ALDH6A1 belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. This protein is a mitochondrial methylmalonate semialdehyde dehydrogenase, and catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids.
Immunogen
ALDH6A1 (NP_005580, 35-69) This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the N-terminal region of human ALDH6A1.
Physical form
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide.
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