Reaction: methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+
- in pathway: Branched-chain amino acid catabolism
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).
Reaction - small molecule participants:
PROP-CoA [mitochondrial matrix]
H+ [mitochondrial matrix]
NADH [mitochondrial matrix]
CO2 [mitochondrial matrix]
CoA-SH [mitochondrial matrix]
NAD+ [mitochondrial matrix]
2M3OPROA [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70893
======
Reaction input - small molecules:
coenzyme A(4-)
NAD(1-)
2-methyl-3-oxopropanoate
Reaction output - small molecules:
propionyl-CoA
hydron
NADH(2-)
carbon dioxide
Reactome.org link: R-HSA-70893