Product Details

Purity

Greater than 85% as determined by SDS-PAGE.

Target Names

HEXB

Uniprot No.

P07686

Research Area

Metabolism

Alternative Names

Beta-N-acetylhexosaminidase subunit beta (Hexosaminidase subunit B) (Cervical cancer proto-oncogene 7 protein) (HCC-7) (N-acetyl-beta-glucosaminidase subunit beta)

Species

Homo sapiens (Human)

Source

E.coli

Expression Region

122-556aa

Target Protein Sequence

TQVQQLLVSITLQSECDAFPNISSDESYTLLVKEPVAVLKANRVWGALRGLETFSQLVYQDSYGTFTINESTIIDSPRFSHRGILIDTSRHYLPVKIILKTLDAMAFNKFNVLHWHIVDDQSFPYQSITFPELSNKGSYSLSHVYTPNDVRMVIEYARLRGIRVLPEFDTPGHTLSWGKGQKDLLTPCYSRQNKLDSFGPINPTLNTTYSFLTTFFKEISEVFPDQFIHLGGDEVEFKCWESNPKIQDFMRQKGFGTDFKKLESFYIQKVLDIIATINKGSIVWQEVFDDKAKLAPGTIVEVWKDSAYPEELSRVTASGFPVILSAPWYLDLISYGQDWRKYYKVEPLDFGGTQKQKQLFIGGEACLWGEYVDATNLTPRLWPRASAVGERLWSSKDVRDMDDAYDRLTRHRCRMVERGIAAQPLYAGYCNHENM
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.

Mol. Weight

57.4 kDa

Protein Length

Full Length of Mature Protein

Tag Info

N-terminal 10xHis-tagged and C-terminal Myc-tagged

Form

Liquid or Lyophilized powder
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.

Buffer

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

3-7 business days

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet & COA

Please contact us to get it.

Description

Explore the intricacies of metabolism with our high-quality Recombinant Human HEXB protein. Beta-hexosaminidase subunit beta, a crucial component of the lysosomal enzyme β-hexosaminidase, is involved in the degradation of GM2 gangliosides and other molecules containing terminal N-acetylhexosamines. It plays an essential role in the metabolism of glycoconjugates, making it an important protein for research in metabolic pathways and related disorders.

Our Recombinant Human HEXB protein is expressed in E. coli and encompasses the full length of the mature protein, covering the 122-556aa region, thus retaining the key functional domains. The N-terminal 10xHis-tag and C-terminal Myc-tag ensure efficient protein purification and detection, streamlining your experimental procedures. With a purity greater than 85% as determined by SDS-PAGE, our Recombinant Human HEXB protein is supplied as a lyophilized powder, providing you with a reliable and consistent product for your metabolic research endeavors.

Target Background

Function

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

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Involvement in disease

GM2-gangliosidosis 2 (GM2G2)

Subcellular Location

Lysosome. Cytoplasmic vesicle, secretory vesicle, Cortical granule.

Protein Families

Glycosyl hydrolase 20 family

Database Links

HGNC: 4879

OMIM: 268800

KEGG: hsa:3074

STRING: 9606.ENSP00000261416

UniGene: Hs.69293

Code	CSB-EP010316HU
Abbreviation	Recombinant Human HEXB protein
MSDS	MSDS Datasheet
Size	$256
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Image	(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Recombinant Human Beta-hexosaminidase subunit beta (HEXB)

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