HEXA encodes a member of the glycosyl hydrolase 20 family of proteins.
The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase.
This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I).
Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.
Applications:Suitable for use in Western Blot, Immunohistochemistry, and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry (paraffin): 1:100-1:300ELISA: 1:20,000Optimal 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：30ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of 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 the Internal region of human HEXA.
