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Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration.

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Summary

Nucleolar abnormalities occur in C9orf72 frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) brain, showing both smaller and larger nucleoli due to repeat RNA and dipeptide repeat protein toxicity.

Keywords:
C9orf72Dipeptide repeat proteinsFTLDNucleolar stressPoly(GR)RNA foci

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • C9orf72 GGGGCC repeat expansion causes FTLD and ALS.
  • Repeat RNA and dipeptide repeat (DPR) proteins are toxic, potentially affecting nucleolar function.
  • The role of nucleolar stress in C9orf72-associated neurodegeneration is not fully understood.

Purpose of the Study:

  • To investigate nucleolar size and stress in C9orf72-associated FTLD (C9FTLD) patient brains.
  • To determine the impact of poly(GR) inclusions and GGGGCC repeat RNA on nucleolar volume.

Main Methods:

  • Volumetric analysis of nucleolar size in C9FTLD frontal cortex neurons.
  • Assessment of poly(GR) inclusions and GGGGCC RNA foci in patient neurons.
  • Expression of poly(GR) in Drosophila neurons to model effects.

Main Results:

  • C9FTLD neurons exhibited bidirectional nucleolar stress: overall smaller nucleoli but enlarged nucleoli in neurons with poly(GR) inclusions.
  • Poly(GR) expression in Drosophila neurons led to enlarged nucleoli.
  • C9FTLD neurons with GGGGCC RNA foci showed a slight increase in nucleolar volume.

Conclusions:

  • Nucleolar abnormalities are a consistent feature in C9FTLD brains.
  • Both DPR proteins and repeat RNA contribute to nucleolar dysfunction through distinct mechanisms.
  • Bidirectional nucleolar stress highlights the complex pathogenesis of C9orf72 neurodegeneration.