GM2 gangliosidosis


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Related to GM2 gangliosidosis: GM1 gangliosidosis, Beta-hexosaminidase A

GM2 gan·gli·o·si·do·sis

one of the hereditary metabolic disorders; several forms exist, including Tay-Sachs disease, Sandhoff disease, AB 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.
References in periodicals archive ?
No curative therapy is available for GM2 gangliosidosis. Seizures generally respond to standard treatment.
There's a cat model for Sandhoff disease, which is GM2 gangliosidosis. And there are some naturally occurring mutations for these kinds of gangliosidoses.
Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GMI and GM2 gangliosidosis. 2003;126:974-987.