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HEXA Antibody

  • 货号：
    CSB-PA12259A0Rb
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Rat brain tissue
      All lanes: HEXA antibody at 5.8μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 61, 20 kDa
      Observed band size: 61 kDa
    • IHC image of CSB-PA12259A0Rb diluted at 1:300 and staining in paraffin-embedded human endometrial cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of PC-3 cells with CSB-PA12259A0Rb at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) HEXA Polyclonal antibody
  • Uniprot No.：
    P06865
  • 基因名：
  • 别名：
    Beta hexosaminidase alpha chain precursor antibody; Beta hexosaminidase subunit alpha antibody; Beta N acetylhexosaminidase antibody; Beta N acetylhexosaminidase subunit alpha antibody; Beta-hexosaminidase A antibody; Beta-hexosaminidase subunit alpha antibody; Beta-N-acetylhexosaminidase subunit alpha antibody; Hexa antibody; HEXA_HUMAN antibody; Hexosaminidase A (alpha polypeptide) antibody; Hexosaminidase A alpha polypeptide antibody; Hexosaminidase A antibody; Hexosaminidase subunit A antibody; MGC99608 antibody; N acetyl beta glucosaminidase antibody; N acetyl beta glucosaminidase subunit alpha antibody; N-acetyl-beta-glucosaminidase subunit alpha antibody; TSD antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Rat
  • 免疫原：
    Recombinant Human Beta-hexosaminidase subunit alpha protein (89-529AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，HEXA Antibody (CSB-PA12259A0Rb)，的标记方式是Non-conjugated。对于HEXA Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA12259B0Rb HEXA Antibody, HRP conjugated ELISA
    FITC CSB-PA12259C0Rb HEXA Antibody, FITC conjugated
    Biotin CSB-PA12259D0Rb HEXA Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    GM2-gangliosidosis 1 (GM2G1)
  • 亚细胞定位：
    Lysosome.
  • 蛋白家族：
    Glycosyl hydrolase 20 family
  • 数据库链接：

    HGNC: 4878

    OMIM: 272800

    KEGG: hsa:3073

    STRING: 9606.ENSP00000268097

    UniGene: Hs.604479