In autopsy tissues of 12 cam of Tay-Sachs disease the N-acetyl-/h-hexosaminidase A and B activities were investigatcd using chromogenic and physiological substrates.In three cases of Tay-Sachs disease, classified as the variant 0, the enzyme activities A and B were missing; in eight cases, classified as the variant B, the enzyme activity A was Abbreuiutions used: Gal, D-galactose; GalNAc, N-acetyl-D-galactosamine; Glc, D-glucose; NeuNAc, N-acetylneuraminic acid; TSG, ganglioside Ghf2 (Tay-Sachs-ganglioside); THC, trihexosylceramidc (sialic acid free residue of ganglioside GhS2); hexosaminidase, N-acetyl-,%D-hexosaminidase, p-2-acetomido-2-deoxy-u-glucoside acetamidodeoxyglucohydrolase (EC 3 . 2 .

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