The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
Best use within three months from the date of receipt of this protein.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% Glutathione, 0.79% Tris HCl
Beta hexosaminidase beta chain
Beta hexosaminidase subunit beta
Beta N acetylhexosaminidase
Beta-hexosaminidase subunit beta chain A
Beta-N-acetylhexosaminidase subunit beta
Cervical cancer proto oncogene 7 protein
Cervical cancer proto-oncogene 7 protein
Epididymis luminal protein 248
Hexosaminidase B (beta polypeptide)
Hexosaminidase subunit B
N acetyl beta glucosaminidase
N-acetyl-beta-glucosaminidase subunit beta
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.
Involvement in disease
Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:268800]; 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.
N-linked glycans at Asn-142 and Asn-190 consist of Man(3)-GlcNAc(2) and Man(5 to 7)-GlcNAc(2), respectively. The beta-A and beta-B chains are produced by proteolytic processing of the precursor beta chain.