Gangliosides are fatty substances necessary for the proper development of the brain and nerve cells (nervous system).
Under normal conditions, gangliosides are continuously broken down, so that an appropriate balance is maintained.
In Tay-Sachs disease, the enzyme necessary for removing excess gangliosides is missing.
This situation allows gangliosides to accumulate throughout the brain and is responsible for the disability associated with the disease.
Without this enzyme, gangliosides cannot be broken down.