Striatal circuit development and its alterations in Huntington's disease

• Published in: Neurobiology of disease Vol. 145; p. 105076
• Main Authors: Lebouc, Margaux, Richard, Quentin, Garret, Maurice, Baufreton, Jérôme
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2020; Elsevier
• Subjects: Basal ganglia; Huntington's disease; Life Sciences; Matrix; Medium spiny neurons; Neurobiology; Neuronal excitability; Neurons and Cognition; Spiny projection neurons; Striatal development; Striosomes; Striosomes; Basal ganglia; Matrix; Spiny projection neurons; Medium spiny neurons; Huntington's disease; Striatal development; Neuronal excitability
• ISSN: 0969-9961; 1095-953X
• DOI: 10.1016/j.nbd.2020.105076

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.