Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) catalyzes the reaction of L-glutamate gamma-semialdehyde and NAD+ to form glutamate and NADH + H+ (Hu et al. 1996). The enzyme is a dimer (Forte-McRobbie and Pietruszko 1986). ALDH4A1 mutations cause type II hyperprolinemia in vivo (Geraghty et al. 1998).
Jimenez-Sanchez, G, Flynn, MP, Nicholson, AJ, Obie, C, Lin, WW, Geraghty, MT, Vaughn, D, Valle, D, Hu, CA
Forte-McRobbie, CM, Pietruszko, R
1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]
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