disease causes dysfunction of ganglion Tay-Sachs disease specific cell type BTO:0001399 BrendaTissueOBO TSD cell Tay-Sachs disease is a lysosomal disease (lipidosis) in which hexosaminidase A, an enzyme that degrades ganglioside GM2, is absent. A hereditary disease that affects young children almost exclusively of eastern European Jewish descent, in which an enzyme deficiency leads to the accumulation of gangliosides in the brain and nerve tissue, resulting in mental retardation, convulsions, blindness, and, ultimately, death.