CAG repeat size in the normal HTT allele and age of onset in Huntington's disease

Movement Disorders

Volumn 26, Issue 13, 2011, Pages 2450-2451

  Aziz, Nasir Ahmad ^a   Van Roon Mom, Willeke M C ^a   Roos, Raymund A C ^a  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ALLELE; CAG REPEAT; DISEASE COURSE; HUMAN; HUNTINGTON CHOREA; LETTER; NONPARAMETRIC TEST; PEARSON SYNDROME; POINT MUTATION; PRIORITY JOURNAL; PROPORTIONAL HAZARDS MODEL;

FEMALE; HUMANS; HUNTINGTON DISEASE; MALE; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; TRINUCLEOTIDE REPEATS;

EID: 82055169038     PISSN: 08853185     EISSN: 15318257     Source Type: Journal    
DOI: 10.1002/mds.23849     Document Type: Letter

References (5)

1. Klempir J, Zidovska J, Stochl J, Ing VK, Uhrova T, Roth J. The number of CAG repeats within the normal allele does not influence the age of onset in Huntington's disease. Mov Disord 2011; 26: 125-129.
2. Aziz NA, Jurgens CK, Landwehrmeyer GB, et al. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease. Neurology 2009; 73: 1280-1285.
3. Djousse L, Knowlton B, Hayden M, et al. Interaction of normal and expanded CAG repeat sizes influences age at onset of Huntington disease. Am J Med Genet A 2003; 119: 279-282.
4. Langbehn DR, Hayden MR, Paulsen JS. CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches. Am J Med Genet B Neuropsychiatr Genet 2010; 153B: 397-408.
5. Squitieri F, Gellera C, Cannella M, et al. Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course. Brain 2003; 126: 946-955.