| 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 |
| liver development |
| response to drug |
| response to biotin |
| anion homeostasis |
| citrulline biosynthetic process |
| midgut development |
| protein homotrimerization |
| urea cycle |
| response to zinc ion |
| arginine biosynthetic process |
| response to insulin stimulus |
| Cellular Component |
| mitochondrial matrix |
| ornithine carbamoyltransferase complex |
| mitochondrial inner membrane |
| Component |
| ornithine carbamoyltransferase complex |
| macromolecular complex |
| protein complex |
| Function |
| transferase activity |
| ornithine carbamoyltransferase activity |
| carboxyl- or carbamoyltransferase activity |
| carboxylic acid binding |
| amino acid binding |
| transferase activity, transferring one-carbon groups |
| binding |
| catalytic activity |
| 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 |