ALDH4A1 oxidises L-GluSS to Glu

Stable Identifier
R-HSA-70679
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored