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

TitleThe relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset
Authors
KeywordsHuntington's disease
Multiple linear regression
Issue Date2007
PublisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/AHG
Citation
Annals Of Human Genetics, 2007, v. 71 n. 3, p. 295-301 How to Cite?
AbstractAge of onset for Huntington's disease (HD) varies inversely with the length of the disease-causing CAG repeat expansion in the HD gene. A simple exponential regression model yielded adjusted R-squared values of 0.728 in a large set of Venezuelan kindreds and 0.642 in a North American, European, and Australian sample (the HD MAPS cohort). We present evidence that a two-segment exponential regression curve provides a significantly better fit than the simple exponential regression. A plot of natural log-transformed age of onset against CAG repeat length reveals this segmental relationship. This two-segment exponential regression on age of onset data increases the adjusted R-squared values by 0.012 in the Venezuelan kindreds and by 0.035 in the HD MAPS cohort. Although the amount of additional variance explained by the segmental regression approach is modest, the two slopes of the two-segment regression are significantly different from each other in both the Venezuelan kindreds [F(2, 439) =11.13, P =2 × 10 -5] and in the HD MAPS cohort [F(2, 688) =38.27, P = 2 × 10 -16]. In both populations, the influence of each CAG repeat on age of onset appears to be stronger in the adult-onset range of CAG repeats than in the juvenile-onset range. © 2006 The Authors Journal compilation © 2006 University College London.
Persistent Identifierhttp://hdl.handle.net/10722/81495
ISSN
2015 Impact Factor: 1.889
2015 SCImago Journal Rankings: 1.191
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorAndresen, JMen_HK
dc.contributor.authorGayán, Jen_HK
dc.contributor.authorDjoussé, Len_HK
dc.contributor.authorRoberts, Sen_HK
dc.contributor.authorBrocklebank, Den_HK
dc.contributor.authorCherny, SSen_HK
dc.contributor.authorCardon, LRen_HK
dc.contributor.authorGusella, JFen_HK
dc.contributor.authorMacdonald, MEen_HK
dc.contributor.authorMyers, RHen_HK
dc.contributor.authorHousman, DEen_HK
dc.contributor.authorWexler, NSen_HK
dc.contributor.authorLorimer, Jen_HK
dc.contributor.authorPorter, Jen_HK
dc.contributor.authorGomez, Fen_HK
dc.contributor.authorMoskowitz, Cen_HK
dc.contributor.authorGerstenhaber, KPen_HK
dc.contributor.authorShackell, Een_HK
dc.contributor.authorMarder, Ken_HK
dc.contributor.authorPenchaszadeh, Gen_HK
dc.contributor.authorRoberts, SAen_HK
dc.contributor.authorBrickman, Aen_HK
dc.contributor.authorBrocklebank, Den_HK
dc.contributor.authorGray, Jen_HK
dc.contributor.authorDlouhy, SRen_HK
dc.contributor.authorWiktorski, Sen_HK
dc.contributor.authorHodes, MEen_HK
dc.contributor.authorConneally, PMen_HK
dc.contributor.authorPenney, JBen_HK
dc.contributor.authorGusella, JFen_HK
dc.contributor.authorCha, JHen_HK
dc.contributor.authorIrizarry, Men_HK
dc.contributor.authorRosas, Den_HK
dc.contributor.authorHersch, Sen_HK
dc.contributor.authorHollingsworth, Zen_HK
dc.contributor.authorYoung, ABen_HK
dc.contributor.authorHousman, DEen_HK
dc.contributor.authorde Young, MMen_HK
dc.contributor.authorBonilla, Een_HK
dc.contributor.authorStillings, Ten_HK
dc.contributor.authorNegrette, Aen_HK
dc.contributor.authorSnodgrass, SRen_HK
dc.contributor.authorMartinezJaurrieta, MDen_HK
dc.contributor.authorRamosArroyoh, MAen_HK
dc.contributor.authorBickham, Jen_HK
dc.contributor.authorRamos, JSen_HK
dc.contributor.authorMarshall, Fen_HK
dc.contributor.authorShoulson, Ien_HK
dc.contributor.authorRey, GJen_HK
dc.contributor.authorFeigin, Aen_HK
dc.contributor.authorArnheim, Nen_HK
dc.contributor.authorAcevedoCruz, Aen_HK
dc.contributor.authorAcosta, Len_HK
dc.contributor.authorAlvir, Jen_HK
dc.contributor.authorFischbeck, Ken_HK
dc.contributor.authorThompson, LMen_HK
dc.contributor.authorYoung, Aen_HK
dc.contributor.authorDure, Len_HK
dc.contributor.authorO'Brien, CJen_HK
dc.contributor.authorPaulsen, Jen_HK
dc.contributor.authorMoran, SPen_HK
dc.contributor.authorKrch, Den_HK
dc.contributor.authorHogarth, Pen_HK
dc.contributor.authorHiggins Jr, DSen_HK
dc.contributor.authorLandwehrmeyer, Ben_HK
dc.contributor.authorHayden, MRen_HK
dc.contributor.authorAlmqvist, EWen_HK
dc.contributor.authorBrinkman, RRen_HK
dc.contributor.authorSuchowersky, Oen_HK
dc.contributor.authorDurr, Aen_HK
dc.contributor.authorDodé, CCatherineen_HK
dc.contributor.authorSquitieri, Fen_HK
dc.contributor.authorMorrison, PJen_HK
dc.contributor.authorNance, Men_HK
dc.contributor.authorRoss, CAen_HK
dc.contributor.authorMargolis, RLen_HK
dc.contributor.authorRosenblatt, Aen_HK
dc.contributor.authorEstrella, GTen_HK
dc.contributor.authorCabrero, DMen_HK
dc.contributor.authorTrent, RJAen_HK
dc.contributor.authorMcCusker, Een_HK
dc.contributor.authorNovelletto, Aen_HK
dc.contributor.authorFrontali, Men_HK
dc.contributor.authorPaulsen, JSen_HK
dc.contributor.authorJones, Ren_HK
dc.contributor.authorZanko, Aen_HK
dc.contributor.authorAshizawa, Ten_HK
dc.contributor.authorLazzarini, Aen_HK
dc.contributor.authorLi, JLen_HK
dc.contributor.authorWheeler, VCen_HK
dc.contributor.authorRuss, ALen_HK
dc.contributor.authorXu, Gen_HK
dc.contributor.authorMysore, JSen_HK
dc.contributor.authorGillis, Ten_HK
dc.contributor.authorHakky, Men_HK
dc.contributor.authorCupples, LAen_HK
dc.contributor.authorSaintHilaire, Men_HK
dc.contributor.authorHersch, SMen_HK
dc.date.accessioned2010-09-06T08:18:27Z-
dc.date.available2010-09-06T08:18:27Z-
dc.date.issued2007en_HK
dc.identifier.citationAnnals Of Human Genetics, 2007, v. 71 n. 3, p. 295-301en_HK
dc.identifier.issn0003-4800en_HK
dc.identifier.urihttp://hdl.handle.net/10722/81495-
dc.description.abstractAge of onset for Huntington's disease (HD) varies inversely with the length of the disease-causing CAG repeat expansion in the HD gene. A simple exponential regression model yielded adjusted R-squared values of 0.728 in a large set of Venezuelan kindreds and 0.642 in a North American, European, and Australian sample (the HD MAPS cohort). We present evidence that a two-segment exponential regression curve provides a significantly better fit than the simple exponential regression. A plot of natural log-transformed age of onset against CAG repeat length reveals this segmental relationship. This two-segment exponential regression on age of onset data increases the adjusted R-squared values by 0.012 in the Venezuelan kindreds and by 0.035 in the HD MAPS cohort. Although the amount of additional variance explained by the segmental regression approach is modest, the two slopes of the two-segment regression are significantly different from each other in both the Venezuelan kindreds [F(2, 439) =11.13, P =2 × 10 -5] and in the HD MAPS cohort [F(2, 688) =38.27, P = 2 × 10 -16]. In both populations, the influence of each CAG repeat on age of onset appears to be stronger in the adult-onset range of CAG repeats than in the juvenile-onset range. © 2006 The Authors Journal compilation © 2006 University College London.en_HK
dc.languageengen_HK
dc.publisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/AHGen_HK
dc.relation.ispartofAnnals of Human Geneticsen_HK
dc.rightsAnnals of Human Genetics. Copyright © Blackwell Publishing Ltd.en_HK
dc.subjectHuntington's diseaseen_HK
dc.subjectMultiple linear regressionen_HK
dc.titleThe relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onseten_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0003-4800&volume=71&spage=295&epage=301&date=2007&atitle=The+relationship+between+CAG+repeat+length+and+age+of+onset+differs+for+Huntington%27s+Disease+patients+with+juvenile+onset+or+adult+onseten_HK
dc.identifier.emailCherny, SS: cherny@hku.hken_HK
dc.identifier.authorityCherny, SS=rp00232en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1111/j.1469-1809.2006.00335.xen_HK
dc.identifier.pmid17181545-
dc.identifier.scopuseid_2-s2.0-34247120606en_HK
dc.identifier.hkuros128313en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-34247120606&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume71en_HK
dc.identifier.issue3en_HK
dc.identifier.spage295en_HK
dc.identifier.epage301en_HK
dc.identifier.isiWOS:000245691800002-
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dc.identifier.scopusauthoridRoberts, S=7403450675en_HK
dc.identifier.scopusauthoridBrocklebank, D=6603151750en_HK
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dc.identifier.scopusauthoridMyers, RH=35380404100en_HK
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dc.identifier.scopusauthoridHersch, SM=55182864800en_HK
dc.identifier.citeulike1231291-

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