Answer:
Based on the given information , the cellular structure , which don not function properly in Tay Sachs disease is <u>Lysosomes </u>
Explanation:
<u>LYSOSOMES -:</u> In many animal cells, a lysosome is a membrane-bound organelle. These are spherical vesicles containing hydrolytic enzymes that can break down many types of biomolecules. A lysosome, with both its membrane proteins and its lumenal proteins, has a particular composition. There are a number of enzymes in lysosomes, helping the cell to break down different biomolecules that it swallows, including peptides, nucleic acids, carbohydrates , and lipids (lysosomal lipase). For optimal function, the enzymes responsible for this hydrolysis need an acidic environment.
<u>TAY SACHS DISEASE -: </u>The autosomal recessive disorder Tay-Sachs is caused by mutations in both gene alleles (HEXA) on chromosome 15. HEXA codes for the enzyme β-hexosaminidase A alpha subunit. This enzyme is contained in organelles, lysosomes, which break down large molecules for the cell to recycle.
Normally, β-hexosaminidase A helps to degrade a lipid called ganglioside GM2, but the enzyme is absent or present only in very small amounts in Tay-Sachs individuals, enabling excessive accumulation of ganglioside GM2 in neurons. In the different types of Tay-Sachs, the progressive neurodegeneration seen depends on the speed and degree of accumulation of GM2 ganglioside, which in turn depends on the amount of functional β-hexosaminidase A present in the body.
Hence , the answer is<u> lysosomes .</u>