methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+

Stable Identifier
R-HSA-70893
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Mitochondrial methylmalonate semialdehyde dehydrogenase (ALDH6A1) catalyzes the reaction of methylmalonate semialdehyde, NAD+, and CoA to form propionyl-CoA, CO2, and NADH + H+. A human ALDH6A1 gene has been cloned. Its sequence is closely homologous to that of the better-characterized rat enzyme (Kedishvili et al. 1992) and a missense mutation in a normally well-conserved codon has been found in the allele of the gene from a patient with a defect in methylmalonic semialdehyde dehydrogenase activity (Chambliss et al. 2000).

Literature References
PubMed ID Title Journal Year
10947204 Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency

Chambliss, KL, Gray, RG, Rylance, G, Pollitt, RJ, Gibson, KM

J Inherit Metab Dis 2000
1527093 CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution

Kedishvili, NY, Popov, KM, Rougraff, PM, Zhao, Y, Crabb, DW, Harris, RA

J Biol Chem 1992
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
methylmalonate-semialdehyde dehydrogenase (acylating) activity of ALDH6A1 [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events