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A developmental component to Huntington's disease.

L Ratié1, S Humbert2

  • 1U1216, CEA, Grenoble Institute Neurosciences, Inserm, université Grenoble Alpes, 38000 Grenoble, France.

Revue Neurologique
|April 13, 2024
PubMed
Summary
This summary is machine-generated.

Huntington's disease may impact neurodevelopment, affecting brain development before symptoms appear. Studies show altered cortical development in affected mice and human gene carriers, suggesting developmental origins for this neurodegenerative disorder.

Keywords:
Cortical circuitsCortical developmentHuntingtinHuntington's disease

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Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Biology

Background:

  • Huntington's disease is a dominant inherited neurodegenerative disorder.
  • Characterized by cortical and striatal neuron dysfunction and death.
  • Striatal degeneration is linked to impaired cortical signaling.

Purpose of the Study:

  • To explore the potential impact of Huntington's disease on neurodevelopment.
  • To examine evidence suggesting developmental alterations in Huntington's disease.
  • To discuss the contribution of abnormal brain development to disease onset.

Main Methods:

  • Review of mouse models of Huntington's disease.
  • Analysis of neuroimaging studies in presymptomatic human carriers.
  • Examination of developmental studies in affected animal models and human fetuses.

Main Results:

  • Presymptomatic Huntington's disease may affect neurodevelopment.
  • Altered cortical development observed in Huntington's disease mouse models.
  • Developmental changes noted in fetuses of human Huntington's disease gene carriers.

Conclusions:

  • Neurodevelopmental alterations may contribute to Huntington's disease pathogenesis.
  • Abnormal brain development could underlie the late manifestation of Huntington's disease.
  • Further research into developmental aspects is crucial for understanding and potentially treating Huntington's disease.