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Details on Person Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase ...

Class:IdSummation:70680
_displayNameMitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase ...
_timestamp2010-02-18 17:29:53
created[InstanceEdit:70671] 2003-03-13 00:00:00
literatureReference[LiteratureReference:70675] Purification and characterization of human liver "high Km" aldehyde dehydrogenase and its identification as glutamic gamma-semialdehyde dehydrogenase.
[LiteratureReference:70681] Mutations in the Delta1-pyrroline 5-carboxylate dehydrogenase gene cause type II hyperprolinemia.
[LiteratureReference:517457] Cloning, characterization, and expression of cDNAs encoding human delta 1-pyrroline-5-carboxylate dehydrogenase
modified[InstanceEdit:517454] D'Eustachio, P, 2010-02-18
textMitochondrial 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).
(summation)[Reaction:70679] ALDH4A1 oxidises L-GluSS to Glu [Homo sapiens]
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