This gene encodes a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A.
Beta-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
Mutations in this gene result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease.
Alternative splicing results in multiple transcript variants.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Optimal 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:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by affinity chromatography.
Form:Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Specificity:Recognizes human GM2A.
Isotype:IgG
Calc Applications Abbrev:WB
Calc Crossreactivity:Hu
Immunogen:Synthetic peptide corresponding to the N-terminal region of human GM2A