4-aminobutyrate aminotransferase, mitochondrial

Definition:

Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).

References:

[1]. L K Medina-Kauwe, et al. 4-Aminobutyrate aminotransferase (GABA-transaminase) deficiency. J Inherit Metab Dis. 1999 Jun;22(4):414-27. [Content Brief]

[2]. Chang Sik Yoon, et al. Cysteine-321 of human brain GABA transaminase is involved in intersubunit cross-linking. Mol Cells. 2004 Oct 31;18(2):214-9. [Content Brief]

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