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  4. Human N-sulphoglucosamine sulphohydrolase(SGSH) ELISA kit

Human N-sulphoglucosamine sulphohydrolase(SGSH) ELISA kit

  • 中文名稱：
    人磺氨基葡糖硫酸胺酶(SGSH)酶聯免疫試劑盒
  • 貨號：
    CSB-EL021200HU
  • 規格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 促銷：
  • 其他：

產品詳情

  • 產品描述：
    人磺氨基葡糖硫酸胺酶(SGSH)酶聯免疫試劑盒（CSB-EL021200HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿、細胞裂解物樣本中的SGSH含量。SGSH即N - 磺基葡糖胺磺基水解酶，它參與硫酸乙酰肝素的降解。若SGSH功能缺陷，會使硫酸乙酰肝素在體內蓄積，引發黏多糖貯積癥ⅢA型。目前對其研究多聚焦于酶替代療法等改善病情的機制，以緩解患者癥狀。試劑盒檢測范圍為31.25 pg/mL-2000 pg/mL，適用于科研領域中對SGSH表達調控機制的研究，例如在疾病模型構建、基因編輯細胞系驗證或溶酶體功能相關分子通路探索中定量分析SGSH的動態變化，為神經退行性疾病、溶酶體貯積癥等基礎研究提供靈敏可靠的工具。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
  • 別名：
    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
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates, cell lysates
  • 檢測范圍：
    31.25 pg/mL-2000 pg/mL
  • 靈敏度：
    7.81 pg/mL
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    Signal Transduction
  • 測定原理：
    quantitative
  • 測定方法：
    Sandwich
  • 精密度：
    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.
  • 線性度：
    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.
    SampleSerum(n=4)
    1:1Average %92
    Range %87-97
    1:2Average %97
    Range %93-102
    1:4Average %84
    Range %81-88
    1:8Average %89
    Range %83-94
  • 回收率：
    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 TypeAverage % RecoveryRange
    Serum (n=5) 10197-106
    EDTA plasma (n=4)9289-95
  • 標準曲線：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    pg/mlOD1OD2AverageCorrected
    20002.256 2.136 2.196 2.069
    10001.916 1.788 1.852 1.725
    5001.253 1.355 1.304 1.177
    2500.813 0.809 0.811 0.684
    1250.467 0.505 0.486 0.359
    62.50.326 0.350 0.338 0.211
    31.250.222 0.238 0.230 0.103
    00.124 0.130 0.127
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

靶點詳情

  • 功能：
    Catalyzes a step in lysosomal heparan sulfate degradation.
  • 基因功能參考文獻：
    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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  • 相關疾病：
    Mucopolysaccharidosis 3A (MPS3A)
  • 亞細胞定位：
    Lysosome.
  • 蛋白家族：
    Sulfatase family
  • 數據庫鏈接：

    HGNC: 10818

    OMIM: 252900

    KEGG: hsa:6448

    STRING: 9606.ENSP00000314606

    UniGene: Hs.31074



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