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HEXb recombinant protein :: Hexosaminidase B Beta (HEXb) Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS2009814 (SPECIAL PROMOTION: Get FREE $5 Starbucks gift card (Maximum 5 gift cards/product). Gift card(s) will be included with shipment)
SDS-PAGE
Unit / Price
0.01 mg  /  $195 +1 FREE 8GB USB
0.05 mg  /  $420 +1 FREE 8GB USB
0.1 mg  /  $645 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Hexosaminidase B Beta (HEXb), Recombinant Protein

 Also Known As   

Recombinant Hexosaminidase B Beta (HEXb)

 Product Gene Name   

HEXb recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    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
 Chromosome Location    Chromosome: 5; NC_000005.9 (73935547..74017113). Location: 5q13
 OMIM    268800
 3D Structure    ModBase 3D Structure for P07686
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 Host    E Coli
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 Purity/Purification    > 95%
 Form/Format    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    Homo sapiens (Human)
 Expression System    Prokaryotic expression
 Residues    Lys315~Tyr456 (Accession # P07686) with N-terminal His-Tag
 Predicted isoelectric point    5.4
 Endotoxin Level    <1.0EU per 1ug (determined by the LAL method)
 Reconstitution    Reconstitute in sterile PBS, pH7.2-pH7.4.
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 Preparation and Storage    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.
 Other Notes    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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Product Description specifically for HEXb recombinant protein

   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.
 Applications Tested/Suitable for HEXb recombinant protein   

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

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 SDS-PAGE of HEXb recombinant protein    HEXb recombinant protein SDS-PAGE image
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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 #    4504373
 NCBI GeneID    3074
 NCBI Accession #    NP_000512.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000521.3 [Other Products]
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 UniProt Primary Accession #    P07686 [Other Products]
 UniProt Related Accession #    P07686 [Other Products]
 Molecular Weight    17.8kDa
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 NCBI Official Full Name    beta-hexosaminidase subunit beta preproprotein
 NCBI Official Synonym Full Names    hexosaminidase B (beta polypeptide)
 NCBI Official Symbol    HEXB [Similar Products]
 NCBI Official Synonym Symbols   
ENC-1AS; HEL-248
[Similar Products]
 NCBI Protein Information    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
 UniProt Protein Name    Beta-hexosaminidase subunit beta
 UniProt Synonym Protein Names   
Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta
 Protein Family    Beta-hexosaminidase
 UniProt Gene Name    HEXB [Similar Products]
 UniProt Synonym Gene Names    Hexosaminidase subunit B; HCC-7 [Similar Products]
 UniProt Entry Name    HEXB_HUMAN
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 NCBI Summary for HEXb    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    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    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    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.
 Disclaimer    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 proteinPathways associated with HEXb recombinant protein
 Reference Product  PubMed Publications
 HEXA recombinant protein  >79 publications with HEXb and HEXA
 GM2A recombinant protein  >9 publications with HEXb and GM2A
 GLB1 recombinant protein  >2 publications with HEXb and GLB1
 GLA recombinant protein  >1 publications with HEXb and GLA
 NAGA recombinant protein  >1 publications with HEXb and NAGA
 Products by Pathway  Pathway Diagram
 Amino Sugar And Nucleotide Sugar Metabolism Pathway antibodies  Amino Sugar And Nucleotide Sugar Metabolism Pathway Diagram
 Amino Sugar And Nucleotide Sugar Metabolism Pathway antibodies  Amino Sugar And Nucleotide Sugar Metabolism Pathway Diagram
 CS/DS Degradation Pathway antibodies  CS/DS Degradation Pathway Diagram
 Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway antibodies  Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway Diagram
 Disease Pathway antibodies  Disease Pathway Diagram
 Glycosaminoglycan Degradation Pathway antibodies  Glycosaminoglycan Degradation Pathway Diagram
 Glycosaminoglycan Degradation Pathway antibodies  Glycosaminoglycan Degradation Pathway Diagram
 Glycosaminoglycan Metabolism Pathway antibodies  Glycosaminoglycan Metabolism Pathway Diagram
 Glycosphingolipid Biosynthesis - Ganglio Series Pathway antibodies  Glycosphingolipid Biosynthesis - Ganglio Series Pathway Diagram
 Glycosphingolipid Biosynthesis - Ganglio Series Pathway antibodies  Glycosphingolipid Biosynthesis - Ganglio Series Pathway Diagram
Diseases associated with HEXb recombinant proteinOrgans/Tissues associated with HEXb recombinant protein
 Disease Name  Pubmed Publications
 Sandhoff Disease Antibodies  >116 publications with HEXb and Sandhoff Disease
 Brain Diseases Antibodies  >114 publications with HEXb and Brain Diseases
 Liver Diseases Antibodies  >8 publications with HEXb and Liver Diseases
 Kidney Diseases Antibodies  >7 publications with HEXb and Kidney Diseases
 Neoplasms Antibodies  >6 publications with HEXb and Neoplasms
 Inflammation Antibodies  >4 publications with HEXb and Inflammation
 Nerve Degeneration Antibodies  >2 publications with HEXb and Nerve Degeneration
 Fibrosis Antibodies  >1 publications with HEXb and Fibrosis
 Hypertrophy Antibodies  >1 publications with HEXb and Hypertrophy
 Weight Loss Antibodies  >1 publications with HEXb and Weight Loss
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >46 publications with HEXb and Brain
 Blood Antibodies  >19 publications with HEXb and Blood
 Liver Antibodies  >13 publications with HEXb and Liver
 Kidney Antibodies  >11 publications with HEXb and Kidney
 Bone Antibodies  >8 publications with HEXb and Bone
 Bone Marrow Antibodies  >5 publications with HEXb and Bone Marrow
 Testis Antibodies  >5 publications with HEXb and Testis
 Muscle Antibodies  >5 publications with HEXb and Muscle
 Ovary Antibodies  >4 publications with HEXb and Ovary
 Skin Antibodies  >3 publications with HEXb and Skin
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