GABA is metabolized in the mitochondrial matrix to succinate by the serial action of two enzymes, 4-aminobutyrate aminotransferase and suucinate semialdehyde dehydrogenase. Failure of the second reaction is associated with a rare human genetic disorder (Malaspina et al. 2016; Pearl et al. 2009).
Pettiford, JM, Forester, K, Wu, Y, Theodore, WH, Knerr, I, Carter Snead O, 3rd, Cortez, MA, Gibson, KM, Pearl, P L, Jakobs, C
Roullet, JB, Gibson, KM, Ainslie, GR, Pearl, P L, Malaspina, P, Vogel, KR
© 2023 Reactome