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Es, which are structures in cells that break down toxic substances and act as recycling centers. Within lysosomes, beta-hexosaminidase a helps break down a fatty substance called gm2 ganglioside. Mutations in the hexa gene disrupt the activity of beta-hexosaminidase a, which prevents the enzyme from breaking down gm2 ganglioside. As a result, this substance accumulates to toxic.
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