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Proc Natl Acad Sci U S A. 2010 Nov 23;107(47):20523-8. doi: 10.1073/pnas.1006869107. Epub 2010 Nov 8.

Deficits in axonal transport precede ALS symptoms in vivo.

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

Abstract

ALS is a fatal neurodegenerative disease characterized by selective motor neuron death resulting in muscle paralysis. Mutations in superoxide dismutase 1 (SOD1) are responsible for a subset of familial cases of ALS. Although evidence from transgenic mice expressing human mutant SOD1(G93A) suggests that axonal transport defects may contribute to ALS pathogenesis, our understanding of how these relate to disease progression remains unclear. Using an in vivo assay that allows the characterization of axonal transport in single axons in the intact sciatic nerve, we have identified clear axonal transport deficits in presymptomatic mutant mice. An impairment of axonal retrograde transport may therefore represent one of the earliest axonal pathologies in SOD1(G93A) mice, which worsens at an early symptomatic stage. A deficit in axonal transport may therefore be a key pathogenic event in ALS and an early disease indicator of motor neuron degeneration.

PMID:
21059924
[PubMed - indexed for MEDLINE]
PMCID:
PMC2996651
Free PMC Article
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