Recombinant Hexosaminidase B Beta (HEXb) | MyBioSource MBS2009814 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Hexosaminidase B Beta (HEXb)

catalog :

MBS2009814

quantity :

0.01 mg

price :

195 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS2009814

products type :

Recombinant Protein

products full name :

Recombinant Hexosaminidase B Beta (HEXb)

products short name :

Hexosaminidase B Beta (HEXb)

other names :

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

products gene name :

HEXb

other gene names :

HEXB; HEXB; ENC-1AS; HEL-248; Hexosaminidase subunit B; HCC-7

uniprot entry name :

HEXB_HUMAN

host :

E Coli

sequence length :

556

sequence :

The target protein is fused with N-terminal His-Tag, its sequence is listed below. MGHHHHHHSGSEF-KLDSFG PINPTLNTTY SFLTTFFKEI SEVFPDQFIH LGGDEVEFKC WESNPKIQDF MRQKGFGTDF KKLESFYIQK VLDIIATINK GSIVWQEVFD DKAKLAPGTI VEVWKDSAYP EELSRVTASG FPVILSAPWY LDLISY

purity :

> 95%

form :

Supplied as lyophilized form in 20mM Tris, 500mM NaCl, pH8.0, containing 1mM EDTA, 1mM DTT, 0.01% sarcosyl, 5% trehalose, and preservative.

storage stability :

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.

tested application :

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

other info1 :

Organism: Homo sapiens (Human). Expression System: Prokaryotic expression. Residues: Lys315~Tyr456 (Accession # P07686) with N-terminal His-Tag. Predicted isoelectric point: 5.4

other info2 :

Endotoxin Level: <1.0EU per 1ug (determined by the LAL method). Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.

products description :

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.

ncbi gi num :

4504373

ncbi acc num :

NP_000512.1

ncbi gb acc num :

NM_000521.3

uniprot acc num :

P07686

ncbi mol weight :

17.8kDa

ncbi pathways :

Amino Sugar And Nucleotide Sugar Metabolism Pathway (82979); Amino Sugar And Nucleotide Sugar Metabolism Pathway (350); CS/DS Degradation Pathway (645311); Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway (645308); Disease Pathway (530764); Glycosaminoglycan Degradation Pathway (82981); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (645297); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (82997); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (372)

ncbi summary :

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]

uniprot summary :

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

size1 :

0.01 mg

price1 :

195 USD

size2 :

0.05 mg

price2 :

420

size3 :

0.1 mg

price3 :

645

more info or order :

MyBioSource product webpage