Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG …

AC Rising, J Xu, A Carlson, VV Napoli… - Experimental …, 2011 - Elsevier
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 …

Time course of early motor and neuropathological anomalies in a knock‐in mouse model of Huntington's disease with 140 CAG repeats

LB Menalled, JD Sison, I Dragatsis… - Journal of …, 2003 - Wiley Online Library
Huntington's disease (HD) is caused by an abnormal expansion of CAG repeats in the gene
encoding huntingtin. The development of therapies for HD requires preclinical testing of …

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

MA Hickey, A Kosmalska, J Enayati, R Cohen, S Zeitlin… - Neuroscience, 2008 - Elsevier
Huntington's disease is a neurodegenerative disorder, caused by an elongation of CAG
repeats in the huntingtin gene. Mice with an insertion of an expanded polyglutamine repeat …

Progressive CAG expansion in the brain of a novel R6/1-89Q mouse model of Huntington's disease with delayed phenotypic onset

SC Vatsavayai, GM Dallérac, AJ Milnerwood… - Brain research …, 2007 - Elsevier
Transgenic models representing Huntington's disease (HD) have proved useful for
understanding the cascade of molecular events leading to the disease. We report an initial …

Striatal atrophy and dendritic alterations in a knock-in mouse model of Huntington's disease

RP Lerner, G Luz del Carmen, C Zhu, MF Chesselet… - Brain research …, 2012 - Elsevier
Huntington's disease (HD) is a progressive neurodegenerative disease characterized by
progressive atrophy of the striatum, cerebral cortex, and white matter tracks. Major …

Behaviour changes in a transgenic model of Huntington's disease

P Klivenyi, Z Bende, Z Hartai, Z Penke… - Behavioural brain …, 2006 - Elsevier
Huntington's disease is an autosomal dominant inherited disorder, caused by an expanded
polyglutamine region of a protein called huntingtin with unknown function. Transgenic mice …

Phenotype onset in Huntington's disease knock‐in mice is correlated with the incomplete splicing of the mutant huntingtin gene

NR Franich, MA Hickey, C Zhu… - Journal of …, 2019 - Wiley Online Library
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by an
expanded CAG repeat within the huntingtin (HTT) gene. The Q140 and HdhQ150 knock‐in …

Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse

RM Cowin, A Roscic, N Bui, D Graham… - Behavioural brain …, 2012 - Elsevier
Huntington's disease (HD) is caused by the expansion of the polyglutamine tract expressed
in the huntingtin protein. Data from patients show a strong negative correlation between …

Lack of association of somatic CAG repeat expansion with striatal neurodegeneration in HD knock-in animal models

D Bai, P Yin, Y Zhang, F Sun, L Chen… - Human Molecular …, 2021 - academic.oup.com
Our previous work has established a huntingtin knock-in (KI) pig model that displays striatal
neuronal loss, allowing us to examine if somatic CAG expansion in striatum accounts for the …

Recent advances in understanding the pathogenesis of Huntington's disease

PH Reddy, M Williams, DA Tagle - Trends in neurosciences, 1999 - cell.com
Huntington's disease (HD) is an autosomal, dominantly inherited neurodegenerative
disorder that is characterized by abnormal involuntary movements (chorea), intellectual …