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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
Tay-Sachs disease is particularly common among Jewish people of Eastern European and Russian (Ashkenazi) origin. About one out of every 2,500 to 3,600 babies born to Ashkenazi Jewish couples have the disease. In the general population about one out of every 320,000 babies born has Tay-Sachs disease. Approximately one in 30 Ashkenazi Jews is a carrier of the gene that causes the disease. Tay-Sachs is also more common among certain French-Canadian, Pennsylvania Dutch, and Cajun families.
Tay-Sachs is caused by a defective gene. Genes are located on chromosomes and serve to direct specific developments and processes within the body. The genetic defect in Tay-Sachs disease results in the lack of an enzyme called hexosaminidase A. Without this enzyme, gangliosides cannot be broken down. They build up within the brain, interfering with nerve functioning. Because Tay-Sachs is a recessive disorder, only people who receive two defective genes (one from the mother and one from the father) will actually have the disease. People who have only one defective gene and one normal gene are called carriers. They carry the defective gene and thus the possibility of passing the gene and/or the disease onto their offspring.
When a carrier and a non-carrier have children, none of their children will actually have Tay-Sachs. The statistical probability is that 50 percent of their children will be carriers themselves. When two carriers have children, their children have a 25 percent chance of having normal genes, a 50 percent chance of being carriers of the defective gene, and a 25 percent chance of having two defective genes. Only the individual with two defective genes actually has the disease.
Classic Tay-Sachs disease strikes infants around the age of six months. Up until this age, the baby appears to develop normally. When Tay-Sachs begins to show itself, the baby stops interacting with other people and
A few variations from this classical progression of Tay-Sachs disease are possible:
Author Info: Tish Davidson A.M., Thomson Gale, Gale, Detroit, Gale Encyclopedia of Children's Health, 2006This feature is for informational purposes only and should not be used to replace the care and information received from your healthcare provider. Please consult a healthcare professional with any health concerns you may have.
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