Western Blot (WB)
(Western Blot: Sample: Human Hela cell lysate; Primary Ab: 2ug/ml Rabbit Anti-Human HEXb Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Rabbit anti-Human Hexosaminidase B Beta Polyclonal Antibody | anti-HEXb antibody
Polyclonal Antibody to Hexosaminidase B Beta (HEXb)
Immunohistochemistry: 5-20 Ug/mL;1:36-140
Immunocytochemistry: 5-20 Ug/mL;1:36-140
Optimal working dilutions must be determined by end user.
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 determinedby 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.
Western Blot (WB)
(Western Blot: Sample: Human Hela cell lysate; Primary Ab: 2ug/ml Rabbit Anti-Human HEXb Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Western Blot (WB)
(Western Blot: Sample: Human Hela cell lysate; Primary Ab: 2ug/ml Rabbit Anti-Human HEXb Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody )
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded prostate gland tissue))
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded prostate gland tissue))
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Liver cancer Tissue))
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Liver cancer Tissue))
NCBI and Uniprot Product Information
NCBI Description
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 Description
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
Research Articles on HEXb
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Product Notes
The HEXb hexb (Catalog #AAA2026696) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Polyclonal Antibody to Hexosaminidase B Beta (HEXb) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Hexosaminidase B Beta can be used in a range of immunoassay formats including, but not limited to, WB; IHC; ICC; IP. Western blotting: 0.5-2 Ug/mL;1:360-1400Immunohistochemistry: 5-20 Ug/mL;1:36-140
Immunocytochemistry: 5-20 Ug/mL;1:36-140 Optimal working dilutions must be determined by end user. Researchers should empirically determine the suitability of the HEXb hexb for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Hexosaminidase B Beta, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.
Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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