Exploring huntington's disease from a neurodevelopmental perspective

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Authors: Chunhui Huang, Xiao Zheng, Wei Li, Zaijun Zhang, Shihua Li, Xiao-Jiang Li, Mingdeng Rong, Sen Yan
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Abstract

gene. The classic concept is that HD is a degenerative disease that primarily affects the striatum, caused by a gain-of-function mutant mHTT that kills neurons. However, increasing evidence suggests that the effects of mHTT on development may be an alternative view of HD. Therefore, we describe the importance of HTT for neurodevelopment and then summarize the effects of mHTT on neurodevelopment that have been revealed so far in different models. Importantly, we provide new insights into the use of different models to study HD development, and propose new therapeutic strategies for intervening in HD early in development to improve disease progression. Furthermore, we explore potential connections between neurodevelopmental abnormalities and neurodegenerative processes in HD. This review provides a systematic synthesis of current knowledge regarding HD development and pathogenesis.

Fields of Study & Subjects

Genetic Neurodegenerative Diseases Amyotrophic Lateral Sclerosis Research Hereditary Neurological Disorders

Publication details

Journal / source
International Journal of Biological Sciences
Volume
22
Issue
3
Pages
1233–1246
Publication date
1 January 2026
Language
EN