Iduronate 2 sulfatase/IDS Active Protein | IDS active protein

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

Cat. Num. AAA8120204

• Gene Names: IDS; MPS2; SIDS
• Purity: >87% as determined by SDS-PAGE
• Synonyms: Iduronate 2 sulfatase/IDS; Iduronate 2 sulfatase/IDS Protein; Human; Recombinant (His Tag); Human Iduronate 2-Sulfatase/IDS Protein (His Tag); IDS Protein; MPS2 Protein; SIDS Protein; iduronate 2-sulfatase; IDS active protein

• Host: HEK293 Cells
• Purity/Purification: >87% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Pro550

• Species: Human
• Activity: Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is >1.0pmoles/min/ug.
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Ser 26
• Tag: C-His
• Protein Construction: A DNA sequence encoding human IDS precursor (NP_000193.1)(Met 1-Pro 550) was expressed with a C-terminal polyhistidine tag.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for IDS active protein

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.

References

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.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.

NCBI and Uniprot Product Information

• NCBI GI #: 4557659
• NCBI GeneID: 3423
• NCBI Accession #: NP_000193.1
• NCBI GenBank Nucleotide #: NM_000202.7
• UniProt Accession #: P22304; Q14604; Q9BRM3; D3DWT4
• Molecular Weight: 34,893 Da
• NCBI Official Full Name: iduronate 2-sulfatase isoform a preproprotein
• NCBI Official Synonym Full Names: iduronate 2-sulfatase
• NCBI Official Symbol: IDS
• NCBI Official Synonym Symbols: MPS2; SIDS
• NCBI Protein Information: iduronate 2-sulfatase
• UniProt Protein Name: Iduronate 2-sulfatase
• Protein Family: Iduronate 2-sulfatase
• UniProt Gene Name: IDS
• UniProt Synonym Gene Names: SIDS; Idursulfase
• UniProt Entry Name: IDS_HUMAN

NCBI Description

This gene encodes a member of the sulfatase family of proteins. The encoded preproprotein is proteolytically processed to generate two polypeptide chains. This enzyme is involved in the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease mucopolysaccharidosis type II, also known as Hunter syndrome. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

Uniprot Description

IDS: Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2); also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Belongs to the sulfatase family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.6.13; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: lysosomal lumen

Molecular Function: iduronate-2-sulfatase activity; metal ion binding

Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; pathogenesis

Disease: Mucopolysaccharidosis, Type Ii