Polyclonal Antibody to Hexosaminidase B Beta (HEXb) | MyBioSource MBS2001521 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Polyclonal Antibody to Hexosaminidase B Beta (HEXb)

catalog :

MBS2001521

quantity :

0.01 mg

price :

105 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot: Sample: Recombinant protein.

image 2 :

DAB staining on IHC-P; Samples: Human Stomach Tissue)

product information

catalog number :

MBS2001521

products type :

Antibody

products full name :

Polyclonal Antibody to Hexosaminidase B Beta (HEXb)

products short name :

[Hexosaminidase B Beta (HEXb)]

other names :

[beta-hexosaminidase subunit beta isoform 1 preproprotein; Beta-hexosaminidase subunit beta; beta-hexosaminidase subunit beta; hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B]

products gene name :

[HEXb]

other gene names :

[HEXB; HEXB; ENC-1AS; HEL-248; HEL-S-111; Hexosaminidase subunit B; HCC-7]

clonality :

Polyclonal

host :

Rabbit

purity :

Antigen-specific affinity chromatography followed by Protein A affinity chromatography

form :

Supplied a ssolution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.

concentration :

1 mg/ml

storage stability :

Storage: Avoid repeated freeze/thaw cycles. Store at 4ºC for frequent use. Aliquot and store at -20ºC for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

tested application :

WB; IHC; ICC; IP.

app notes :

Western blotting: 0.5-2 Ug/mL;1:500-2000. Immunohistochemistry: 5-20 Ug/mL;1:50-200. Immunocytochemistry: 5-20 Ug/mL;1:50-200. Optimal working dilutions must be determined by end user.

image1 heading :

Western Blot (WB)

image2 heading :

Immunohistochemistry (IHC)

other info1 :

Organism Species: Homo sapiens (Human). Source: Polyclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant HEXb (Lys315~Tyr456 (Accession # P07686)) expressed in E.coli. Immunogen: Recombinant HEXb (Lys315-Tyr456)(Accesssion#P07686)expressed in E. coli

other info2 :

Conjugated Antibody: The APC conjugated antibody version of this item is also available as catalog #MBS2045364

ncbi gi num :

4504373

ncbi acc num :

NP_000512.1

ncbi gb acc num :

NM_000521.3

uniprot acc num :

P07686

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: Carbohydrate Metabolism - amino sugar and nucleotide sugar; EC 3.2.1.52; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - other glycan degradation; Hydrolase. Chromosomal Location of Human Ortholog: 5q13.3. Cellular Component: acrosomal vesicle; azurophil granule; extracellular region; lysosomal lumen; membrane. Molecular Function: acetylglucosaminyltransferase activity; beta-N-acetylhexosaminidase activity; protein heterodimerization activity; protein homodimerization activity. Biological Process: astrocyte cell migration; cellular calcium ion homeostasis; cellular protein metabolic process; chondroitin sulfate catabolic process; ganglioside catabolic process; glycosphingolipid metabolic process; hyaluronan catabolic process; keratan sulfate catabolic process; lipid storage; locomotory behavior; lysosome organization and biogenesis; male courtship behavior; myelination; neuromuscular process controlling balance; neutrophil degranulation; oligosaccharide catabolic process; oogenesis; penetration of zona pellucida; phospholipid biosynthetic process; positive regulation of transcription from RNA polymerase II promoter; regulation of cell shape; sensory perception of sound; skeletal system development. Disease: Sandhoff Disease

size1 :

0.01 mg

price1 :

105 USD

size2 :

0.02 mg

price2 :

125

size3 :

0.05 mg

price3 :

165

size4 :

0.1 mg

price4 :

210

size5 :

0.2 mg

price5 :

310

size6 :

1 mg

price6 :

725

more info or order :

MyBioSource product webpage