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- サプライヤー
- TargteMol
- カタログNo.
- TMPY-00828
- 製品名称
- Iduronate 2 sulfatase/IDS Protein, Human, Recombinant (His)
- タンパク質名
- Iduronate 2 sulfatase/IDS
- Species
- Human
- HOST
- HEK293 Cells
- 50μg: -
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Overview
| Synonyms | MPS2, IDS, SIDS, iduronate 2-sulfatase |
|---|---|
| Characteristics | Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is > 1.0 pmoles/min/μg. |
| Endotoxin Level | < 1.0 EU/μg of the protein as determined by the LAL method. |
| Purity | 96.60% |
| Description | Iduronate 2-Sulfatase, also known as IDS, is a member of the highly conserved sulfatase family of enzymes that catalyze the hydrolysis of O- and N-sulfate esters from a variety of substrates. The human Iduronate 2-Sulfatase/IDS consists of a signal peptide, a propeptide, and a mature chain that may be further processed into two chains. Among the identified 18 human sulfatases, Iduronate 2-Sulfatase/IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG), heparan sulfate, and dermatan sulfate. Multiple mutations in this X-chromosome localized gene result in Iduronate 2-Sulfatase/IDS enzymatic deficiency and lead to the sex-linked Mucopolysaccharidosis Type II (MPS II ), also known as Hunter Syndrome characterized by the lysosomal accumulation of the GAG and their excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe due to the level of Iduronate 2-Sulfatase/IDS enzyme. Retroviral-mediated Iduronate 2-Sulfatase/IDS gene transfer into lymphoid cells would be a promising gene therapeutic strategy. |
| Reference | Timms K.M.,et al.,(1995), 130 kb of DNA sequence reveals two new genes and a regional duplication distal to the human iduronate-2-sulfate sulfatase locus.Genome Res. 5:71-78.,Wilson P.J.,et al.,(1990), Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA. Proc. Natl. Acad. Sci. U.S.A. 87:8531-8535.,Wilson P.J.,et al., (1993), Sequence of the human iduronate 2-sulfatase (IDS) gene.Genomics 17:773-775. |
| URL | https://www.targetmol.com/recombinant-protein/iduronate_2_sulfatase_ids_protein_human_recombinant_his_tag_ |
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