ALDH4A1 oxidises L-GluSS to Glu

Stable Identifier
R-HSA-70679
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
L-glutamate gamma-semialdehyde + NAD+ => glutamate + NADH + H+
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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.

Jimenez-Sanchez, G, Flynn, MP, Nicholson, AJ, Obie, C, Lin, WW, Geraghty, MT, Vaughn, D, 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
Participants
Participates
Catalyst Activity

1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]

Orthologous Events
Cross References
Rhea
Authored
Cite Us!