Definition: An essential cofactor for the degradation of G(M2)GANGLIOSIDE by lysosomal BETA-N-ACETYLHEXOSAMINIDASES. Genetic mutations resulting in loss of G(M2) activator protein are one of the causes of TAY-SACHS DISEASE, AB VARIANT.
Other names Activator Protein, GM2; Hexosaminidase Activator; GM2 Activator Protein; GM(2) Activating Protein
