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

Stable Identifier
Reaction [transition]
Homo sapiens
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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
1527093 CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution

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

J Biol Chem 1992
10947204 Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency

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

J Inherit Metab Dis 2000
Catalyst Activity

methylmalonate-semialdehyde dehydrogenase (acylating) activity of ALDH6A1 [mitochondrial matrix]

Orthologous Events
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