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).
Applications:Suitable for use in Western Blot, Immunohistochemistry and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000, incubate membrane with diluted primary antibody in TBS, 5% milk, 0.1% Tween 20 at 4°C with gentle shaking, overnight
Immunohistochemistry: 1:50-1:200ELISA (peptide): 1:20,000-1:40,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of total human HEXA. Species Crossreactivity: Mouse and rat
Isotype：IgG
Calc Applications Abbrev：E IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to human HEXA. Species sequence homology: Porcine (92%), Bovine (92%), Equine (92%), Sheep (92%), Rabbit (83%), Canine (92%), Chicken (80%)
