A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes include mutations of enzymes in the beta-n-acetylhexosaminidases system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of gangliosides, a subclass of acidic glycosphingolipids.


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