Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.

In the present study we report on the use of speed congenics to generate a C57BL/6J congenic line of HD-model R6/2 mice carrying 110 CAG repeats, which uniquely exhibits minimal intergenerational instability. We also report the first identification of the R6/2 transgene insertion site. The relativel...

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Autores principales:	Randi-Michelle Cowin, Nghiem Bui, Deanna Graham, Jennie R Green, Stephan Grueninger, Lisa A Yuva-Paylor, Arsalan U Syed, Andreas Weiss, Richard Paylor
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2011
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/bd5678873d0346399ed7e86622ea635b
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spelling	oai:doaj.org-article:bd5678873d0346399ed7e86622ea635b2021-11-18T07:32:50ZOnset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.1932-620310.1371/journal.pone.0028409https://doaj.org/article/bd5678873d0346399ed7e86622ea635b2011-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22163300/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203In the present study we report on the use of speed congenics to generate a C57BL/6J congenic line of HD-model R6/2 mice carrying 110 CAG repeats, which uniquely exhibits minimal intergenerational instability. We also report the first identification of the R6/2 transgene insertion site. The relatively stable line of 110 CAG R6/2 mice was characterized for the onset of behavioral impairments in motor, cognitive and psychiatric-related phenotypes as well as the progression of disease-related impairments from 4 to 10 weeks of age. 110Q mice exhibited many of the phenotypes commonly associated with the R6/2 model including reduced activity and impairments in rotarod performance. The onset of many of the phenotypes occurred around 6 weeks and was progressive across age. In addition, some phenotypes were observed in mice as early as 4 weeks of age. The present study also reports the onset and progression of changes in several molecular phenotypes in the novel R6/2 mice and the association of these changes with behavioral symptom onset and progression. Data from TR-FRET suggest an association of mutant protein state changes (soluble versus aggregated) in disease onset and progression.Randi-Michelle CowinNghiem BuiDeanna GrahamJennie R GreenStephan GrueningerLisa A Yuva-PaylorArsalan U SyedAndreas WeissRichard PaylorPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 6, Iss 12, p e28409 (2011)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Randi-Michelle Cowin Nghiem Bui Deanna Graham Jennie R Green Stephan Grueninger Lisa A Yuva-Paylor Arsalan U Syed Andreas Weiss Richard Paylor Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
description	In the present study we report on the use of speed congenics to generate a C57BL/6J congenic line of HD-model R6/2 mice carrying 110 CAG repeats, which uniquely exhibits minimal intergenerational instability. We also report the first identification of the R6/2 transgene insertion site. The relatively stable line of 110 CAG R6/2 mice was characterized for the onset of behavioral impairments in motor, cognitive and psychiatric-related phenotypes as well as the progression of disease-related impairments from 4 to 10 weeks of age. 110Q mice exhibited many of the phenotypes commonly associated with the R6/2 model including reduced activity and impairments in rotarod performance. The onset of many of the phenotypes occurred around 6 weeks and was progressive across age. In addition, some phenotypes were observed in mice as early as 4 weeks of age. The present study also reports the onset and progression of changes in several molecular phenotypes in the novel R6/2 mice and the association of these changes with behavioral symptom onset and progression. Data from TR-FRET suggest an association of mutant protein state changes (soluble versus aggregated) in disease onset and progression.
format	article
author	Randi-Michelle Cowin Nghiem Bui Deanna Graham Jennie R Green Stephan Grueninger Lisa A Yuva-Paylor Arsalan U Syed Andreas Weiss Richard Paylor
author_facet	Randi-Michelle Cowin Nghiem Bui Deanna Graham Jennie R Green Stephan Grueninger Lisa A Yuva-Paylor Arsalan U Syed Andreas Weiss Richard Paylor
author_sort	Randi-Michelle Cowin
title	Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
title_short	Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
title_full	Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
title_fullStr	Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
title_full_unstemmed	Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.
title_sort	onset and progression of behavioral and molecular phenotypes in a novel congenic r6/2 line exhibiting intergenerational cag repeat stability.
publisher	Public Library of Science (PLoS)
publishDate	2011
url	https://doaj.org/article/bd5678873d0346399ed7e86622ea635b
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Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability.

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