Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage Authored: Jassal, B, 2011-09-21 Edited: Jassal, B, 2011-09-21 Pubmed12909021 Pubmed1915858 Reactome Database ID Release 431605717 Reactome, http://www.reactome.org ReactomeREACT_115670 Reviewed: Stephan, R, 2011-10-31 The Ganglioside GM2 activator protein (GM2A) is a small lysosomal lipid transfer protein that extracts a single GM2 molecule from membranes and presents it in a soluble form to beta-hexosaminidase A for cleavage (Wright et al. 2003). Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB) (MIM:272750), also known as Tay-Sachs disease AB variant (Schroeder et al. 1991). GM2A:GM2