Iduronate 2 sulfatase/IDS Protein, Human, Recombinant (His)

カタログ番号 TMPY-00828

別名: MPS2, IDS, SIDS, iduronate 2-sulfatase

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.

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Iduronate 2 sulfatase/IDS Protein, Human, Recombinant (His)

パッケージサイズ	在庫状況	単価(税別)
50 μg	在庫あり	￥ 88,500
100 μg	約5 days	￥ 151,500
200 μg	約5 days	￥ 257,000
500 μg	約5 days	￥ 521,000

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生物学的特性に関する説明

Technical Params

Product Properties

参考文献

生物学的情報

Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is > 1.0 pmoles/min/μg.

説明

Species	Human
Expression Host	HEK293 Cells
Tag	C-His
Accession Number	P22304-1
別名	MPS2, IDS, SIDS, iduronate 2-sulfatase
Construction	Human IDS precursor (NP_000193.1) (Met 1-Pro 550)
Protein Purity	> 87 % as determined by SDS-PAGE
分子量	61 kDa (predicted)

Endotoxin	< 1.0 EU/μg of the protein as determined by the LAL method.
Formulation	Lyophilized from a solution filtered through a 0.22 μm filter, containing PBS, pH 7.4. Typically, a mixture containing 5% to 8% trehalose, mannitol, and 0.01% Tween 80 is incorporated as a protective agent before lyophilization.
Reconstitution	A Certificate of Analysis (CoA) containing reconstitution instructions is included with the products. Please refer to the CoA for detailed information.
Stability & Storage	It is recommended to store recombinant proteins at -20°C to -80°C for future use. Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots.
Shipping	In general, Lyophilized powders are shipping with blue ice.
Research Background	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.

参考文献

1. 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. 2. Wilson P.J.,et al., (1993), Sequence of the human iduronate 2-sulfatase (IDS) gene.Genomics 17:773-775. 3. 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.

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Keywords

Iduronate 2 sulfatase/IDS Protein, Human, Recombinant (His) MPS2 IDS SIDS iduronate 2-sulfatase MPS 2 MPS-2 recombinant recombinant-proteins proteins protein