Question

Tay-Sachs disease is due to the abnormal
A) Ganglioside
B) Glycogen
C) Sphingomyelin
D) Glycosphingolipid

Answer 1

Tay-Sachs disease is a rare genetic disease that is caused due to the destruction of nerve cells in the spinal cord. It is characterized by many symptoms like seizures, loss of hearing, inability to move and finally death. This disease occurs mostly by the age of four and is less common in later childhood and adulthood.

Complete answer:
Tay-Sachs disease is caused due to the mutation in a gene known as the HEXA gene that codes for a subunit of hexosaminidase enzyme called hexosaminidase A. Hexosaminidase enzyme is involved in the degradation of gangliosides in the lysosome. This mutation results in the alteration of function of the enzyme which in turn results in the accumulation of a type of ganglioside known as the GM2 ganglioside. As a result the ganglioside accumulates in the lysosome of the neuron which leads to nerve cell destruction. Tay-Sachs disease is an autosomal recessive disorder which suggests that only when both the parents have the disease there is a chance that the child might also have the disease. Tay-Sachs disease can be detected by blood test that measures hexosaminidase A activity.

Hence the correct answer is (A) Ganglioside.

Note: Tay-Sachs disease gives us an idea of the importance of fatty acid degradation. If the degradation process is not carried out properly it can lead to many such diseases that can cause harm to the body. So care must be taken to properly regulate these processes.