How often does Tay-Sachs disease occur?

Tay-Sachs disease is a rare genetic disorder that occurs in approximately 1 in 3,600 live births in the United States. It is more common among Ashkenazi Jews, with an incidence of about 1 in 2,700 live births. The disease is caused by a mutation in the HEXA gene, which leads to a deficiency of an enzyme called hexosaminidase A. This enzyme is responsible for breaking down a fatty substance called GM2 ganglioside, which accumulates in the brain and other organs, causing progressive neurological damage.