R&D Systems代理BAF4119 Human alpha-L-Iduronidase/IDUA Biotinylated Affinity Pur PAb (50 UG)

2025-06-28

货号:BAF4119

品牌:R&D Systems

规格:50ug

目录价:¥5580.00

市场价格:¥4464.00

会员价格:¥4464.00

  • 到货时间:3~4周

    金山科研平台,产品价格货期咨询微信:jinshanbio Molecule Information: alpha-L-Iduronidase/IDUA Aliases: IDA; IDUA; MPS1 Entrez Gene IDs: 3425 (Human); 15932 (Mouse); 360904 (Rat) Background: alpha-L-Iduronidase/IDUA alpha-L-Iduronidase, encoded by the IDUA gene, is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non-reducing terminal a-L-iduronic acid residues in GAGs, including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). MPS I causes progressive cellular, tissue, and organ damage.

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