Western blot analysis of ALDH6A1 (arrow) using rabbit ALDH6A1 polyclonal antibody (Cat # PAB3128). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the ALDH6A1 gene (Lane 2) (Origene Technologies).
Formalin-fixed and paraffin-embedded human hepatocellular carcinoma reacted with ALDH6A1 polyclonal antibody (Cat # PAB3128) , which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.
This protein belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. The product of this gene, a mitochondrial methylmalonate semialdehyde dehydrogenase, 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. [provided by RefSeq