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

Stable Identifier
R-HSA-70893
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participates
Catalyst Activity

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

Orthologous Events
Cite Us!