Identification |
HMDB Protein ID
| CDBP00870 |
Secondary Accession Numbers
| Not Available |
Name
| Ornithine carbamoyltransferase, mitochondrial |
Description
| Not Available |
Synonyms
|
- OTCase
- Ornithine transcarbamylase
|
Gene Name
| OTC |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in carboxyl- or carbamoyltransferase activity |
Specific Function
| Not Available |
GO Classification
|
Biological Process |
midgut development |
protein homotrimerization |
urea cycle |
response to zinc ion |
arginine biosynthetic process |
response to insulin stimulus |
liver development |
response to drug |
response to biotin |
anion homeostasis |
citrulline biosynthetic process |
Cellular Component |
mitochondrial matrix |
ornithine carbamoyltransferase complex |
mitochondrial inner membrane |
Component |
ornithine carbamoyltransferase complex |
macromolecular complex |
protein complex |
Function |
transferase activity, transferring one-carbon groups |
binding |
catalytic activity |
transferase activity |
ornithine carbamoyltransferase activity |
carboxyl- or carbamoyltransferase activity |
carboxylic acid binding |
amino acid binding |
Molecular Function |
ornithine carbamoyltransferase activity |
phospholipid binding |
phosphate ion binding |
amino acid binding |
Process |
metabolic process |
cellular metabolic process |
cellular amino acid and derivative metabolic process |
cellular amino acid metabolic process |
|
Cellular Location
|
- Mitochondrion matrix
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | Arginine and Proline Metabolism | | | Urea Cycle | | | Prolidase Deficiency (PD) | | Not Available | Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) | | Not Available | Hyperprolinemia Type II | | Not Available |
|
Gene Properties |
Chromosome Location
| X |
Locus
| Xp21.1 |
SNPs
| OTC |
Gene Sequence
|
>1065 bp
ATGCTGTTTAATCTGAGGATCCTGTTAAACAATGCAGCTTTTAGAAATGGTCACAACTTC
ATGGTTCGAAATTTTCGGTGTGGACAACCACTACAAAATAAAGTGCAGCTGAAGGGCCGT
GACCTTCTCACTCTAAAAAACTTTACCGGAGAAGAAATTAAATATATGCTATGGCTATCA
GCAGATCTGAAATTTAGGATAAAACAGAAAGGAGAGTATTTGCCTTTATTGCAGGGGAAG
TCCTTAGGCATGATTTTTGAGAAAAGAAGTACTCGAACAAGATTGTCTACAGAAACAGGC
TTTGCACTTCTGGGAGGACATCCTTGTTTTCCTACCACACAAGATATTCATTTGGGTGTG
AATGAAAGTCTCACGGACACGGCCCGTGTATTGTCTAGCATGGCAGATGCAGTATTGGCT
CGAGTGTATAAACAATCAGATTTGGACACCCTTGCTAAAGAAGCATCCATCCCAATTATC
AATGGGCTGTCAGATTTGTACCATCCTATCCAGATCCTGGCTGATTACCTCACGCTCCAG
GAACACTATAGCTCTCTGAAAGGTCTTACCCTCAGCTGTTTCGGGGATGGGAACAATATC
CTGCACTCCATCATGATGAGCGCAGCGAAATTCGGAATGCACCTTCAGGCAGCTACTCCA
AAGGGTTATGAGCCGGATGCTAGTGTAACCAAGTTGGCAGAGCAGTATGCCAAAGAGAAT
GGTACCAAGCTGTTGCTGACAAATGATCCATTGGAAGCAGCGCATGGAGGCAATGTATTA
ATTACAGACACTTGGATAAGCATGGGACGAGAAGAGGAGAAGAAAAAGCGGCTCCAAGCT
TTCCAAGGTTACCAAGTTACAATGAAGACTGCTAAAGTTGCTGCCTCTGACTGGACATTT
TTACACTGCTTGCCCAGAAAGCCAGAAGAAGTGGATGATGAAGTCTTTTATTCTCCTCGA
TCACTAGTGTTCCCAGAGGCAGAAAACAGAAAGTGGACAATCATGGCTGTCATGGTGTCC
CTGCTGACAGATTACTCACCTCAGCTCCAGAAGCCTAAATTTTGA
|
Protein Properties |
Number of Residues
| 354 |
Molecular Weight
| 39934.775 |
Theoretical pI
| 8.633 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Ornithine carbamoyltransferase, mitochondrial
MLFNLRILLNNAAFRNGHNFMVRNFRCGQPLQNKVQLKGRDLLTLKNFTGEEIKYMLWLS
ADLKFRIKQKGEYLPLLQGKSLGMIFEKRSTRTRLSTETGFALLGGHPCFLTTQDIHLGV
NESLTDTARVLSSMADAVLARVYKQSDLDTLAKEASIPIINGLSDLYHPIQILADYLTLQ
EHYSSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQAATPKGYEPDASVTKLAEQYAKEN
GTKLLLTNDPLEAAHGGNVLITDTWISMGQEEEKKKRLQAFQGYQVTMKTAKVAASDWTF
LHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPQLQKPKF
|
External Links |
GenBank ID Protein
| 189407 |
UniProtKB/Swiss-Prot ID
| P00480 |
UniProtKB/Swiss-Prot Entry Name
| OTC_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| K02100 |
GeneCard ID
| OTC |
GenAtlas ID
| OTC |
HGNC ID
| HGNC:8512 |
References |
General References
| Not Available |