What is the most prevalent genetic disorder in Ashkenazi Jews?

The most prevalent genetic disorder in Ashkenazi Jews is Tay-Sachs disease. This condition is caused by a deficiency of the enzyme hexosaminidase A, leading to the accumulation of GM2 gangliosides in the brain, which results in progressive neurological decline. The carrier frequency for Tay-Sachs among Ashkenazi Jews is approximately 1 in 27, which is significantly higher than in the general population.

In contrast, while Gaucher disease is indeed more common among Ashkenazi Jews and involves a deficiency in the enzyme glucocerebrosidase, Tay-Sachs disease is more widely recognized as the predominant genetic disorder of this group due to its higher prevalence and the severe implications of the condition, particularly in terms of early death in affected children. Canavan disease and Fanconi anemia, while present in this population, do not reach the same level of frequency as Tay-Sachs.