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ornithine aminotransferase (Ornithine-oxo-acid aminotransferase, OAT)
Function:
- amino-acid biosynthesis; L-proline biosynthesis
- L-glutamate 5-semialdehyde from L-ornithine: step 1/1
- catalyzes transfer of an amino group from ornithine to alpha-ketoglutarate yielding glutamic-5-semi-aldehyde & glutamic acid
L-ornithine + a 2-oxo acid
L-glutamate 5-semialdehyde + an L-amino acid
Cofactor: pyridoxal phosphate
Structure:
- homotetramer
- belongs to the class-3 pyridoxal-phosphate-dependent aminotransferase family
Compartment: mitochondrial matrix
Pathology:
- defects in OAT are the cause of hyperornithinemia
General
aminotransferase
mitochondrial protein
Properties
SIZE: entity length = 439 aa
MW = 49 kD
COMPARTMENT: mitochondrial matrix
MOTIF: cofactor-binding site [292-292]
FOR-BINDING-OF: pyridoxal phosphate
Database Correlations
OMIM correlations
UniProt P04181
Pfam PF00202
KEGG correlations
ENZYME 2.6.1.13
References
- UniProt :accession P04181
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=OAT