Description
| Product Name: | Human IDS Recombinant Protein |
| Product Code: | RPPB1846 |
| Size: | 10µg |
| Species: | Human |
| Target: | IDS |
| Synonyms: | Iduronate 2-Sulfatase, Alpha-L-Iduronate Sulfate Sulfatase, SIDS, Iduronate 2-Sulfatase 14 KDa Chain, Iduronate 2-Sulfatase 42 KDa Chain, Hunter Syndrome, EC 3.1.6.13, MPS2, Iduronate 2-sulfatase, Alpha-L-iduronate sulfate sulfatase. |
| Source: | Sf9 Insect cells |
| Physical Appearance: | Sterile Filtered clear solution. |
| Formulation: | IDS protein solution (0.25mg/ml) containing PhosphateBuffered Saline (pH 7.4) and 10% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks.�Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 90% as determined by SDS-PAGE. |
| Amino Acid Sequence: | SETQANSTTD ALNVLLIIVD DLRPSLGCYG DKLVRSPNID QLASHSLLFQNAFAQQAVCA PSRVSFLTGR RPDTTRLYDF NSYWRVHAGN FSTIPQYFKE NGYVTMSVGK VFHPGISSNHTDDSPYSWSF PPYHPSSEKY ENTKTCRGPD GELHANLLCP VDVLDVPEGT LPDKQSTEQAIQLLEKMKTS ASPFFLAVGY HKPHIPFRYP KEFQKLYPLE NITLAPDPEVPDGLPPVAYN PWMDIRQRED VQALNISVPY GPIPVDFQRK IRQSYFASVS YLDTQVGRLL SALDDLQLANSTIIAFTSDH GWALGEHGEW AKYSNFDVAT HVPLIFYVPG RTASLPEAGE KLFPYLDPFDSASQLMEPGR QSMDLVELVS LFPTLAGLAG LQVPPRCPVP SFHVELCREGKNLLKHFRFR DLEEDPYLPG NPRELIAYSQ YPRPSDIPQW NSDKPSLKDI KIMGYSIRTI DYRYTVWVGFNPDEFLANFS DIHAGELYFV DSDPLQDHNM YNDSQGGDLF QLLMPLEHHH HHH |
Iduronate 2-Sulfatase also known as IDS,belongs to the highly-conserved sulfatase family of enzymes which catalyze thehydrolysis of O-sulfate and N-salfate esters from a variety of substrates. IDS isessential for the lysosomal degradation of the glycosaminoglycans (GAG) heparansulfate as well as dermatan sulfate. Furthermore, IDS hydrolyzes the 2-sulfategroup of the IDS units of the GAG.
IDS Human Recombinant produced in Sf9 Baculovirus cells is a single, glycosylated polypeptide chain containing 533 amino acids (26-550a.a) and having a molecular mass of 60.3kDa. (Molecular size on SDS-PAGE will appear at approximately 35-70kDa). IDS is fused to an 8 amino acid His-tag at C-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Hydrolase; EC 3.1.6.13; Glycan Metabolism - glycosaminoglycan degradation Chromosomal Location of Human Ortholog: Xq28 Cellular Component: lysosomal lumen Molecular Function:metal ion binding; iduronate-2-sulfatase activity Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; pathogenesis Disease: Mucopolysaccharidosis, Type Ii |
| NCBI Summary: | The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in 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. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013] |
| UniProt Code: | P22304 |
| NCBI GenInfo Identifier: | 124174 |
| NCBI Gene ID: | 3423 |
| NCBI Accession: | P22304.1 |
| UniProt Secondary Accession: | P22304,Q14604, Q9BRM3, D3DWT4, |
| UniProt Related Accession: | P22304 |
| Molecular Weight: | 34,893 Da |
| NCBI Full Name: | Iduronate 2-sulfatase |
| NCBI Synonym Full Names: | iduronate 2-sulfatase |
| NCBI Official Symbol: | IDS�� |
| NCBI Official Synonym Symbols: | MPS2; SIDS�� |
| NCBI Protein Information: | iduronate 2-sulfatase; idursulfase; iduronate 2-sulfatase 14 kDa chain; iduronate 2-sulfatase 42 kDa chain; alpha-L-iduronate sulfate sulfatase |
| UniProt Protein Name: | Iduronate 2-sulfatase |
| UniProt Synonym Protein Names: | Alpha-L-iduronate sulfate sulfatase; Idursulfase |
| Protein Family: | Iduronate 2-sulfatase |
| UniProt Gene Name: | IDS�� |
| UniProt Entry Name: | IDS_HUMAN |
