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Overview
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GA2-ganglioside is an asialo glycosphingolipid, also known as asialo-GM2 ganglioside. GA2 ganglioside accumulates in the cells of individuals with Sandhoff disease, a lysosomal storage disorder where β-hexosaminase is dysfunctional and unable to break down GA2 ganglioside to lactosylceramide (LacCer) (Sandhoff, 1968); a possible treatment for Sandhoff disease is substrate reduction therapy (Marshall, 2019).
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Overview