Anti-ALDH6A1 monoclonal antibody, clone 258DU9.4.5

Antibody Isotype

IgG1, κ

Clone

258DU9.4.5

Species Reactivity

Human

Conjugate

Application Notes

WB: 1:1000

Alternative Names

ALDH6A1; MMSDH; Methylmalonate-semialdehyde dehydrogenase [acylating]; mitochondrial; Aldehyde dehydrogenase family 6 member A1

Entrez Gene ID

UniProt ID

Antigen Description

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.

Pathway

Branched-chain amino acid catabolism; Carbon metabolism; Inositol phosphate metabolism; Malonate semialdehyde pathway, propanoyl-CoA => Acetyl-CoA; Metabolism; Metabolism of amino acids and derivatives; Propanoate metabolism;