抗人 HLCS 抗体对
01AB01498
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 ε-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.
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技术参数
- 靶点基因名
- HLCS
- 蛋白别名
- holocarboxylase synthetase; HLCS
- UniProt
- P50747
- 物种
- Human
产品详情
- 靶点名称
- HLCS
- 蛋白名称
- holocarboxylase synthetase
- 蛋白别名
- Biotin--protein ligase; Biotin apo-protein ligase) [Includes: Biotin--[methylmalonyl-CoA-carboxytransferase] ligase; Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Holocarboxylase synthetase; HCS; Biotin--[methylcrotonoyl-CoA-carboxylase] ligase; Biotin--[acetyl-CoA-carboxylase] ligase]
- 靶点基因名
- HLCS
- UniProt
- P50747
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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 ε-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.