ornithine carbamoyltransferase activity
phosphate ion binding
phospholipid binding - Gene Ontology Cellular Component:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Acetylation
Amino-acid biosynthesis
Arginine biosynthesis
Complete proteome
Disease mutation
Mitochondrion
Phosphoprotein
Polymorphism
Reference proteome
Transferase
Transit peptide
Urea cycle
Publication
- PubMed ID:
- 6372096 2836378 14702039 15772651 15489334 3895227 3782067 8081373 24275569 8112735 19318352 9852088 10813810 8364586 7627182 8544185 3170748 2556444 2474822 2347583 1671317 1721894 1480464 8099056 8019569 8081398 7951259 8530002 7474905 8807340 8956038 8956045 8830175 9286441 9065786 9143919 9266388 9452024 9452049 9452065 10502831 10070627 10737985 11793483 16959974