The HLCS Polyclonal Antibody (PAC059740) is a valuable tool for researchers studying the HLCS protein, which plays a critical role in the transfer of a methyl group from methylcobalamin to homocysteine to produce methionine. This key metabolic process is essential for DNA methylation, protein synthesis, and neurotransmitter production.Produced in rabbits, this antibody is highly specific to human samples and has been validated for use in Western blot applications. By binding to the HLCS protein, researchers can accurately detect and analyze its expression in various cell types.
This makes the antibody ideal for studies focused on understanding metabolic pathways, particularly those related to methionine and homocysteine metabolism.The HLCS Polyclonal Antibody is a valuable tool for researchers investigating conditions related to aberrant methionine metabolism, such as hyperhomocysteinemia and homocystinuria. By gaining a deeper understanding of the role of the HLCS protein in these conditions, researchers may uncover new therapeutic targets and treatment strategies.
Antibody Name:
HLCS Antibody (PACO59740)
Antibody SKU:
PACO59740
Size:
50ug
Host Species:
Rabbit
Tested Applications:
ELISA, IHC
Recommended Dilutions:
ELISA:1:2000-1:10000, IHC:1:200-1:500
Species Reactivity:
Human
Immunogen:
Recombinant Human Biotin--protein ligase protein (603-714AA)
IHC image of PACO59740 diluted at 1:200 and staining in paraffin-embedded human endometrial cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of PACO59740 diluted at 1:200 and staining in paraffin-embedded human prostate tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Background:
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.
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]
