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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ALDH4A1 oxidises L-GluSS to Glu
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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
Input
Output
Participant Of
hasEvent
Catalyst Activity
Catalyst Activity
Title
1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]
Physical Entity
ALDH4A1 dimer [mitochondrial matrix]
Activity
1-pyrroline-5-carboxylate dehydrogenase activity (0003842)
Orthologous Events
ALDH4A1 oxidises L-GluSS to Glu (Danio rerio)
ALDH4A1 oxidises L-GluSS to Glu (Gallus gallus)
ALDH4A1 oxidises L-GluSS to Glu (Mus musculus)
ALDH4A1 oxidises L-GluSS to Glu (Rattus norvegicus)
ALDH4A1 oxidises L-GluSS to Glu (Sus scrofa)
ALDH4A1 oxidises L-GluSS to Glu (Xenopus tropicalis)
Cross References
Rhea
Authored
D'Eustachio, P  (2003-06-24)
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