4-aminobutyrate aminotransferase, mitochondrial; gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; GABA aminotransferase; GABA-AT; L-AIBAT; (S)-3-amino-2-methylpropionate transaminase (ABAT, GABAT)
(Redirected from (S)-3-amino-2-methylpropionate transaminase)
Jump to navigation
Jump to search
Function
- catalyzes conversion of:
- gamma-aminobutyrate to succinate semialdehyde &
- L-beta-aminoisobutyrate to methylmalonatesemialdehyde
- can also convert delta-aminovalerate & beta-alanine
4-aminobutanoate + 2-oxoglutarate
<-->
succinate semialdehyde + L-glutamate
(S)-3-amino-2-methylpropanoate + 2-oxoglutarate
<-->
2-methyl-3-oxopropanoate + L-glutamate
Cofactor: pyridoxal phosphate
Structure
- homodimer (putative)
- belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family
Compartment
Expression
Pathology
- defects in ABAT are a cause of GABA-AT deficiency
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P80404.html
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=ABAT
- ↑ SHMPD; The Singapore human mutation and polymorphism database http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=ABAT+%40+GABAT