抗人 ARSB 抗体对
01AB00724
Arylsulfatase B (ARSB), also known as N-acetylgalactosamine-4-sulfatase, is a lysosomal enzyme involved in the degradation of glycosaminoglycans (GAGs), specifically dermatan sulfate and chondroitin sulfate. It hydrolyzes sulfate groups from these GAGs, facilitating their breakdown and recycling within lysosomes. ARSB consists of a tetrameric structure with complex alpha-helices and beta-sheets that contribute to its enzymatic activity. The enzyme's functionality depends on factors such as pH and local concentration, which regulate its subunit interactions and activity levels. ARSB is also found outside lysosomes, localized to cell membranes in tissues like the liver and colon, where it influences processes like cell adhesion and migration. Mutations in the ARSB gene lead to mucopolysaccharidosis type VI (MPS VI), a lysosomal storage disorder characterized by the accumulation of GAGs. This results in cellular dysfunction, inflammation, and organ enlargement.
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技术参数
- 靶点基因名
- ARSB
- 蛋白别名
- arylsulfatase B; ARSB
- UniProt
- P15848
- 物种
- Human
产品详情
- 靶点名称
- ARSB
- 蛋白名称
- arylsulfatase B
- 蛋白别名
- Arylsulfatase B; ASB; N-acetylgalactosamine-4-sulfatase; G4S
- 靶点基因名
- ARSB
- UniProt
- P15848
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Arylsulfatase B (ARSB), also known as N-acetylgalactosamine-4-sulfatase, is a lysosomal enzyme involved in the degradation of glycosaminoglycans (GAGs), specifically dermatan sulfate and chondroitin sulfate. It hydrolyzes sulfate groups from these GAGs, facilitating their breakdown and recycling within lysosomes. ARSB consists of a tetrameric structure with complex alpha-helices and beta-sheets that contribute to its enzymatic activity. The enzyme's functionality depends on factors such as pH and local concentration, which regulate its subunit interactions and activity levels. ARSB is also found outside lysosomes, localized to cell membranes in tissues like the liver and colon, where it influences processes like cell adhesion and migration. Mutations in the ARSB gene lead to mucopolysaccharidosis type VI (MPS VI), a lysosomal storage disorder characterized by the accumulation of GAGs. This results in cellular dysfunction, inflammation, and organ enlargement.