Human N-sulphoglucosamine sulphohydrolase(SGSH) ELISA kit

Instructions
Code CSB-EL021200HU
Size 96T,5×96T,10×96T
Trial Size 24T ELISA kits trial application
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Product Details

Target Name N-sulfoglucosamine sulfohydrolase
Alternative Names Heparan sulfate sulfatase ELISA Kit; Heparan sulphate sulphatase ELISA Kit; HSS ELISA Kit; MPS 3A ELISA Kit; MPS3 A ELISA Kit; MPS3A ELISA Kit; Mucopolysaccharidosis type IIIA ELISA Kit; N sulfoglucosamine sulfohydrolase (sulfamidase) ELISA Kit; N sulfoglucosamine sulfohydrolase ELISA Kit; N-sulphoglucosamine sulphohydrolase ELISA Kit; SFMD ELISA Kit; SGSH ELISA Kit; SPHM_HUMAN ELISA Kit; Sulfoglucosamine sulfamidase ELISA Kit; Sulphamidase ELISA Kit; Sulphoglucosamine sulphamidase ELISA Kit
Abbreviation SGSH
Uniprot No. P51688
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 Signal Transduction
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 SGSH in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
  Sample Serum(n=4)  
1:1 Average % 92  
Range % 87-97  
1:2 Average % 97  
Range % 93-102  
1:4 Average % 84  
Range % 81-88  
1:8 Average % 89  
Range % 83-94  
Recovery
The recovery of human SGSH 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 Type Average % Recovery Range  
Serum (n=5) 101 97-106  
EDTA plasma (n=4) 92 89-95  
             
             
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
pg/ml OD1 OD2 Average Corrected  
2000 2.256 2.136 2.196 2.069  
1000 1.916 1.788 1.852 1.725  
500 1.253 1.355 1.304 1.177  
250 0.813 0.809 0.811 0.684  
125 0.467 0.505 0.486 0.359  
62.5 0.326 0.350 0.338 0.211  
31.25 0.222 0.238 0.230 0.103  
0 0.124 0.130 0.127    
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 7-14 working days

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Target Background

Function
(From Uniprot)
Catalyzes a step in lysosomal heparan sulfate degradation.
Gene References into Functions
  1. CSF enzyme activity levels for either SGSH (in MPS IIIA subjects) or NAGLU (in MPS IIIB) significantly differed from normal controls. Several other behavioral or functional measures were found to be uninformative in this population, including timed functional motor tests. PMID: 27590925
  2. We have identified ocular features of a patient with Sanfilippo syndrome type IIIA harboring a novel SGHS mutation that were not previously known to occur in this disease - namely, a progressive retinopathy with distinctive features, cystic macular changes responsive to carbonic anhydrase inhibitors, and complex electroretinographic abnormalities consistent with postreceptoral dysfunction. PMID: 26331342
  3. results demonstrate that a single systemic scAAVrh74-hSGSH delivery mediated efficient restoration of SGSH activity and resulted in a near complete correction of MPS IIIA molecular pathology PMID: 25592334
  4. The crystal structure of glycosylated sulfamidase provides insight into the diverse effects of pathogenic mutations on sulfamidase function in mucopolysaccharidosis type IIIA. PMID: 24816101
  5. Pre-symptomatic treatment of progressive neurodegenerative disease (mucopolysaccharidosis type IIIA) via intra-cerebrospinal fluid injection of recombinant human SGSH mediates highly significant reductions in neuropathology in a canine model. PMID: 21550404
  6. Processing and secretion of p.Ser298Pro sulfamidase suggests that small amounts of the newly synthesized enzyme are transported to lysosomes PMID: 21671382
  7. Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: Diagnostic, clinical, and biological implications PMID: 11668611
  8. Sanfilippo syndrome (subtypes A and B) in Turkey: identification of novel mutations in SGSH and NAGLU PMID: 11793481
  9. expression studies of four novel mutations PMID: 15146460
  10. analysis of a nonsense mutation (Y40X) and two de novo missense mutations (E300V; Q307P) in heparan N-sulphatase in a mucopolysaccharidosis IIIA patient [case report] PMID: 15902564
  11. By assessing the degree of developmental regression over time a group of 7 pts with a slowly progressive course of MPSIIIA were identified. In these 7 pts and in 3 other mildly affected pts missense mutation c.892T>C (p.Ser298Pro) was found on 1 allele PMID: 18407553

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Involvement in disease Mucopolysaccharidosis 3A (MPS3A)
Subcellular Location Lysosome
Protein Families Sulfatase family
Database Links

HGNC: 10818

OMIM: 252900

KEGG: hsa:6448

STRING: 9606.ENSP00000314606

UniGene: Hs.31074

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