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https://ghr.nlm.nih.gov/condition/tay-sachs-disease#
http://www.nhs.uk/conditions/Tay-Sachs-disease/Pages/Introduction.aspx
https://www.genome.gov/10001220/learning-about-taysachs-disease/

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There is no known cure for Tay-Sachs, so treatment consists of making the child as comfortable as possible by treating symptoms. Tay-Sachs is untreatable, however screening parents who are considering of starting a family can help in identifying the chances of a child to have Tay-Sachs. Tay-Sachs is inherited in an autosomal recessive pattern, which means that the two parents, which are both carriers of the disease, has a 25% chance that the child will be unaffected, 50% chance that their child will also be a carrier, meaning they carry the gene, but is unaffected by Tay-Sachs, and 25% of having Tay-Sachs.

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Tay-Sachs disease is a very rare disease in the general public, however it is documented that this disease is more common in people of the Ashkenazi Jewish Heritage. Some other groups of people who are affected more commonly are the French-Canadians of Quebec, the older Amish communities of Pennsylvania, and the Cajun Population of Louisiana. It is estimated that around 1 in  360,000 children are born with Tay-Sachs. Due to the fact that children with Tay-Sachs only live up to their early childhood, and that this disease is inherited, only children get Tay-Sachs.

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Symptoms of Tay-Sachs usually don't show up in children until they are around 3-6 months old. Because of the harming of the nervous system, the child affected with Tay-Sachs will begin to slowly lose the ability to move and start slowing down development. Some other symptoms of Tay-Sachs are, the loss of motor skills, an exaggerated startle reaction to loud noises, seizures, vision and hearing loss, intellectual disability, and paralysis. An eye abnormality that children with Tay-Sachs have is called a cherry-red spot, and can be identified through an eye examination. Children with severe Tay-Sachs only live to around 5 years of age, or early childhood.

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A mutation in a child's HEXA gene causes Tay-Sachs. This particular gene is the gene that provides information in the creation of the enzyme called beta-hexosaminidase A (Hex-A). This enzyme plays a critical role in cells in the brain and spinal cord. The enzyme Hex-A is located in the lysosome, where the enzyme helps break down the specific fatty acid called GM2 ganglioside (GM2). The mutation in the HEXA gene causes Hex-A to be absent within the cell, therefore the cell will be unable to break down GM2, in turn the building up of GM2 will rise to toxic levels and eventually lead to the destruction of the cell. As said before, these cells that require the Hex-A enzymes are usually in the brain and spinal cord area, so the destruction of nervous system cells can cause major damage to the nervous system and the brain.(Diagram below)

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Demonstration of how Tay-Sachs works within a cell

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Demonstration of how Tay-Sachs works within a cell

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A lysosome is a special formed vacuole by the Golgi body. These special organelles contain powerful hydrolytic enzymes used to digest substances entering the cell or organelles that are of no further use to the cell. They are usually considered the stomach/recycling center of the cell.

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The Organelle in which Tay-Sachs affects is the Lysosome

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The Organelle in which Tay-Sachs affects is the Lysosome

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Tay-Sachs is an inherited disorder that destroys nerve cells in the Brain and Spinal Cord.