For ten points, identify this autosomal recessive disease, most often carried by Ashkenazi Jews.
It is triggered by a mutation on the β-hexosaminidase α-subunit gene and is characterized by the inability of that hydrolytic enzyme to fully catalyse the breakdown of gangliosides, derivatives of fatty acids, leading to the buildup of GM2 in the nerve cells of the brain. Afflicted individuals present with hyperacusis and a cherry red spot on the retina, and in later stages, mental and physical deterioration as nerve cells are distorted by the accumulation of gangliosides. The anomalous presence of this disorder within certain French Canadian and Cajun populations led to the "Jewish Fur Trader" hypothesis, but it is more commonly connected with another ethnic group. For ten points, identify this autosomal recessive disease, most often carried by Ashkenazi Jews.
It is triggered by a mutation on the β-hexosaminidase α-subunit gene and is characterized by the inability of that hydrolytic enzyme to fully catalyse the breakdown of gangliosides, derivatives of fatty acids, leading to the buildup of GM2 in the nerve cells of the brain.
{Tay-Sachs} disease