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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Huntington disease


Other Names for this Disease
  • HD
  • Huntington chorea
  • Huntington's chorea
  • Huntington's disease
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Inheritance

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How is Huntington disease inherited?

Huntington disease (HD) is inherited in an autosomal dominant manner.[1] This means that having a change (mutation) in only one of the 2 copies of the responsible gene (the HTT gene) is enough to cause the condition. When a person with HD has children, each child has a 50% (1 in 2) chance to inherit the mutated gene and develop the condition.

Although HD can rarely be caused by a de novo mutation in the HTT gene (a mutation that occurs for the first time and is not inherited from a parent), most people with HD have an affected parent. The family history can sometimes appear negative for various reasons even though a parent carries, or carried, a mutation.[1]

As HD is passed through generations, the size of the trinucleotide repeat often increases. A longer repeat in the HTT gene may cause an earlier onset of symptoms. This phenomenon is called anticipation.[2]
Last updated: 7/8/2015

References
  1. Simon C Warby, Rona K Graham, and Michael R Hayden. Huntington Disease. GeneReviews. December 11, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1305/.
  2. Huntington disease. Genetics Home Reference. June, 2013; http://ghr.nlm.nih.gov/condition/huntington-disease.


Other Names for this Disease
  • HD
  • Huntington chorea
  • Huntington's chorea
  • Huntington's disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.