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

Stable Identifier
R-HSA-70893
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
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methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+
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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
Input
Output
Participant Of
hasEvent
Catalyst Activity
Catalyst Activity
Title
methylmalonate-semialdehyde dehydrogenase (acylating) activity of ALDH6A1 [mitochondrial matrix]
Physical Entity
ALDH6A1 [mitochondrial matrix]
Activity
methylmalonate-semialdehyde dehydrogenase (acylating) activity (0004491)
Orthologous Events
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Caenorhabditis elegans)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Canis familiaris)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Danio rerio)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Dictyostelium discoideum)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Drosophila melanogaster)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Gallus gallus)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Mus musculus)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Rattus norvegicus)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Sus scrofa)
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ (Xenopus tropicalis)