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

H2O + L-glutamate 5-semialdehyde + NAD(+) => 2 H(+) + L-glutamate + NADH, L-glutamate gamma-semialdehyde + NAD+ => glutamate + NADH + H+

ReviewStatus

5/5

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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
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
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