Disrupted striatal neuron inputs and outputs in Huntington's disease
A Reiner, YP Deng - CNS neuroscience & therapeutics, 2018 - Wiley Online Library
Huntington's disease (HD) is a hereditary progressive neurodegenerative disorder caused
by a CAG repeat expansion in the gene coding for the protein huntingtin, resulting in a …
by a CAG repeat expansion in the gene coding for the protein huntingtin, resulting in a …
Huntington disease as a neurodevelopmental disorder and early signs of the disease in stem cells
K Wiatr, WJ Szlachcic, M Trzeciak, M Figlerowicz… - Molecular …, 2018 - Springer
Huntington disease (HD) is a dominantly inherited disorder caused by a CAG expansion
mutation in the huntingtin (HTT) gene, which results in the HTT protein that contains an …
mutation in the huntingtin (HTT) gene, which results in the HTT protein that contains an …
Mouse models of Huntington's disease
PP Farshim, GP Bates - Huntington's disease, 2018 - Springer
The identification of the mutation causing Huntington's disease (HD) has led to the
generation of a large number of mouse models. These models are used to further enhance …
generation of a large number of mouse models. These models are used to further enhance …
Transcriptional regulatory networks underlying gene expression changes in Huntington's disease
Transcriptional changes occur presymptomatically and throughout Huntington's disease
(HD), motivating the study of transcriptional regulatory networks (TRN s) in HD. We …
(HD), motivating the study of transcriptional regulatory networks (TRN s) in HD. We …
Alteration of GABA ergic neurotransmission in Huntington's disease
M Garret, Z Du, M Chazalon, YH Cho… - CNS Neuroscience & …, 2018 - Wiley Online Library
Hereditary Huntington's disease (HD) is characterized by cell dysfunction and death in the
brain, leading to progressive cognitive, psychiatric, and motor impairments. Despite …
brain, leading to progressive cognitive, psychiatric, and motor impairments. Despite …
Molecular insights into cortico-striatal miscommunications in Huntington's disease
MB Veldman, XW Yang - Current opinion in neurobiology, 2018 - Elsevier
Highlights•Diverse mouse models of HD have important phenotypic and molecular
similarities.•Disconnectivity of the cortico-striatal synapse drives the disease …
similarities.•Disconnectivity of the cortico-striatal synapse drives the disease …
Early alteration of epigenetic-related transcription in Huntington's disease mouse models
Transcriptional dysregulation in Huntington's disease (HD) affects the expression of genes
involved in survival and neuronal functions throughout the progression of the pathology. In …
involved in survival and neuronal functions throughout the progression of the pathology. In …
Perturbations in the p53/miR-34a/SIRT1 pathway in the R6/2 Huntington's disease model
RH Reynolds, MH Petersen, CW Willert… - Molecular and Cellular …, 2018 - Elsevier
The three factors, p53, the microRNA-34 family and Sirtuin 1 (SIRT1), interact in a positive
feedback loop involved in cell cycle progression, cellular senescence and apoptosis. Each …
feedback loop involved in cell cycle progression, cellular senescence and apoptosis. Each …
High-throughput functional analysis distinguishes pathogenic, nonpathogenic, and compensatory transcriptional changes in neurodegeneration
Discriminating transcriptional changes that drive disease pathogenesis from nonpathogenic
and compensatory responses is a daunting challenge. This is particularly true for …
and compensatory responses is a daunting challenge. This is particularly true for …
The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin
L Galvan, L Francelle, MC Gaillard, L de Longprez… - Brain, 2018 - academic.oup.com
The neurobiological functions of a number of kinases expressed in the brain are unknown.
Here, we report new findings on DCLK3 (doublecortin like kinase 3), which is preferentially …
Here, we report new findings on DCLK3 (doublecortin like kinase 3), which is preferentially …