HLCS Antibody – Mouse Monoclonal

HLCS: Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase. Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency); also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin. Belongs to the biotin--protein ligase family.

Protein type: Mitochondrial; EC 6.3.4.9; Cofactor and Vitamin Metabolism - biotin; Ligase; EC 6.3.4.11; EC 6.3.4.15; EC 6.3.4.10

Cellular Component: nuclear lamina; nuclear matrix; mitochondrion; cytoplasm; chromatin; cytosol

Molecular Function: tRNA(Ile)-lysidine synthase; biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity; enzyme binding; protein homodimerization activity; biotin-[acetyl-CoA-carboxylase] ligase activity; biotin-[methylmalonyl-CoA-carboxytransferase] ligase activity; nucleotide binding; biotin-protein ligase activity; lipoate-protein ligase activity; catalytic activity; biotin binding; biotin-[methylcrotonoyl-CoA-carboxylase] ligase activity; ATP binding; ligase activity

Biological Process: cell proliferation; metabolic process; histone modification; protein modification process; protein amino acid biotinylation