The HLCS Polyclonal Antibody (PACO09736) is a valuable tool for researchers studying the HLCS protein, an essential enzyme in the biosynthesis of biotin. This antibody, produced in rabbits, exhibits high specificity and sensitivity for detecting HLCS in human samples, making it suitable for Western blot applications. By targeting the HLCS protein, researchers can investigate its role in biotin metabolism and its potential implications in various physiological processes.HLCS, also known as holocarboxylase synthetase, is a critical enzyme involved in the activation of biotin-dependent carboxylases, which play key roles in fatty acid synthesis and energy metabolism.
Dysregulation of HLCS function has been linked to biotin deficiency disorders and metabolic abnormalities. Understanding the function of HLCS is essential for uncovering its impact on human health and developing targeted therapies for related conditions.Overall, the HLCS Polyclonal Antibody (PACO09736) serves as a valuable tool for researchers interested in exploring the role of HLCS in biotin metabolism and its implications for human health. Its high specificity and reactivity make it an ideal choice for studies in biochemistry, nutrition, and metabolic disorders.
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: EC 6.3.4.11; Mitochondrial; EC 6.3.4.15; EC 6.3.4.9; EC 6.3.4.10; Cofactor and Vitamin Metabolism - biotin; LigaseChromosomal Location of Human Ortholog: 21q22.13Cellular Component: nuclear lamina; nuclear matrix; mitochondrion; cytoplasm; chromatin; cytosolMolecular Function: protein binding; 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; biotin-protein ligase activity; biotin binding; ATP binding; biotin-[methylcrotonoyl-CoA-carboxylase] ligase activityBiological Process: cell proliferation; vitamin metabolic process; histone modification; protein amino acid biotinylation; water-soluble vitamin metabolic process; biotin metabolic processDisease: Holocarboxylase Synthetase Deficiency
UniProt Protein Details:
NCBI Summary:
This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011]
