Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • GM2 gangliosidosis, type 1
  • HexA deficiency
  • B variant GM2 gangliosidosis
  • Hexosaminidase A deficiency
  • Hexosaminidase alpha-subunit deficiency (variant B)
  • Sphingolipidosis, Tay-Sachs
  • TSD
  • Gangliosidosis GM2 , type 1

Tay Sachs disease
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Gangliosidosis (GM2) type 1, also known as Tay-Sachs disease, is a rare inherited disorder that causes progressive destruction of nerve cells in the brain and spinal cord. Tay-Sachs is caused by the absence of a vital enzyme called hexosaminidase-A (Hex-A). Without Hex-A, a fatty substance, or lipid, called GM2 ganglioside accumulates abnormally in cells, especially in the nerve cells of the brain. This ongoing accumulation causes progressive damage to the cells. [1]

References
  1. Learning about Tay-Sachs Disease. National Human Genome Research Institute Web site. 2007 Available at: http://www.genome.gov/page.cfm?pageID=10001220 . Accessed November 14, 2007.

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