Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Striatal Circuit Development and Its Alterations in Huntington’s Disease

Version 1 : Received: 30 May 2020 / Approved: 31 May 2020 / Online: 31 May 2020 (18:20:17 CEST)

A peer-reviewed article of this Preprint also exists.

Journal reference: Neurobiology of Disease 2020, 145, 105076
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 question of when during development do the first disease-related striatal alterations emerge or 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.


striatal development; Huntington’s disease; spiny projection neurons; medium spiny neurons; neuronal excitability; striosomes; matrix; basal ganglia

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