The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.3 - 1 µg/ml. Detects a band of approximately 28 kDa (predicted molecular weight: 21 kDa).
Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3.
Involvement in disease
Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB) [MIM:272750]; also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B.
The serines in positions 32 and 33 are absent in 80% of the sequenced protein.
Anti-GM2A antibody (ab113440) at 0.300000011921 µg/ml + Rat kidney lysate in RIPA buffer at 35 µg Developed using the ECL technique
Predicted band size : 21 kDa
Western blot - GM2A antibody (ab113440)
Lane 1 : Anti-GM2A antibody (ab113440) at 1 µg/ml Lane 2 : Anti-GM2A antibody (ab113440) at 1 mg/ml Lane 3 : anti- DYKDDDDK Tag at 1/30000 dilution
Lane 1 : HEK293 lysate (in RIPA buffer) overexpressing Human GM2A with DYKDDDDK tag Lane 2 : Mock-transfected HEK293 lysate (in RIPA buffer) Lane 3 : HEK293 lysate (in RIPA buffer) overexpressing Human GM2A with DYKDDDDK tag