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FUS-related proteinopathies: lessons from animal models

Nicholas A Lanson1, Udai Bhan Pandey

  • 1Department of Genetics, Louisiana State University Health Sciences Center, New Orleans, LA 70112-2223, USA.

Brain Research
|February 21, 2012
PubMed

Insights

Mutations in FUS and TDP-43 are linked to neurodegenerative diseases like ALS and FTLD. This review explores how FUS protein dysfunction, particularly RNA-mediated effects, contributes to these conditions.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are linked to mutations in FUS and TDP-43.
  • RNA-mediated proteinopathy is an emerging mechanism in neurodegenerative disorders.
  • FUS and TDP-43 are structurally and functionally similar DNA/RNA binding proteins.

Purpose of the Study:

  • To review recent advances in understanding FUS protein functions.
  • To discuss insights from FUS animal models regarding ALS pathogenesis.
  • To explore how ALS-linked mutations affect FUS functions.

Main Methods:

  • Literature review of FUS functions and related research.
  • Analysis of findings from FUS animal models.
  • Discussion of molecular mechanisms in ALS pathogenesis.

Main Results:

  • FUS plays a role in RNA metabolism, including splicing and microRNA biogenesis.
  • ALS-linked mutations are suspected to perturb normal FUS functions.
  • FUS animal models offer key insights into disease mechanisms.

Conclusions:

  • Understanding FUS protein function is crucial for elucidating ALS pathogenesis.
  • RNA-mediated mechanisms are increasingly recognized in neurodegeneration.
  • Further research into FUS dysfunction may reveal therapeutic targets for ALS and FTLD.

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