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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Juvenile Huntington disease


Other Names for this Disease
  • Huntington disease, juvenile onset
  • JHD
  • Juvenile onset HD
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Inheritance


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How is Juvenile Huntington disease (HD) inherited?

Juvenile HD is inherited in an autosomal dominant, which means that one copy of the altered gene in each cell is sufficient to cause the disorder. An affected person usually inherits the altered gene from one affected parent. As the altered HTT gene is passed from one generation to the next, the size of the CAG trinucleotide repeat often increases in size. A larger number of repeats is usually associated with an earlier onset of signs and symptoms (anticipation). A larger number of repeats is usually associated with an earlier onset of signs and symptoms. Most often, children with juvenile HD inherit the expanded CAG trinucleotide repeat from their fathers, although on occasion they inherit it from their mothers.[1]
Last updated: 3/15/2011

References
  1. Huntington disease . Genetics Home Reference. OCtober 2008; http://ghr.nlm.nih.gov/condition/huntington-disease . Accessed 6/27/2011.