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GM2 Activator Deficiency is referred to as the AB variant of GM2 Gangliosidosis and is similar to the features of the other variants, Tay-Sachs Disease and Sandhoff Disease. The disorder is caused by lack of the GM2 Activator that facilitates the breakdown of ganglioside by beta-hexosaminidase A, resulting in an accumulation of ganglioside, as in the other GM2 Gangliosidosis variants. |