Is tay-sachs disease caused by a mutation?

Tay-Sachs disease is an inherited genetic disorder that is caused by a mutation in the HEXA gene. This mutation results in the absence or reduced activity of the enzyme hexosaminidase A, which is responsible for breaking down a fatty substance called GM2 ganglioside. As GM2 ganglioside accumulates in the brain, it leads to the progressive degeneration of nerve cells and eventually results in severe neurological symptoms and premature death.