Institut de Neurosciences Cognitives et Intégratives d'Aquitaine (UMR5287)

Aquitaine Institute for Cognitive and Integrative Neuroscience



INCIA - UMR 5287- CNRS
Université de Bordeaux

Zone nord Bat 2 2ème étage
146, rue Léo Saignat
33076 Bordeaux cedex
France

Téléphone 05.57.57.15.51
Télécopie 05.56.90.14.21

Supervisory authorities

CNRS Ecole Pratique des Hautes Etudes Université de Bordeaux

Our partners

Neurocampus Unitéde Formation de Biologie

GDR

GDR Robotique GDR Mémoire GDR Multi-électrodes

Search




Home > Latest publications

Striatal circuit development and its alterations in Huntington’s \ Margaux Lebouc 1, Quentin Richard 1, Maurice Garret 2, Jérôme Baufreton 3 disease

by Loïc Grattier - published on

https://pubmed-ncbi-nlm-nih-gov.insb.bib.cnrs.fr/32898646/

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.

Keywords: Basal ganglia; Huntington’s disease; Matrix; Medium spiny neurons; Neuronal excitability; Spiny projection neurons; Striatal development; Striosomes.