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Striatal circuit development and its alterations in Huntington's disease.

Authors
  • Lebouc, Margaux1
  • Richard, Quentin1
  • Garret, Maurice2
  • Baufreton, Jérôme3
  • 1 Université de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France; CNRS, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France. , (France)
  • 2 Université de Bordeaux, Institut des Neurosciences Cognitives et Intégratives d'Aquitaine, UMR 5287, F-33000 Bordeaux, France; CNRS, Institut des Neurosciences Cognitives et Intégratives d'Aquitaine, UMR 5287, F-33000 Bordeaux, France. Electronic address: [email protected] , (France)
  • 3 Université de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France; CNRS, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France. Electronic address: [email protected] , (France)
Type
Published Article
Journal
Neurobiology of Disease
Publisher
Elsevier
Publication Date
Sep 05, 2020
Pages
105076–105076
Identifiers
DOI: 10.1016/j.nbd.2020.105076
PMID: 32898646
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Huntington's disease (HD) is an inherited neurodegenerative disorder that usually starts during midlife with progressive alterations of motor and cognitive functions. The disease is caused by a CAG repeat expansion within the huntingtin gene leading to severe striatal neurodegeneration. Recent studies conducted on pre-HD children highlight early striatal developmental alterations starting as soon as 6 years old, the earliest age assessed. These findings, in line with data from mouse models of HD, raise the questions of when during development do the first disease-related striatal alterations emerge and whether they contribute to the later appearance of the neurodegenerative features of the disease. In this review we will describe the different stages of striatal network development and then discuss recent evidence for its alterations in rodent models of the disease. We argue that a better understanding of the striatum's development should help in assessing aberrant neurodevelopmental processes linked to the HD mutation. Copyright © 2019. Published by Elsevier Inc.

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