G(M2) Activator Protein

**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.

**Tree numbers:** - D08.211.790.249

**Synonyms:** - GM(2) Activating Protein - GM2 Activator Protein - Hexosaminidase Activator