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Exploring huntington's disease from a neurodevelopmental perspective.

Chunhui Huang1, Xiao Zheng1, Wei Li1

  • 1The Sixth Affiliated Hospital of Jinan University, Dongguan, 523710, China; State Key Laboratory of Bioactive Molecules and Druggability Assessment, Guangdong Basic Research Center of Excellence for Natural Bioactive Molecules and Discovery of Innovative Drugs, Guangdong Provincial Key Laboratory of Non-human Primate Research, Guangdong-Hong Kong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China; Stomatological Hospital, School of Stomatology, Southern Medical University, Guangzhou, 510280, China.

International Journal of Biological Sciences
|January 29, 2026
PubMed
Summary
This summary is machine-generated.

Huntington's disease (HD) may stem from developmental issues, not just neuron death. Targeting mutant huntingtin (HTT) during neurodevelopment offers new therapeutic avenues for this inherited neurodegenerative disorder.

Keywords:
HTTHuntington's diseaseanimal modelbrain organoidsearly interventionmHTTneurodevelopment

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Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Biology

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder caused by mutations in the huntingtin (HTT) gene.
  • The traditional view of HD focuses on mutant huntingtin (mHTT) causing neuron death in the striatum.
  • Emerging evidence suggests mHTT's impact on neurodevelopment offers an alternative perspective on HD pathogenesis.

Purpose of the Study:

  • To review the role of HTT in neurodevelopment.
  • To summarize the effects of mHTT on neurodevelopment across various models.
  • To propose novel therapeutic strategies targeting early developmental interventions in HD.

Main Methods:

  • Literature review synthesizing current knowledge on HD development and pathogenesis.
  • Analysis of findings from diverse models investigating mHTT's impact on neurodevelopment.
  • Exploration of connections between neurodevelopmental abnormalities and neurodegenerative processes in HD.

Main Results:

  • The huntingtin protein (HTT) is crucial for normal neurodevelopment.
  • Mutant HTT (mHTT) significantly disrupts neurodevelopmental processes.
  • Different models reveal varied effects of mHTT on developing neural systems.

Conclusions:

  • Rethinking HD as a neurodevelopmental disorder opens new therapeutic possibilities.
  • Early intervention during development may alter HD progression.
  • Understanding neurodevelopmental deficits is key to addressing HD pathogenesis.