Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Format
Liquid
Concentration
1.0 mg/mL
Buffer
PBS, 0.05 % Sodium Azide, 0.5 % protein stabilizer
Preservative
Sodium azide
Precaution of Use
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Target
Hexosaminidase A (HEXA)
Alternative Name
beta-Hexosaminidase alpha / HEXA
Background
This gene encodes the alpha 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. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).Synonyms: Beta-N-acetylhexosaminidase subunit alpha, N-acetyl-beta-glucosaminidase subunit alpha