Tay-Sachs disease is a human genetic abnormality that results in cells accumulating and becoming clogged with very large, complex, undigested lipids. Which cellular organelle must be involved in this condition

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04-03-2020
Biology

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Tay-Sachs disease is a human genetic abnormality that results in cells accumulating and becoming clogged with very large, complex, undigested lipids. Which cellular organelle must be involved in this condition

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MadisonTwist MadisonTwist · Answer 1 · 2020-03-04T05:26:10+01:00

Answer:

Lysosome is the cellular organelle that involved in Tay Sachs disease condition.

Explanation:

This is a rare inherited disorder that occur in the brain due to mutation in HEXA gene that unable to produce beta-hexosaminidase.

This enzyme mainly located in the lysosomes and helps in break down of fatty acid substance called GM2 ganglioside.

Mutation in HEXA gene causes disrupt in the activity of beta hexosaminidase a that prevents enzyme from breaking down into GM2 ganglioside. as a result the toxic level enters into the neuron in the brain and spinal cord and thus causes symptoms of Tay Sachs disease.

Symptoms-

Deafness,progressive blindness,decrease muscle strength,paralysis,seizure,muscular stiffness,slow growth,red spot on macula,increased startle response etc.