Tay Sachs is a disease caused by a mutation in a gene that codes for an enzyme used to break down complex molecules. Patients that have Tay Sachs accumulate excessive fat in brain cells and nerve cells. Normal functioning of this cell structure results in the digestion and removal of harmful fat accumulations, old cell parts, and invaders. Due to a mutation, the enzyme needed to remove fat accumulations does not function. Based on this information, what cellular structure is not functioning properly?

Based on the given information , the cellular structure , which don not function properly in Tay Sachs disease  is Lysosomes

Explanation:

LYSOSOMES -: 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.

TAY SACHS DISEASE -:  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 lysosomes .

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