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 …

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 …

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 …

Transcriptional regulatory networks underlying gene expression changes in Huntington's disease

SA Ament, JR Pearl, JP Cantle, RM Bragg… - Molecular systems …, 2018 - embopress.org
Transcriptional changes occur presymptomatically and throughout Huntington's disease
(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 …

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 …

Early alteration of epigenetic-related transcription in Huntington's disease mouse models

I Hervás-Corpión, D Guiretti, M Alcaraz-Iborra… - Scientific reports, 2018 - nature.com
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 …

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 …

High-throughput functional analysis distinguishes pathogenic, nonpathogenic, and compensatory transcriptional changes in neurodegeneration

I Al-Ramahi, B Lu, S Di Paola, K Pang, M de Haro… - Cell systems, 2018 - cell.com
Discriminating transcriptional changes that drive disease pathogenesis from nonpathogenic
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 …