Ornithine aminotransferase
| ornithine aminotransferase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC no. | 2.6.1.13 | ||||||||
| CAS no. | 9030-42-6 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
| ornithine aminotransferase | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | OAT | ||||||
| NCBI gene | 4942 | ||||||
| HGNC | 8091 | ||||||
| OMIM | 258870 | ||||||
| RefSeq | NM_000274 | ||||||
| UniProt | P04181 | ||||||
| Other data | |||||||
| EC number | 2.6.1.13 | ||||||
| Locus | Chr. 10 q26 | ||||||
| |||||||
Ornithine aminotransferase is an enzyme involved in the ultimate formation of proline from ornithine. Ornithine aminotransferase forms the initial intermediate in this process. It catalyzes the reverse reaction as well, and is therefore essential in creating ornithine from the starting substrate proline. Glutamate acts as the amine donor for ornithine aminotransferase, forming α-ketoglutarate. In the reverse direction, α-ketoglutarate accepts the amine group, forming glutamate and leading to the ultimate conversion of proline to ornithine. A deficiency of this enzyme causes ornithine aminotransferase deficiency, also known as gyrate atrophy of choroid and retina.[1]
Ornithine aminotransferase catalyzes the following reaction:
- L-ornithine + a 2-oxo acid = L-glutamate 5-semialdehyde + an L-amino acid
See also
References
- ^ "Gyrate atrophy of the choroid and retina". National Institutes of Health. Retrieved 2012-08-23.
External links
- Ornithine+aminotransferase at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
- Seiler N (2000). "Ornithine aminotransferase, a potential target for the treatment of hyperammonemias". Curr Drug Targets. 1 (2): 119–53. doi:10.2174/1389450003349254. PMID 11465067.
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