HEXA Antibody, Biotin conjugated
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中文名稱：HEXA兔多克隆抗體, Biotin偶聯
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貨號：CSB-PA12259D0Rb
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) HEXA Polyclonal antibody
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Uniprot No.：
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基因名：
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別名：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
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Beta-hexosaminidase subunit alpha protein (89-529AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Biotin
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
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靶點詳情
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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.
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基因功能參考文獻：
- 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
- 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
- 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
- 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
- Human prostate cancer cells are characterised by a significant decrease in HexA activity. PMID: 24389457
- DNA reveals novel mutations in Iranian subjects causing Tay-Sachs disease in the alpha and beta subunits of HexA. PMID: 24518553
- GM2 gangliosidosis is caused by the gene mutation. (review) PMID: 23370522
- Identification of six novel missense mutations in children affected with Tay Sachs disease from India. PMID: 22723944
- 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
- MtsD, MtsF and MtsH are fusion proteins with a methyltransferase domain and a corrinoid-binding domain. PMID: 19732345
- 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
- Beta-hexosaminidase over-expression affects lysosomal glycohydrolases expression and glycosphingolipid metabolism in mammalian cells. PMID: 22147196
- 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
- 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
- Down-regulation of beta-N-acetyl-D-glucosaminidase increases Akt1 activity in thyroid anaplastic cancer cells PMID: 21637923
- 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
- Eight novel mutations PMID: 12180151
- 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
- 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
- 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
- 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
- analysis of the HEXA gene in Italian patients with infantile and late onset Tay-Sachs disease PMID: 16088929
- The plasma membrane associated beta-hexosaminidase A is fully processed, suggesting its lysosomal origin. PMID: 16212960
- 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
- 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
- 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
- Describe strategy for detecting HexA mutations/activity in prenatal diagnosis of Tay-Sachs disease. PMID: 18425478
- 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
- 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)
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亞細胞定位：Lysosome.
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蛋白家族：Glycosyl hydrolase 20 family
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