The relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset

Annals of Human Genetics

Volumn 71, Issue 3, 2007, Pages 295-301

  Andresen, J Michael ^a   Gayán, Javier ^b   Djoussé, Luc ^c   Roberts, Simone ^d   Brocklebank, Denise ^b   Cherny, Stacey S ^e   Cardon, Lon R ^b   Gusella, James F ^f   Macdonald, Marcy E ^f   Myers, Richard H ^c   Housman, David E ^a,a   Wexler, Nancy S ^d   Lorimer, Judith ^d   Porter, Julie ^d   Gomez, Fidela ^d   Moskowitz, Carol ^d   Gerstenhaber, Kelly Posner ^d   Shackell, Edith ^d   Marder, Karen ^d   Penchaszadeh, Graciela ^d   Roberts, Simone A ^d   Brickman, Adam ^d   Gray, Jacqueline ^g   Dlouhy, Stephen R ^g   Wiktorski, Sandra ^g   Hodes, Marion E ^g   Conneally, P Michael ^g   Penney, John B ^f   Cha, Jang Ho ^f   Irizarry, Micheal ^f   Rosas, Diana ^f   Hersch, Steven ^f   Hollingsworth, Zane ^f   Young, Anne B ^f   de Young, Margot Mieja ^h   Bonilla, Ernesto ^h   Stillings, Theresa ^h   Negrette, Americo ^h   Snodgrass, S Robert ⁱ   Martinez Jaurrieta, Maria Dolores ^j   Ramos Arroyoh, Maria A ^j   Bickham, Jacqueline ^k   Ramos, Juan Sanchez ^l   Marshall, Frederick ^m   Shoulson, Ira ^m   Rey, Gustavo J ⁿ   Feigin, Andrew ^o   Arnheim, Norman ^p   Acevedo Cruz, Amarilis ^q   Acosta, Leticia ^r   Alvir, Jose ^s   Fischbeck, Kenneth ^t   Thompson, Leslie M ^u   Young, Angela ^v   Dure, Leon ^v   O'Brien, Christopher J ^w   Paulsen, Jane S ^x   Moran, Shelley Peery ^y   Krch, Denise ^z   Hogarth, Penelope ^aa   Higgins, Donald S ^ab   Landwehrmeyer, Bernhard ^ac   Hayden, Michael R ^ad   Almqvist, Elisabeth W ^ad   Brinkman, Ryan R ^ad   Suchowersky, Oksana ^ae   Durr, Alexandra ^af   Dode C ^ag   Squitieri, Ferdinando ^ah   Morrison, Patrick J ^ai,aj   Nance, Martha ^ak   Ross, Christopher A ^al   Margolis, Russell L ^al   Rosenblatt, Adam ^al   Estrella, Gómez Tortosai ^am   Cabrero, David Mayo ^am   Trent, Ronald J A ^an   McCusker, Elizabeth ^ao   Novelletto, Andrea ^ap   Frontali, Marina ^aq   Jones, Randi ^ar   Zanko, Andrea ^as   Ashizawa, Tetsuo ^at   Lazzarini, Alice ^au   Li, Jian Liang ^c,av,aw   Wheeler, Vanessa C ^ax   Russ, Ana L ^am   Xu, Gang ^c   Mysore, Jayalakshmi S ^f   Gillis, Tammy ^f   Hakky, Michael ^f   Cupples, L Adrienne ^av   Saint Hilaire, Marie ^c   Hersch, Steven M ^f   more..

^a MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

^b UNIVERSITY OF OXFORD   (United Kingdom)

^c BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d Och Spine at New York Presbyterian Hospitals   (United States)

^e UNIVERSITY OF HONG KONG   (Hong Kong)

^f MASSACHUSETTS GENERAL HOSPITAL   (United States)

^g INDIANA UNIVERSITY   (United States)

^h UNIVERSIDAD DEL ZULIA   (Venezuela)

ⁱ CHILDREN S HOSPITAL LOS ANGELES   (United States)

^j Universidad Pública de Navarra   (Spain)

^k UNIVERSITY OF TEXAS   (United States)

^l UNIVERSITY OF SOUTH FLORIDA   (United States)

^m UNIVERSITY OF ROCHESTER   (United States)

ⁿ MIAMI CHILDREN'S HOSPITAL   (United States)

^o NORTH SHORE UNIVERSITY HOSPITAL   (United States)

^p UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^q MOUNT SINAI MEDICAL CENTER   (United States)

^r UNIVERSITY OF CALIFORNIA COOPERATIVE EXTENSION   (United States)

^s NEW YORK UNIVERSITY   (United States)

^t NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

^u UNIVERSITY OF CALIFORNIA   (United States)

^v UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^w THOMAS JEFFERSON UNIVERSITY   (United States)

^x UNIVERSITY OF IOWA   (United States)

^y NEW YORK UNIVERSITY MEDICAL CENTER   (United States)

^z CITY UNIVERSITY OF NEW YORK   (United States)

^aa OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

^ab ALBANY MEDICAL COLLEGE   (United States)

^ac UNIVERSITY OF ULM   (Germany)

^ad UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^ae UNIVERSITY OF CALGARY   (Canada)

^af PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^ag Hôpital de la Salpêtrière ^*  (France)

^ah IRCCS NEUROMED   (Italy)

^ai BELFAST CITY HOSPITAL   (United Kingdom)

^aj UNIVERSITY OF ULSTER   (United Kingdom)

^ak HENNEPIN COUNTY MEDICAL CENTER   (United States)

^al JOHNS HOPKINS UNIVERSITY   (United States)

^am Clínica Nuestra Señora de la Concepción   (Spain)

^an UNIVERSITY OF SYDNEY   (Australia)

^ao WESTMEAD HOSPITAL   (Australia)

^ap UNIVERSITY OF CALABRIA   (Italy)

^aq CNR   (Italy)

^ar EMORY UNIVERSITY   (United States)

^as UNIVERSITY OF CALIFORNIA   (United States)

^at BAYLOR COLLEGE OF MEDICINE   (United States)

^au ROBERT WOOD JOHNSON MEDICAL SCHOOL   (United States)

^av BOSTON UNIVERSITY   (United States)

^aw PFIZER INC   (United States)

^ax NONE

more..

Author keywords

Huntington's disease; Multiple linear regression

Indexed keywords

ADENINE; CYTOSINE; GUANINE; TRINUCLEOTIDE;

AGE DISTRIBUTION; ANALYSIS OF VARIANCE; ARTICLE; AUSTRALIA; COHORT ANALYSIS; CONTROLLED STUDY; EUROPE; HUMAN; HUNTINGTON CHOREA; MULTIPLE LINEAR REGRESSION ANALYSIS; NORTH AMERICA; ONSET AGE; PATHOGENESIS; PRIORITY JOURNAL; REFERENCE VALUE; SAMPLING; STATISTICAL MODEL; TRINUCLEOTIDE REPEAT; VENEZUELA;

EID: 34247120606     PISSN: 00034800     EISSN: 14691809     Source Type: Journal    
DOI: 10.1111/j.1469-1809.2006.00335.x     Document Type: Article

References (11)

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3. Djoussé, L., Knowlton, B. et al. (2003) Interaction of normal and expanded CAG repeat sizes influences age at onset of Huntington disease. Am J Med Genet 119A, 279-282.
4. Gusella, J. F. & MacDonald, M. E. (1995) Huntington's disease. Semin Cell Biol 6, 21-28.
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7. Langbehn, D. R., Brinkman, R. R. et al. (2004) A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length. Clin Genet 65, 267-277.
8. Li, J. L., Hayden, M. R. et al. (2003) A Genome Scan for Modifiers of Age at Onset in Huntington Disease: The HD MAPS Study. Am J Hum Genet 73, 682-687.
9. Maltecca, F., Filla, A. et al. (2003) Intergenerational instability and marked anticipation in SCA-17. Neurology 61, 1441-1443.
10. Rivaud-Pechoux, S., Durr, A. et al. (1998) Eye movement abnormalities correlate with genotype in autosomal dominant cerebellar ataxia type I. Ann Neurol 43, 297-302.
11. Wexler, N. S., Lorimer, J. et al. (2004) Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A 101, 3498-3503.