- Any disease characterized, in part, by the abnormal accumulation within the nervous system of specific gangliosides, e.g., GM2 g., Tay-Sachs disease, caused by hexosaminidase A enzyme deficiency with accumulation of GM2 ganglioside. SYN: gangliosialidosis, ganglioside lipidosis.- GM1 g. three forms exist: infantile, generalized; juvenile; and adult; g. characterized by accumulation of a specific monosialoganglioside, GM1; due to deficiency of GM1-β-galactosidase. SYN: generalized g..- GM2 g. one of the hereditary metabolic disorders; several forms exist, including Tay-Sachs disease, Sandhoff disease, AV variant and adult onset; characterized by accumulation of a specific metabolite, GM2 ganglioside, due to deficiency of hexosaminidase A or B, or GM2 activator factor.- infantile, generalized GM1 g. one of the hereditary metabolic diseases of infancy; resembles Tay-Sachs disease, except other organ systems (bone, liver, kidney) are affected. SYN: familial neuroviscerolipidosis, pseudo-Hurler disease, Type 1 GM1 g..
* * *gan·gli·o·si·do·sis .gaŋ-glē-.ō-sī-'dō-səs n, pl -do·ses -.sēz any of several inherited metabolic diseases (as Tay-Sachs disease) characterized by an enzyme deficiency which causes accumulation of gangliosides in the tissues
* * *gan·gli·o·si·do·sis (gang″gle-o-si-doґsis) pl. gangliosidoґses. Any of a group of lysosomal storage diseases generally characterized by abnormal accumulation of ganglioside GM1 or GM2 and related glycoconjugates due to a deficiency of specific lysosomal hydrolases, and by progressive psychomotor deterioration usually beginning in infancy or childhood and usually fatal. Two subgroups exist (GM1 and GM2), each having several forms of varying severity.
Medical dictionary. 2011.