HEXB Antibody, FITC conjugated

說明書

中文名稱：

HEXB兔多克隆抗體, FITC偶聯
貨號：

CSB-PA010316LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) HEXB Polyclonal antibody
Uniprot No.：

P07686
基因名：

HEXB
別名：

Beta hexosaminidase beta chain antibody; Beta hexosaminidase subunit beta antibody; Beta N acetylhexosaminidase antibody; Beta-hexosaminidase subunit beta chain A antibody; Beta-N-acetylhexosaminidase subunit beta antibody; Cervical cancer proto oncogene 7 protein antibody; Cervical cancer proto-oncogene 7 protein antibody; ENC 1AS antibody; Epididymis luminal protein 248 antibody; HCC 7 antibody; HCC-7 antibody; HCC7 antibody; HEL 248 antibody; HEX B antibody; Hexb antibody; HEXB_HUMAN antibody; Hexosaminidase B (beta polypeptide) antibody; Hexosaminidase B antibody; Hexosaminidase subunit B antibody; HexosaminidaseB antibody; N acetyl beta glucosaminidase antibody; N-acetyl-beta-glucosaminidase subunit beta antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Beta-hexosaminidase subunit beta protein (408-528AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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 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. During fertilization is responsible, at least in part, for the zona block to polyspermy. Present in the cortical granules of non-activated oocytes, is exocytosed during the cortical reaction in response to oocyte activation and inactivates the sperm galactosyltransferase-binding site, accounting for the block in sperm binding to the zona pellucida.
基因功能參考文獻：
1. a modified human hexosaminidase subunit beta (HexB), which we have termed mod2B, composed of homodimeric beta subunits that contain amino acid sequences from the alpha subunit that confer GM2 ganglioside-degrading activity and protease resistance. PMID: 27018595
2. Mutations of the HEXB gene is associated with maple syrup urine disease or Sandhoff disease. PMID: 27682710
3. report on the heterogeneity of the mutational spectrum of the HEXB gene in Indian patients with Sandhoff disease PMID: 26582265
4. The absence of beta-N-acetyl-hexosaminidase activity does not alter the differentiation of i-DCs from HSCs, but it is critical for the activation of CD4(+)T cells because knock-down of HEXA or HEXB gene causes a loss of function of i-DCs. PMID: 21997228
5. Concentration and specific activity of N-acetyl-B-hexosaminidase in palatine tonsils in patients with tonsillar hypertrophy and chronic tonsillitis both in childhood and adulthood significantly increase in comparison to healthy individuals. PMID: 23911049
6. A total of 19 HEXB variants were found in the 1092 genomes of which 5 are suspected of having a deleterious effect on hexosaminidase activity. PMID: 24461908
7. DNA from Iranian Tay-Sachs patients reveals a novel mutation in HEXB predicting a termination codon or nonsense mutation. PMID: 24518553
8. A patient with Sandhoff disease also is found to have a compound macro-deletion in HEXB. PMID: 23886397
9. A highly significant correlation of HEX-7 and %CDT has been found. Because of exclusion of the P isoform, HEX-7 could be a useful supplementary marker for detecting chronic alcohol abuse. PMID: 23906468
10. Expression of beta-hexosaminidase in the neurons of Sandhoff disease patients rescues transgenic mice from neurodegeneration. PMID: 22863301
11. minigene studies revealed the presence of a novel alternative spliced HEXB mRNA variant also present in normal cells PMID: 22848519
12. Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients. PMID: 23046579
13. We describe a novel HEXB mutation that is shared among 4 patients with Sandhoff disease. PMID: 22191674
14. Plasma beta-hexosaminidase and beta-galactosidase) levels are higher in patients with Alzheimer's disease-type 2 diabetes mellitus (T2DM) compared to those with T2DM alone. PMID: 21321400
15. The X-ray crystal structure of beta-hexosaminidase B provides new insights into mutations that cause Sandhoff disease. PMID: 12706724
16. novel c.1556A>G transition in exon 12 of the HEXB gene associated with chronic Sandhoff's disease PMID: 17251047
17. Beta-hexosaminidase is a peptidoglycan hydrolase that surprisingly exerts its mycobactericidal effect at the macrophage plasma membrane during mycobacteria-induced secretion of lysosomes PMID: 18180457
18. Elevated activity of beta-hexosaminidase observed in subjects with asthma suggests that the beta-hex isozyme could take part in airway inflammation and remodeling in asthma. PMID: 18204279
19. Lysosome-related genes, such as CLN2, CLN3, and HEXB, may be involved in the pathogenesis of adipose tissue hypertrophy in TED. PMID: 18552385
20. These results reveal a new aspect of beta-hexosaminidase biology and suggest that a fully processed membrane-associated form of Hex is translocated from the lysosomal membrane to the PM by an as yet unknown mechanism. W PMID: 18588514
21. Results describe the molecular genetics of Sandhoff disease in Italy and provide new insights into the molecular basis of the disease through HEXB mutation. PMID: 18758829
22. A new D459A missense HEXB mutation was discovered in six juvenile patients with Sandhoff disease. PMID: 18930675
23. Gene therapy reduced GM(2) storage and ameliorated neuroinflammation in the brain of HexB(-/-) mice, as well as attenuated behavioral deficits. PMID: 19278737
24. Data suggest that cigarette smoking can inhibit, by the influence on N-acetyl-beta-hexosaminidase activity, catabolism of oligosaccharide chains in cancer tissues. PMID: 19615986
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相關疾病：

GM2-gangliosidosis 2 (GM2G2)
亞細胞定位：

Lysosome. Cytoplasmic vesicle, secretory vesicle, Cortical granule.
蛋白家族：

Glycosyl hydrolase 20 family
數據庫鏈接：

HGNC: 4879

OMIM: 268800

KEGG: hsa:3074

STRING: 9606.ENSP00000261416

UniGene: Hs.69293

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