The affected Gene is on Chromosome 5 and affects the beta-subunit.

This is a deficiency in the hexoaminidase B enzyme.

The Hexoaminidase removes an N-acetylgalactosamine.

Symptoms of Sandhoff Disease are the same as Tay-Sachs, but they progress more rapidly.

The accumulating substance in Sandhoff-Jatzkewitz Disease are Globoside and GM2 gangliosides.