What genetic disorder is linked to a deficiency of the enzyme hexosaminidase?

The disorder linked to a deficiency of the enzyme hexosaminidase is Tay Sachs disease. This condition is characterized by the body's inability to break down a specific type of fat called GM2 ganglioside due to the lack of hexosaminidase A, an enzyme crucial for metabolizing these fatty substances. The accumulation of GM2 ganglioside, particularly in the nerve cells, leads to the progressive deterioration of neurological function.

Individuals affected by Tay Sachs typically present symptoms such as developmental delays, loss of motor skills, and severe neurological impairment, often leading to a shortened lifespan. The genetic basis of this disorder is associated with mutations in the HEXA gene, which provides instructions for making the hexosaminidase A enzyme. This condition is inherited in an autosomal recessive pattern, meaning that a child would need to inherit two copies of the mutated gene—one from each parent—to manifest the disease.

The link between Tay Sachs disease and the deficiency of hexosaminidase explains why this option is the correct choice in the context of the question.