Tay-Sachs disease is a genetic abnormality involving the metabolism of gangliosides which are important components of neuron cell membranes.
Tay-Sachs and several other debilitating diseases occur in much greater proportion in the Ashkenazi Jews (communities found Northern and Central Europe) compared to the rest of the world’s population. After the first Jewish-Roman war in Judea which resulted in the destruction the temple in 70 CE (current era), a small band of Jews migrated to Europe. It is now believed that just four women who lived 2,000-3,000 years ago are the ancestors of today’s Ashkenazi Jews.
- Briefly describe how the genetic abnormality leads to malfunction of neurons.
- What parts of the nervous system are involved and where are we in terms of current research and understanding of Tay-Sachs disease?