Anti-Human HLCS Antibody Pair

Product Description: Holocarboxylase synthetase (HLCS) is a monomeric biotin ligase encoded by the HLCS gene on chromosome 21q22.13, with a full-length protein comprising 726 amino acids and a predicted molecular weight of 81 kDa. HLCS catalyzes the covalent attachment of biotin to the epsilon-amino group of lysine residues in five biotin-dependent carboxylases, a process essential for the metabolism of proteins, fats, and carbohydrates. The enzyme functions through a two-step ATP-dependent reaction and is expressed from three promoters, resulting in multiple protein isoforms detected in human tissues. Beyond its classical metabolic role, HLCS may also biotinylate histones in the nucleus, suggesting a potential function in gene regulation. Deficiency of HLCS, a rare autosomal recessive disorder, impairs biotin-dependent carboxylase activity, leading to metabolic disturbances that can cause serious health problems if untreated. Protein Function: Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase [The Uniprot Consortium]