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Causes
By Mayo Clinic staff
Huntington's disease is an inherited condition caused by a single abnormal gene. Doctors refer to the illness as an autosomal dominant disorder because only one copy of the defective gene, inherited from either parent, is necessary to produce the disease. If one parent has the single faulty gene, the chance that an offspring will have the defect is 50 percent. Because signs and symptoms typically first appear in middle age, some parents may not know they carry the gene until they've already had children and possibly passed on the trait.
If your child doesn't inherit the faulty gene, he or she won't develop Huntington's disease and can't pass it on to the next generation. Everyone who has the gene eventually develops Huntington's disease, if he or she lives long enough.
In 2006, researchers discovered that the protein expressed by the Huntington's gene interacts with another protein to disturb the way that cholesterol accumulates in the brain. Cholesterol is essential for healthy brain cells and the network among those brain cells — but the cholesterol needs to be in proper levels and in the proper locations. When the network of brain cells is disrupted, motor skills, cognitive skills and speech can be affected. If scientists can figure out a way to disrupt this interaction between the proteins, they may eventually be able to develop a targeted drug therapy.
- Huntington's disease: Hope through research. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm. Accessed March 27, 2009.
- Huntington's disease. The Merck Manuals Online Medical Library: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/sec16/ch221/ch221e.html#sec16-ch221-ch221e-49. Accessed March 27, 2009.
- SuttonBrown MA, et al. Huntington disease: Clinical features and diagnosis. http://uptodate.com/home/index.html. Accessed March 12, 2009.
- Learning about Huntington's disease. National Human Genome Research Institute. http://www.genome.gov/page.cfm?pageID=10001215. Accessed March 27, 2009.
- Trushina E, et al. Mutant huntingtin inhibits clathrin-independent endocytosis and causes accumulation of cholesterol in vitro and in vivo. Human Molecular Genetics. 2006;15:3578.
- Katsuno M, et al. Getting a handle on Huntington's disease: The case for cholesterol. Nature Medicine. 2009;15:253.
- FDA approves first drug for treatment of chorea in Huntington's disease. U.S. Food and Drug Administration. http://www.fda.gov/bbs/topics/NEWS/2008/NEW01874.html. Accessed March 28, 2009.
- Kim SU. Stem cell-based cell therapy in neurological diseases: A review. Journal of Neuroscience Research. In press. Accessed March 28, 2009.
- ACR16. Neurosearch.com. http://www.neurosearch.com/Default.aspx?ID=752. Accessed March 28, 2009.
