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Tay-Sachs disease is a genetic disorder that results in the destruction of neurons due to a buildup of sphingolipids in the cells. Which organelle is malfunctioning in Tay-Sachs?

a) Lysosomes
b) Golgi apparatus
c) Endoplasmic reticulum
d) Peroxisomes

1 Answer

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Final answer:

Tay-Sachs disease is caused by a malfunction in the lysosomes due to a deficiency of a key enzyme. It is an autosomal recessive disorder, affecting children who inherit two copies of the mutated gene.

Step-by-step explanation:

Tay-Sachs disease is a genetic disorder characterized by the destruction of neurons due to a buildup of sphingolipids within the cells. The organelle that is malfunctioning in Tay-Sachs is the lysosome. This malfunction is due to a deficiency in a specific enzyme that normally breaks down sphingolipids in the lysosomes.

Children become affected with Tay-Sachs when they inherit two copies of the mutated gene, one from each parent, making it an autosomal recessive disorder. Carriers of the Tay-Sachs gene, who have only one copy of the mutated gene, are not affected and do not show symptoms of the disease.

In neurodegenerative disorders, symptoms are related to which areas of the nervous system are affected by neuronal death. Tay-Sachs, as a neurodegenerative disorder, specifically causes damage to the neurons in the brain, leading to a severe decline in mental and physical capabilities, often resulting in early childhood death.

The correct option is A.

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