Species: Human
Application Note: Measured by its ability to hydrolyze 4methylumbelliferylNacetylβDglucosaminide(4MUGlcNAc). The specific activity is > 3000 pmols/min/μg
Form: Lyophilized powder
Buffer (with preservative): Batch dependent (please contact us for details)
Concentration:
Background: 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 ID: P07686
Region Sequence: C-terminal polyhistidine tag; Ala43-Met556 (P07686)
ExpressionSystem: HEK293 Cells
Conjugation: Unconjugated
Full Name: hexosaminidase subunit beta
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