Tay-Sachs disease represents a disorder, which is passed on from parents to children, that is genetically inherited. It is a progressive neurodegenerative disease damaging nerve cells.
Children, who are affected by Tay-Sachs, as a rule, do not go over the age of 5. Less severe forms of the disease can develop in young children, teens, as well as people aged 20-30.
The most severe form of Tay-Sachs appears in babies, who are just a few months old. Thus, they do not develop at the usual pace as their development gets held back. They experience loss of vision and usually show an abnormal reaction if have a fright. Another sign is a gradual development of paralysis. Among other consequences of the disease is getting deaf, convulsions, and finally inability to swallow or breathe.
As of now, unfortunately no cure or any efficient way to postpone the speed of development of Tay-Sachs is found. A big number of scientists all over the world are trying to make a research and study different options for treating numerous diseases that are caused by exceeding production of cell products in nerve cells or other kinds of cells. According to researchers, all these diseases have something in common. This discovery makes it more probable that progress in one sphere will help to advance in others. The crossover effect of this study has already assisted in realizing the process of normal cells work and how this can help in treating these diseases.
Tay-Sachs disease was first found in 1881. It is connected with problems in lysosomes that represent the centers of the cell serving for waste processing. Mutated genes are held accountable for producing enzymes that are not that effective as normal when there is a need to split fatty cell products called gangliosides. This leads to excessive production of gangliosides in the lysosomes that then overload cells. Eventually this results in damaging nerve cells.
Tay-Sachs belongs to 50 diseases that are united by damaged waste processing and overloading amount of products in lysosomes. It the 1960s, these were called lysosomal storage disorders. Each of those 50 disorders led to damaging different areas and organs of the body. They also affect people at different ages. As far as Tay-Sachs disease is concerned, it gets extremely severe in brain cells.
According to clinicians, the attempts concerning Tay-Sachs disease during last generation have been highly successful. In the 1970s there appeared programs that were aimed at screening potential parents, who could have the gene leading to Tay-Sachs disease as well as prenatal testing to detect the enzyme produced by the gene. The implementation of such programs in the United States and Canada has assisted in lowering the number of sufferers by 90 percent.
The latest studies carried out on mice show that gene therapy, presupposing delivering normal genes to the brain in order to boost the splitting of gangliosides, helped to postpone the beginning of the disease, reduce inflammation, improve function and prolong their life. Other researches deal with finding out if reducing calories postpones the disease, the potential positive effects of transplanting stem cells, as well as the role of inflammation.
However, there is no single solution to combating Tay-Sachs disease. Researchers stay optimistic with animal research that says a combination of different kinds of treatment has better results.
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