TAY_SACHS AND GM FOODS

Tay-Sachs is an autosomal recessive disease that occurs mainly in infants of Ashkenazi Jewish decent. The disease causes an abnormality in the gene responsible for the coding of hexosaminidase A (HEXA) that breaks down a fatty material called ganglioside GM2. This fatty material builds in the brain leading to blindness, deafness, paralysis and death by the age of 5. Infants begin to show signs of Tay-Sachs between three and six months of age. A juvenile form has been observed that manifests itself between the ages of two and five with similar symptoms as the infantile manifestation and death occurs at about age 15. There is also a chronic manifestation that occurs around age five, but the symptoms are much weaker displa

yed through muscle weakness, slurred speech, tremors and occasionally mental illness. A mature manifestation occurs between the teen years and the 30’s resulting in many of the same symptoms as the chronic form; it is also known as LOTS or Late Onset Tay-Sachs. Amniocentesis or chorionic villus sampling can be done to determine if the child will be born with TSD or blood test can be done on an infant to see if the child is affected by TSD. The only known means of prevention is preconception genetic counseling to assess the risk of offspring being affected by TSD. There are no effective treatments for Tay-Sachs as all treatment are aimed at the alleviation of symptoms. TSD is a lifelong condition and there are no known cures. If both parents carry the recessive gene there is a one in

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