Tay-Sachs Disease - Description

Gangliosides (pronounced GANG-glee-uh-SIDES) are fatty substances needed for the proper development of the brain and nerve cells. Under normal conditions, gangliosides are continuously broken down so that the correct amount of gangliosides is always present.

The chemical required to break down gangliosides is an enzyme. Enzymes are chemical compounds present in all cells. These compounds make possible thousands of different reactions needed to keep cells operating normally. In a person with Tay-Sachs disease, the enzyme needed to break down gangliosides is missing. As a result, gangliosides continue to build up in the brain. When the brain becomes clogged with this fatty material it is no longer able to function normally.

Tay-Sachs is especially common among Jewish people of eastern European and Russian origin, sometimes referred to as Ashkenazic Jews. About 1 in every 3,600 babies born to Ashkenazic Jewish couples will have Tay-Sachs disease. The disease is also relatively common among certain French-Canadian and Cajun French families. Tay-Sachs disease is quite rare in families of other ethnic backgrounds.