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Expanding the Toolkit for In Vivo Imaging of Axonal Transport
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Expanding the Toolkit for In Vivo Imaging of Axonal Transport

Published on: December 23, 2021

Deficits in axonal transport precede ALS symptoms in vivo

Lynsey G Bilsland1, Erik Sahai, Gavin Kelly

  • 1Molecular NeuroPathobiology Laboratory, Cancer Research UK London Research Institute, London WC2A 3LY, United Kingdom.

Proceedings of the National Academy of Sciences of the United States of America
|November 10, 2010
PubMed

Insights

Axonal transport deficits, particularly retrograde transport, are early indicators of motor neuron degeneration in familial Amyotrophic Lateral Sclerosis (ALS) caused by SOD1 mutations. These impairments worsen as the disease progresses.

Area of Science:

  • Neuroscience
  • Neurodegenerative Diseases
  • Motor Neuron Disorders

Background:

  • Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease causing motor neuron death and paralysis.
  • Mutations in superoxide dismutase 1 (SOD1) are linked to familial ALS cases.
  • Axonal transport defects are suspected contributors to ALS pathogenesis, but their role in disease progression is not fully understood.

Purpose of the Study:

  • To investigate the role and timing of axonal transport deficits in the pathogenesis of ALS.
  • To characterize axonal transport in presymptomatic and symptomatic stages of SOD1 mutant mouse models.

Main Methods:

  • Utilized an in vivo assay to analyze axonal transport in single axons within the intact sciatic nerve.
  • Examined axonal transport in presymptomatic and early symptomatic stages of mice expressing human mutant SOD1(G93A).

Main Results:

  • Identified significant axonal transport deficits in presymptomatic SOD1(G93A) mutant mice.
  • Observed an impairment of axonal retrograde transport as an early pathological event.
  • Demonstrated that these transport deficits worsen during the early symptomatic stage of the disease.

Conclusions:

  • Impaired axonal retrograde transport is a key early pathogenic event in SOD1-linked ALS.
  • Axonal transport deficits serve as an early indicator of motor neuron degeneration in ALS.
  • Understanding these early defects may offer therapeutic targets for slowing ALS progression.

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