Reactome | methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+

methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+

Stable Identifier

R-HSA-70893

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(S)-2-methyl-3-oxopropanoate + CoA + H2O + NAD(+) => H(+) + hydrogencarbonate + NADH + propanoyl-CoA

ReviewStatus

5/5

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methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + 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
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
10947204	Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency Chambliss, KL, Gray, RG, Rylance, G, Pollitt, RJ, Gibson, KM	J Inherit Metab Dis	2000