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Product Name
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Hexosaminidase B Beta (HEXb), Recombinant Protein ★Popular Item★
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Also Known As
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Recombinant Hexosaminidase B Beta (HEXb)
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Product Gene Name
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[Similar Products]
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Research Use Only
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For Research Use Only. Not for use in diagnostic procedures.
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Sequence
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The target protein is fused with N-terminal His-Tag, its sequence is listed below. MGHHHHHHSG SEF-KLDSFG PINPTLNTTY SFLTTFFKEI SEVFPDQFIH LGGDEVEFKC WESNPKIQDF MRQKGFGTDF KKLESFYIQK VLDIIATINK GSIVWQEVFD DKAKLAPGTI VEVWKDSAYP EELSRVTASG FPVILSAPWY LDLISY
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Chromosome Location
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Chromosome: 5; NC_000005.9 (73935547..74017113). Location: 5q13
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OMIM
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268800
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3D Structure
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ModBase 3D Structure for P07686
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Host
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E Coli
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Purity/Purification
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> 95%
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Form/Format
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Supplied as lyophilized form in 20mM Tris, 500mM NaCl, pH8.0, containing 1mM EDTA, 1mM DTT, 0.01% sarcosyl, 5% trehalose, and preservative.
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Organism
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Homo sapiens (Human)
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Expression System
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Prokaryotic expression
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Residues
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Lys315~Tyr456 (Accession # P07686) with N-terminal His-Tag
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Predicted isoelectric point
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5.4
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Endotoxin Level
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<1.0EU per 1ug (determined by the LAL method)
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Reconstitution
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Reconstitute in sterile PBS, pH7.2-pH7.4.
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Preparation and Storage
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Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.
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Other Notes
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Small volumes of HEXb recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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About the Marker: Effective Size Range: 10kDa to 70kDa. Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa. Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy. Ready-to-use: No need to heat, dilute or add reducing agents before use.
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Applications Tested/Suitable for HEXb recombinant protein
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SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
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SDS-PAGE of HEXb recombinant protein
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NCBI/Uniprot data below describe general gene information for HEXb. It may not necessarily be applicable to this product. |
NCBI GI #
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4504373
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NCBI GeneID
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3074
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NCBI Accession #
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NP_000512.1
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[Other Products]
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NCBI GenBank Nucleotide #
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NM_000521.3
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[Other Products]
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UniProt Primary Accession #
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P07686
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[Other Products]
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UniProt Related Accession #
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P07686
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[Other Products]
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Molecular Weight
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17.8kDa
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NCBI Official Full Name
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beta-hexosaminidase subunit beta preproprotein
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NCBI Official Synonym Full Names
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hexosaminidase B (beta polypeptide)
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NCBI Official Symbol
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HEXB
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[Similar Products]
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NCBI Official Synonym Symbols
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ENC-1AS; HEL-248
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[Similar Products]
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NCBI Protein Information
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beta-hexosaminidase subunit beta; HCC-7; hexosaminidase subunit B; epididymis luminal protein 248; beta-N-acetylhexosaminidase subunit beta; cervical cancer proto-oncogene 7 protein; N-acetyl-beta-glucosaminidase subunit beta
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UniProt Protein Name
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Beta-hexosaminidase subunit beta
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UniProt Synonym Protein Names
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Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta
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Protein Family
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Beta-hexosaminidase
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UniProt Gene Name
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HEXB
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[Similar Products]
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UniProt Synonym Gene Names
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Hexosaminidase subunit B; HCC-7
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[Similar Products]
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UniProt Entry Name
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HEXB_HUMAN
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NCBI Summary for HEXb
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Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014]
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UniProt Comments for HEXb
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HEXB: Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2); also known as Sandhoff disease. GM2- gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2- gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula. Belongs to the glycosyl hydrolase 20 family.
Protein type: Glycan Metabolism - other glycan degradation; EC 3.2.1.52; Hydrolase; Carbohydrate Metabolism - amino sugar and nucleotide sugar; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosaminoglycan degradation
Chromosomal Location of Human Ortholog: 5q13
Cellular Component: lysosomal lumen; membrane; acrosome
Molecular Function: protein homodimerization activity; protein heterodimerization activity; beta-N-acetylhexosaminidase activity
Biological Process: oogenesis; myelination; male courtship behavior; keratan sulfate metabolic process; glycosaminoglycan metabolic process; ganglioside catabolic process; locomotory behavior; pathogenesis; hyaluronan catabolic process; regulation of cell shape; oligosaccharide catabolic process; sequestering of lipid; sensory perception of sound; chondroitin sulfate catabolic process; penetration of zona pellucida; keratan sulfate catabolic process; neuromuscular process controlling balance; skeletal development; sphingolipid metabolic process; phospholipid biosynthetic process; chondroitin sulfate metabolic process; cellular calcium ion homeostasis; cellular protein metabolic process; lysosome organization and biogenesis; carbohydrate metabolic process; positive regulation of transcription from RNA polymerase II promoter; glycosphingolipid metabolic process; hyaluronan metabolic process; astrocyte cell migration
Disease: Sandhoff Disease
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Research Articles on HEXb
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1. 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.
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Precautions
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All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
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Disclaimer
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While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with HEXb recombinant protein | Pathways associated with HEXb recombinant protein |
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Diseases associated with HEXb recombinant protein | Organs/Tissues associated with HEXb recombinant protein |
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