FunctionCatalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Involvement in diseaseDefects in ABAT are a cause of gamma-aminobutyrate transaminase deficiency (GABA-AT deficiency) [MIM:613163]. The phenotype of this deficiency includes psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities.
Sequence similaritiesBelongs to the class-III pyridoxal-phosphate-dependent aminotransferase family.