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Huntington Disease

Known as: Huntington Chorea, Progressive Chorea, Hereditary, Chronic (Huntington), Huntington Disease [Disease/Finding] 
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth… 
National Institutes of Health

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2011
Review
2011
  • D. Selkoe
  • Cold Spring Harbor Perspectives in Biology
  • 2011
  • Corpus ID: 117081
Over the last three decades, advances in biochemical pathology and human genetics have illuminated one of the most enigmatic… 
Review
2010
Review
2010
  • R. Roos
  • Orphanet Journal of Rare Diseases
  • 2010
  • Corpus ID: 17350013
Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic… 
Highly Cited
2005
Highly Cited
2005
  • B. Harper
  • Journal of the Royal Society of Medicine
  • 2005
  • Corpus ID: 2560042
Huntington disease affects between 6500 and 8000 people in the UK and has a worldwide prevalence of about 1 in 20 000. It is a… 
Highly Cited
2004
Highly Cited
2004
Huntington disease is one of nine inherited neurodegenerative disorders caused by a polyglutamine tract expansion. Expanded… 
Highly Cited
2001
Highly Cited
2001
Huntingtin is a 350-kilodalton protein of unknown function that is mutated in Huntington's disease (HD), a neurodegenerative… 
Highly Cited
2000
Highly Cited
2000
Huntington's Disease (HD) is caused by an expansion of a polyglutamine tract within the huntingtin (htt) protein. Pathogenesis in… 
Highly Cited
1996
Highly Cited
1996
! The Unified Huntington’s Disease Rating Scale (UHDRS) was developed as a clinical rating scale to assess four domains of… 
Highly Cited
1996
Highly Cited
1996
The Unified Huntington's disease Rating Scale (UHDRS) was developed as a clinical rating scale to assess four domains of clinical… 
Highly Cited
1985
Highly Cited
1985
In postmortem brain specimens from 163 clinically diagnosed cases of Huntington's disease (HD) the striatum exhibited marked… 
Highly Cited
1983
Highly Cited
1983
Family studies show that the Huntington's disease gene is linked to a polymorphic DNA marker that maps to human chromosome 4. The…