Exploring huntington's disease from a neurodevelopmental perspective
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