Tay-Sachs disease is a terminal disease of the nervous system. It was first identified in 1881. Tay-Sachs disease occurs because of problems with lysosomes. Lysosomes are organelles in cells which essentially process waste. They are kind of like the garbage men of the cell! I came up with that analogy all by myself! Clever, I know.
This article explains how a person with Tay-Sachs has mutated genes that result in the production of enzymes less effective in breaking down gangliosides. These enzymes are found in lysosomes. Gangliosides are fatty cell products. Since these enzymes are less effective, gangliosides build up in the lysosomes and eventually overload the cell. This build up causes damage to nerve cells.
This mess up on a cellular level results in terrible outcomes for people with Tay-Sachs disease. This article talks about symptoms of the disease include deafness, blindness, decreased muscle tone, loss of motor skills, loss of muscle function/paralysis, and many others. Tay-Sachs is an inherited genetic disease. The child must receive the gene from both parents in order to be affected by it, otherwise the child will just be a carrier. This is why nerve damage caused by the disease often begins when the child is still in the womb. Symptoms usually start popping up when the child is 3 to 6 months old. Unfortunately, most people with Tay-Sachs disease don't live past 4 or 5 years old.
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