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 …
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 …
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
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 …
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
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 …
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 …
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 …
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 …
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 …
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 …
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 …
disorder that is characterized by abnormal involuntary movements (chorea), intellectual …
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