Background: GM2-AP is a 193 amino acid protein that localizes to the lysosome. Existing as a small glycolipid transport protein, GM2-AP acts as a ganglioside-specific cofactor that, together with beta-hexosaminidase A (HEXA), stimulates the breakdown of glycolipid GA2 and ganglioside GM2 and is important for the degradation of proteins containing terminal N-acetyl hexosamines. Mutations in the gene encoding GM2-AP are the cause of GM2-gangliosidosis type AB (GM2GAB), which is also known as Tay-Sachs disease AB variant, and is an autosomal recessive disease that is characterized by ganglioside GM2 accumulation in the presence of both hexosaminidase A and B.
Description: Rabbit polyclonal to GM2A
Immunogen: KLH conjugated synthetic peptide derived from GM2A
Specificity: ·Reacts with Human, Mouse and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 21 kDa;
·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;
·Immunocytochemistry/Immunofluorescence: 1/100;
·Immunoprecipitation: 1/50;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.