Reactome | ALDH4A1 oxidises L-GluSS to Glu

ALDH4A1 oxidises L-GluSS to Glu

Stable Identifier

R-HSA-70679

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

L-glutamate gamma-semialdehyde + NAD+ => glutamate + NADH + H+

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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).

Literature References

PubMed ID	Title	Journal	Year
3944130	Purification and characterization of human liver "high Km" aldehyde dehydrogenase and its identification as glutamic gamma-semialdehyde dehydrogenase. Forte-McRobbie, CM, Pietruszko, R	J Biol Chem	1986
9700195	Mutations in the Delta1-pyrroline 5-carboxylate dehydrogenase gene cause type II hyperprolinemia. Geraghty, MT, Vaughn, D, Nicholson, AJ, Lin, WW, Jimenez-Sanchez, G, Obie, C, Flynn, MP, Valle, D, Hu, CA	Hum Mol Genet	1998