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  4. Human Beta-hexosaminidase subunit alpha(HEXA) ELISA kit

Human Beta-hexosaminidase subunit alpha(HEXA) ELISA kit

  • 中文名稱：
    人β氨基己糖苷酶亞基α(HEXA)酶聯免疫試劑盒
  • 貨號：
    CSB-EL010315HU
  • 規格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產品詳情

  • 產品描述：
    人β氨基己糖苷酶亞基α(HEXA)酶聯免疫試劑盒（CSB-EL010315HU）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HEXA含量。HEXA 是一個重要靶點。它相關基因編碼的蛋白質對神經系統正常功能維持很關鍵。研究機制上，聚焦于其在代謝途徑中的作用，以及與遺傳疾病的關聯，旨在通過調控 HEXA 活性，為相關神經系統疾病的治療提供新策略。試劑盒檢測范圍為15.6 pg/mL-1000 pg/mL，該產品適用于體外研究HEXA在疾病模型中的表達動態、藥物干預對酶活性的影響，以及基因突變與蛋白質功能關聯性分析等科研領域，為神經生物學、代謝疾病機制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：
    Beta hexosaminidase alpha chain precursor ELISA Kit; Beta hexosaminidase subunit alpha ELISA Kit; Beta N acetylhexosaminidase ELISA Kit; Beta N acetylhexosaminidase subunit alpha ELISA Kit; Beta-hexosaminidase A ELISA Kit; Beta-hexosaminidase subunit alpha ELISA Kit; Beta-N-acetylhexosaminidase subunit alpha ELISA Kit; Hexa ELISA Kit; HEXA_HUMAN ELISA Kit; Hexosaminidase A (alpha polypeptide) ELISA Kit; Hexosaminidase A alpha polypeptide ELISA Kit; Hexosaminidase A ELISA Kit; Hexosaminidase subunit A ELISA Kit; MGC99608 ELISA Kit; N acetyl beta glucosaminidase ELISA Kit; N acetyl beta glucosaminidase subunit alpha ELISA Kit; N-acetyl-beta-glucosaminidase subunit alpha ELISA Kit; TSD 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：
    15.6 pg/mL-1000 pg/mL
  • 靈敏度：
    3.9 pg/mL
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    Neuroscience
  • 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 HEXA 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 % 101    
    Range % 94-106    
    1:2 Average % 97    
    Range % 91-103    
    1:4 Average % 93    
    Range % 87-99    
    1:8 Average % 88    
    Range % 85-92    
  • 回收率：
    The recovery of human HEXA 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) 98 93-102    
    EDTA plasma (n=4) 87 80-92    
                   
                   
  • 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/ml OD1 OD2 Average Corrected    
    1000 2.245 2.164 2.205 2.053    
    500 1.407 1.295 1.351 1.199    
    250 0.832 0.812 0.822 0.670    
    125 0.485 0.499 0.492 0.340    
    62.5 0.334 0.316 0.325 0.173    
    31.2 0.231 0.226 0.229 0.077    
    15.6 0.192 0.199 0.196 0.044    
    0 0.152 0.151 0.152 　    
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

靶點詳情

  • 功能：
    Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides. The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.
  • 基因功能參考文獻：
    1. Reported data present, for the first time, reference values for urinary activities of HEX and its isoenzymes HEX A and HEX B in children and adolescent. PMID: 28846871
    2. The alpha mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type alpha. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA beta chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum to the cytosol and are degraded by the ... PMID: 27682588
    3. reports a new missense mutation in the HEXA gene in two German siblings with late-onset Tay-Sachs disease and prominent psychiatric symptoms PMID: 25860343
    4. The silencing of the HEXA gene had a stronger immune inhibitory effect, thereby indicating a major involvement of beta-N-acetyl-hexosaminidase A isoenzyme within this mechanism. PMID: 21997228
    5. Human prostate cancer cells are characterised by a significant decrease in HexA activity. PMID: 24389457
    6. DNA reveals novel mutations in Iranian subjects causing Tay-Sachs disease in the alpha and beta subunits of HexA. PMID: 24518553
    7. GM2 gangliosidosis is caused by the gene mutation. (review) PMID: 23370522
    8. Identification of six novel missense mutations in children affected with Tay Sachs disease from India. PMID: 22723944
    9. identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and attempted to correlate these mutations with the clinical presentation of the patients PMID: 22789865
    10. MtsD, MtsF and MtsH are fusion proteins with a methyltransferase domain and a corrinoid-binding domain. PMID: 19732345
    11. HEXA gene in Argentinean patients affected with Tay-Sachs disease, overall 14 different mutations were identified, 8 of them were novel and lead to premature stop codons, drastic residues changes or a splicing defect. PMID: 22441121
    12. Beta-hexosaminidase over-expression affects lysosomal glycohydrolases expression and glycosphingolipid metabolism in mammalian cells. PMID: 22147196
    13. We report the first Jordanian Arab Tay-Sachs disease patient diagnosed by deficient beta-hexosaminidase A activity, mutation analysis revealed homozygosity for a nonsense HEXA mutation, c.78G>A (p.W26X) PMID: 21967858
    14. Two mutations were identified c.1A>G (p.MIV), which obliterated the initiating methionine in codon 1, and c.1177C>T (p.R393X), which predicted a termination codon or nonsense mutation in infantile Tay-Sachs diseae in the Persian population. PMID: 21796138
    15. Down-regulation of beta-N-acetyl-D-glucosaminidase increases Akt1 activity in thyroid anaplastic cancer cells PMID: 21637923
    16. Detected mutations in the HEXA gene through gene sequencing and, by combining the HEXA enzyme assay and the HEXA gene sequencing assay, were able to clarify Tay Sachs carrier status. PMID: 19858779
    17. Eight novel mutations PMID: 12180151
    18. plasma activity of total Hex does not appear to be a reliable marker of erosion and cartilage degradation in rheumatoid arthritis patients; liver function appears to be the major determinant for the plasma Hex activity in these patients PMID: 12413610
    19. second novel HEXA mutation specific to the IJ TDS carriers: a substitution of cytosine 1351 by guanosine (C1351G), resulting in the change of leucine to valine in position 451. PMID: 14648242
    20. the founder effect in a rapidly expanding population arising from a bottleneck provides a robust parsimonious hypothesis explaining the spread of 1278insTATC-linked TSD in Ashkenazi Jews PMID: 14727180
    21. Therefore, our bicistronic lentivector supplying both alpha- and beta-subunits of beta-hexosaminidases may provide a potential therapeutic tool for the treatment of Sandhoff disease. PMID: 15953731
    22. analysis of the HEXA gene in Italian patients with infantile and late onset Tay-Sachs disease PMID: 16088929
    23. The plasma membrane associated beta-hexosaminidase A is fully processed, suggesting its lysosomal origin. PMID: 16212960
    24. the X-ray crystallographic structure of Hex A to 2.8 A resolution. The crystal structure of Hex A reveals an alphabeta heterodimer, with each subunit having a functional active site PMID: 16698036
    25. The highest activities of exoglycosidases were observed in high-grade gliomas, and a positive correlation of enzyme activities and degree of malignancy was noted. PMID: 16710745
    26. isoenzyme composition of N-acetyl-beta-D-hexosaminidase in seminal plasma from patients with secretory azoospermia is significantly different from controls, but this difference does not represent a useful marker of secretory azoospermia PMID: 16776631
    27. Describe strategy for detecting HexA mutations/activity in prenatal diagnosis of Tay-Sachs disease. PMID: 18425478
    28. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was larger than that in the late-onset disease group. PMID: 18490185
    29. A significantly higher activity of N-acetyl-beta-D-hexosaminidase in all laryngeal cancer specimens compared with that in healthy tissue homogenates, was observed. PMID: 19298806

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  • 相關疾病：
    GM2-gangliosidosis 1 (GM2G1)
  • 亞細胞定位：
    Lysosome.
  • 蛋白家族：
    Glycosyl hydrolase 20 family
  • 數據庫鏈接：

    HGNC: 4878

    OMIM: 272800

    KEGG: hsa:3073

    STRING: 9606.ENSP00000268097

    UniGene: Hs.604479



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