ArticleExperimental neurology2011
Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats.
Article in Experimental neurology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 32 citations in OpenAlex.
- Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse.Proceedings of the National Academy of Sciences of the United States of America · 2011Trial
- Longitudinal multimodal MRI characterization of a knock-in mouse model of Huntington's disease reveals early gray and white matter alterations.Human molecular genetics · 2022Article
- Accumulation of Endogenous Mutant Huntingtin in Astrocytes Exacerbates Neuropathology of Huntington Disease in Mice.Molecular neurobiology · 2021Article
- Progression of basal ganglia pathology in heterozygous Q175 knock-in Huntington's disease mice.The Journal of comparative neurology · 2021Article
- Huntington's disease mouse models: unraveling the pathology caused by CAG repeat expansion.Faculty reviews · 2021Review
- Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene.Journal of neuroscience research · 2019Article
- Disrupted striatal neuron inputs and outputs in Huntington's disease.CNS neuroscience & therapeutics · 2018Review
- Cell-Specific Deletion of PGC-1α from Medium Spiny Neurons Causes Transcriptional Alterations and Age-Related Motor Impairment.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2018Article
- Cholinergic interneurons in the Q140 knockin mouse model of Huntington's disease: Reductions in dendritic branching and thalamostriatal input.The Journal of comparative neurology · 2016Article
- A Longitudinal Motor Characterisation of the HdhQ111 Mouse Model of Huntington's Disease.Journal of Huntington's disease · 2016Article
- Article
- Use of a force-sensing automated open field apparatus in a longitudinal study of multiple behavioral deficits in CAG140 Huntington's disease model mice.Behavioural brain research · 2015Article
- Differential loss of thalamostriatal and corticostriatal input to striatal projection neuron types prior to overt motor symptoms in the Q140 knock-in mouse model of Huntington's disease.Frontiers in systems neuroscience · 2014Article
- Loss of corticostriatal and thalamostriatal synaptic terminals precedes striatal projection neuron pathology in heterozygous Q140 Huntington's disease mice.Neurobiology of disease · 2013Article
- Long-term analyses of innervation and neuromuscular integrity in the Trembler-J mouse model of Charcot-Marie-Tooth disease.Journal of neuropathology and experimental neurology · 2013Article
- A broad phenotypic screen identifies novel phenotypes driven by a single mutant allele in Huntington's disease CAG knock-in mice.PloS one · 2013Article
- Mouse models of polyglutamine diseases: review and data table. Part I.Molecular neurobiology · 2012Review
- Dysregulation of dopamine receptor D2 as a sensitive measure for Huntington disease pathology in model mice.Proceedings of the National Academy of Sciences of the United States of America · 2012Article
- Abnormal burst patterns of single neurons recorded in the substantia nigra reticulata of behaving 140 CAG Huntington's disease mice.Neuroscience letters · 2012Article
- Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175.PloS one · 2012Article
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
Abstract
The discovery of the gene mutation responsible for Huntington's disease (HD), huntingtin, in 1993 allowed for a better understanding of the pathology of and enabled the development of animal models. HD is caused by the expansion of a polyglutamine repeat region in the N-terminal of the huntingtin protein. Here we examine the behavioral, transcriptional, histopathological and anatomical characteristics of a knock-in HD mouse model with a 140 polyglutamine expansion in the huntingtin protein. This CAG 140 model contains a portion of the human exon 1 with 140 CAG repeats knocked into the mouse huntingtin gene. We have longitudinally examined the rearing behavior, accelerating rotarod, constant speed rotarod and gait for age-matched heterozygote, homozygote and non-transgenic mice and have found a significant difference in the afflicted mice. However, while there were significant differences between the non-transgenic and the knock-in mice, these behaviors were not progressive. As in HD, we show that the CAG 140 mice also have a significant decrease in striatally enriched mRNA transcripts. In addition, striatal neuronal intranuclear inclusion density increases with age. Lastly these CAG 140 mice show slight cortical thinning compared to non-transgenic mice, similarly to the cortical thinning recently reported in HD.
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