HEXB Antibody

Code: CSB-PA009172

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50μg100μg

US$119

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Species Reactivity: Human

Raised in: Rabbit

Application: WB, IHC, IF, ELISA

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Product Details
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Target Background

Product Details

Uniprot NO.

P07686

Target Names

HEXB

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Synthesized peptide derived from the C-terminal region of Human Hexb.

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Isotype

IgG

Purification Method

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Form

Liquid
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Tested Applications

WB, IHC, IF, ELISA

Protocols

Western Blotting(WB) Protocol Immunohistochemistry (IHC) Protocol Immunofluorescence (IF) Protocol ELISA Protocol

Storage

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

Lead Time

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.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Usage

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

Datasheet & COA

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Target Background

Function(From Uniprot)

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.

Gene References into Functions

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
Mutations of the HEXB gene is associated with maple syrup urine disease or Sandhoff disease. PMID:27682710
report on the heterogeneity of the mutational spectrum of the HEXB gene in Indian patients with Sandhoff disease PMID:26582265
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
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
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
DNA from Iranian Tay-Sachs patients reveals a novel mutation in HEXB predicting a termination codon or nonsense mutation. PMID:24518553
A patient with Sandhoff disease also is found to have a compound macro-deletion in HEXB. PMID:23886397
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
Expression of beta-hexosaminidase in the neurons of Sandhoff disease patients rescues transgenic mice from neurodegeneration. PMID:22863301
minigene studies revealed the presence of a novel alternative spliced HEXB mRNA variant also present in normal cells PMID:22848519
Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients. PMID:23046579
We describe a novel HEXB mutation that is shared among 4 patients with Sandhoff disease. PMID:22191674
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
The X-ray crystal structure of beta-hexosaminidase B provides new insights into mutations that cause Sandhoff disease. PMID:12706724
novel c.1556A>G transition in exon 12 of the HEXB gene associated with chronic Sandhoff's disease PMID:17251047
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
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
Lysosome-related genes, such as CLN2, CLN3, and HEXB, may be involved in the pathogenesis of adipose tissue hypertrophy in TED. PMID:18552385
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
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
A new D459A missense HEXB mutation was discovered in six juvenile patients with Sandhoff disease. PMID:18930675
Gene therapy reduced GM(2) storage and ameliorated neuroinflammation in the brain of HexB(-/-) mice, as well as attenuated behavioral deficits. PMID:19278737
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