Human iduronate sulfatase,IDS ELISA Kit

Instructions
Code CSB-E09471h
Size 96T,5×96T,10×96T
Trial Size 24T ELISA kits trial application
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Product Details

Target Name iduronate 2-sulfatase
Alternative Names Alpha L iduronate sulfate sulfatase ELISA Kit; Alpha-L-iduronate sulfate sulfatase ELISA Kit; AW214631 ELISA Kit; Ids ELISA Kit; IDS_HUMAN ELISA Kit; Iduronate 2 sulfatase 14 kDa chain ELISA Kit; Iduronate 2 sulfatase 42 kDa chain ELISA Kit; Iduronate 2 sulfatase ELISA Kit; Iduronate 2-sulfatase 14 kDa chain ELISA Kit; Iduronate sulfatase ELISA Kit; Idursulfase ELISA Kit; MPS2 ELISA Kit; RP23-29M4.1 ELISA Kit; SIDS ELISA Kit
Abbreviation IDS
Uniprot No. P22304
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates, cell lysates
Detection Range 31.25 pg/mL-2000 pg/mL
Sensitivity 7.81 pg/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Tags & Cell Markers
Assay Principle quantitative
Measurement Sandwich
Precision
Intra-assay Precision (Precision within an assay): CV%<8%
Three samples of known concentration were tested twenty times on one plate to assess.
Inter-assay Precision (Precision between assays): CV%<10%
Three samples of known concentration were tested in twenty assays to assess.
Linearity
To assess the linearity of the assay, samples were spiked with high concentrations of human IDS in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
SampleSerum(n=4)
1:1Average %92
Range %87-95
1:2Average %89
Range %85-94
1:4Average %95
Range %89-99
1:8Average %102
Range %98-105
Recovery
The recovery of human IDS spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
Sample TypeAverage % RecoveryRange
Serum (n=5) 9693-102
EDTA plasma (n=4)8780-92
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
pg/mlOD1OD2AverageCorrected
20002.257 2.357 2.307 2.201
10001.976 1.872 1.924 1.818
5001.532 1.578 1.555 1.449
2501.172 1.151 1.162 1.056
1250.711 0.728 0.720 0.614
62.50.426 0.434 0.430 0.324
31.250.262 0.270 0.266 0.160
00.105 0.106 0.106
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 3-5 working days

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Target Data

Function Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
Gene References into Functions
  1. IDS structure revealed by X-ray crystallography provides essential insight into multiple mechanisms by which pathogenic mutations interfere with enzyme function, and a compelling explanation for severe Hunter syndrome phenotypes. PMID: 28593992
  2. Study analyzed the genotype-phenotype relationship for 17 patients with mucopolysaccharidosis II and performed expression studies for 12 variants, nine of which have not been reported previously; speculated that very low or cell-type-specific IDS residual activity is sufficient to prevent the neuronal phenotype. PMID: 28543354
  3. Study identified 16 novel mutations in the IDS gene and revealed that the severe type of mucopolysaccharidosis type II is strongly associated with large structural alteration of the gene. PMID: 27246110
  4. Functional characterization of all the novel sequence variants identified in the study would be helpful to confirm the clinical significance and to determine the effect of these variations on the function of respective proteins (IDUA and IDS) PMID: 27146977
  5. A splicing mutation, c.709-1G>A, was detected in the proband, for which his mother was heterozygous. PMID: 28186595
  6. Extensive iduronate 2-sulfatase (Hunter syndrome) (IDS) gene deletions were identified in four mucopolysaccharidosis type II (MPSII) patients. PMID: 26762690
  7. Two new mutations were discovered: p.K236N (c.708G>C) and p.Q80K (c.238C>A) which resulted in a severe phenotype and early death of Muccopolysaccharridosis Type II patients from Bulgaria and Macedonia. PMID: 22286622
  8. p.Ser142Phe and p.Ile360Tyrfs*31 mutations caused the severe disease manifestation PMID: 24780617
  9. This study evaluated a novel mutation in the IDS gene among 8 male Hunter syndrome patients; there was a quantitative deficiency of NK and B cell with normal responses in other immune parameters. PMID: 25038527
  10. 30 novel iduronate sulfatase mutations have been identified in mucopolysaccharidosis type II Latin American patients. PMID: 24125893
  11. Identification of a splice site mutation in the IDS gene associated with mucopolysaccharidosis type II. PMID: 23867855
  12. a novel (p.R468P) and five known (p.R88C, p.D148V, p.G224A, p.Y348X, and p.R468Q) IDS mutations were shown to result in proteins with little or no IDS activity and altered protein processing, when expressed in COS7 cells PMID: 22990955
  13. A report of a novel IDS nonsense mutation resulting in mucopolysaccharidosis type II in several patients from a Chinese family. PMID: 22622771
  14. genetically analyze patients with severe Hunter syndrome that showed a total deletion of the iduronate-2-sulphatase (IDS) gene PMID: 22492741
  15. Family members with 3 generations of X-inactivation with Hunter syndrome have 1568A>G missence mutation in the IDS gene PMID: 21062272
  16. LCR-initiated rearrangements at the IDS locus, completed with Alu-mediated recombination or non-homologous end joining PMID: 21593745
  17. Hunter syndrome in Thailand is caused by a diverse set of defects affecting both IDS protein production and activity. PMID: 18500569
  18. study describes a woman with mild manifestations of Hunter syndrome who gave birth to a daughter; both the mother and daughter carried the p.R443X mutation in exon 9 of the ID2S gene PMID: 21108396
  19. The in vivo correction of heritable gene lesions at the RNA level operating via a correction mechanism akin to RNA-editing, was observed for IDS mutant transcript. PMID: 20104590
  20. The results illustrated that the deletion and frame-shift mutation of c.876-877 del TC detected in IDS gene was a novel pathologic mutation,, which was the underlying cause of MPS II of this patient. PMID: 19933090
  21. patterns of cytosine methylation in the entire IDS gene PMID: 15146464
  22. Mucopolysaccharidosis type II patients with sever CNS involvement and age of onset by 3 years of age had four IDS amino acid substitutions S333L,C53X,E341K, and P480R. PMID: 15500445
  23. a total of 17 identified missense, small deletion, and nonsense mutations were further characterized by transient expression studies. PMID: 15614569
  24. large deletion correlated with the severe phenotype of this Hunter syndrome patient. PMID: 15909065
  25. The IDS gene was analyzed in Japanese patients with mucopolysaccharidosis II. PMID: 16133661
  26. IDS activity in female carriers was less than a half of the normal level PMID: 16480701
  27. These findings suggest methylation patterns in the beginning of IDS genomic region are polymorphic in humans and that hypermethylation in this region in some individuals predisposes them to CpG mutations resulting in Hunter syndrome. PMID: 16617305
  28. The balance between constitutive and cryptic splice sites in the IDS gene is very sensitive. PMID: 16699754
  29. A new point mutation (T1140C) in exon 8 of the IDS gene was found in Hunter syndrome. PMID: 16735228
  30. the IDS gene is prone to splicing mutations in Portuguese patients with mucopolysaccharidosis type II PMID: 17063374
  31. analysis of iduronate-2-sulfatase enzymatic activity, protein processing and structure PMID: 17091340
  32. Two novel mutations were identified in the human iduronate-2-sulfatase (IDS) gene in two patients from unrelated families with mucopolysaccharidosis type II(MPS II). PMID: 17284421
  33. Identification of a novel nonsense mutation (p.Y54X) in the IDS gene of severely affected MPS II patients of African origin. PMID: 17616540
  34. The molecular characterization of one novel missense mutation (p.S305P) and 1 splice site mutation (c.1006 +5G > C) associated with mucopolysaccharidosis type II was presented. PMID: 17655837
  35. frame-shift deletion mutation (1062 del 16) was identified in exon 7 of the patient's IDS gene PMID: 17657858
  36. A new mutation, an A>T change at nucleotide 595, substitutes a premature stop codon for a lysine at amino acid 199 of the IDS enzyme. PMID: 18546295
  37. IDS has a role in glucose-stimulated insulin secretion via a mechanism that involves the activation of exocytosis through phosphorylation of PKCalpha and MARCKS. PMID: 19602578
  38. novel mutations in Italian patients with mucopolysaccharidosis type II PMID: 11462244

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Involvement in disease Mucopolysaccharidosis 2 (MPS2)
Subcellular Location Lysosome
Protein Families Sulfatase family
Tissue Specificity Liver, kidney, lung, and placenta.
Database Links

HGNC: 5389

OMIM: 300823

KEGG: hsa:3423

STRING: 9606.ENSP00000339801

UniGene: Hs.460960

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