抗人 IDS 抗体对
01AB01163
Iduronate 2-sulfatase (IDS) is a lysosomal enzyme that catalyzes the hydrolysis of sulfate esters in glycosaminoglycans, specifically heparan sulfate and dermatan sulfate. The 550-amino acid protein is encoded by the IDS gene on the X chromosome and undergoes post-translational processing to form a mature enzyme. IDS contains eight potential N-linked glycosylation sites, which are important for its stability and lysosomal targeting. The enzyme's crystal structure reveals insights into its catalytic mechanism and the impact of pathogenic mutations. Deficiency of IDS leads to mucopolysaccharidosis type II (Hunter syndrome), an X-linked lysosomal storage disorder characterized by the accumulation of partially degraded glycosaminoglycans. This accumulation results in progressive damage to various tissues and organs, with clinical manifestations ranging from mild to severe forms of the disease.
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技术参数
- 靶点基因名
- IDS
- 基因别名
- SIDS
- 蛋白别名
- iduronate 2-sulfatase; IDS
- UniProt
- P22304
- 物种
- Human
产品详情
- 靶点名称
- IDS
- 蛋白名称
- iduronate 2-sulfatase
- 蛋白别名
- Iduronate 2-sulfatase; Alpha-L-iduronate sulfate sulfatase; Idursulfase) [Cleaved into: Iduronate 2-sulfatase 42 kDa chain; Iduronate 2-sulfatase 14 kDa chain]
- 靶点基因名
- IDS
- 基因别名
- SIDS
- UniProt
- P22304
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Iduronate 2-sulfatase (IDS) is a lysosomal enzyme that catalyzes the hydrolysis of sulfate esters in glycosaminoglycans, specifically heparan sulfate and dermatan sulfate. The 550-amino acid protein is encoded by the IDS gene on the X chromosome and undergoes post-translational processing to form a mature enzyme. IDS contains eight potential N-linked glycosylation sites, which are important for its stability and lysosomal targeting. The enzyme's crystal structure reveals insights into its catalytic mechanism and the impact of pathogenic mutations. Deficiency of IDS leads to mucopolysaccharidosis type II (Hunter syndrome), an X-linked lysosomal storage disorder characterized by the accumulation of partially degraded glycosaminoglycans. This accumulation results in progressive damage to various tissues and organs, with clinical manifestations ranging from mild to severe forms of the disease.