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Neuron type-specific increase in lamin B1 contributes to nuclear dysfunction in Huntington's disease.

Rafael Alcalá-Vida1,2,3, Marta Garcia-Forn1,2,3, Carla Castany-Pladevall1,2,3

  • 1Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia.

EMBO Molecular Medicine
|December 28, 2020
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Summary

Increased lamin B1 protein levels contribute to Huntington's disease (HD) pathology by disrupting nuclear function and transport. Normalizing lamin B1 levels in mice with HD improved neurological function, suggesting a new therapeutic target.

Keywords:
LADR6/1 mousechromatin accessibilitynuclear morphologynuclear permeability

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Lamins are essential nuclear proteins regulating nuclear structure and function.
  • Huntington's disease (HD) is a neurodegenerative disorder caused by CAG repeat expansions, leading to mutant huntingtin protein aggregation.
  • Altered nuclear function and transport are implicated in HD pathogenesis.

Purpose of the Study:

  • To investigate the role of lamin B1 in the pathophysiology of Huntington's disease.
  • To explore lamin B1 as a potential therapeutic target for HD.

Main Methods:

  • Fluorescence-activated nuclear suspension imaging to quantify lamin B1 levels in neuronal nuclei.
  • ChIP-sequencing to analyze lamin-associated chromatin domains and chromatin accessibility.
  • Pharmacological intervention with betulinic acid in a mouse model of HD.

Main Results:

  • Increased lamin B1 levels were observed in neurons of HD models, correlating with nuclear morphology changes and disrupted nucleocytoplasmic transport.
  • ChIP-sequencing revealed altered lamin-associated chromatin domains, changes in chromatin accessibility, and transcriptional dysregulation in HD hippocampi.
  • Betulinic acid treatment normalized lamin B1 levels, restored nuclear homeostasis, and ameliorated motor and cognitive deficits in R6/1 HD mice.

Conclusions:

  • Elevated lamin B1 is a novel pathogenic mechanism in Huntington's disease.
  • Targeting lamin B1 offers a promising therapeutic strategy for HD intervention.
  • Restoring nuclear homeostasis via lamin B1 modulation can alleviate HD-related neurological dysfunction.