Pathogenesis of two axonopathies does not require axonal neurofilaments

J. Eyer, D. W. Cleveland, P. C. Wong and A. C. Peterson ()
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J. Eyer: INSERM CJF 97-08 and University of Angers
D. W. Cleveland: Ludwig Institute for Cancer Research, University of California, San Diego
P. C. Wong: Johns Hopkins University School of Medicine
A. C. Peterson: Laboratory of Developmental Biology, Molecular Oncology Group, H-5, Royal Victoria Hospital, McGill University

Nature, 1998, vol. 391, issue 6667, 584-587

Abstract: Abstract Neurofilaments are a major component of the axonal cytoskeleton and their abnormal accumulation is a prominent feature of the cytopathology encountered in several neurodegenerative diseases1,2,3,4,5,6,7,8. Thus, an attractive and widely held model of pathogenesis involves the participation of disrupted neurofilaments as a common toxic intermediate9,10,11,12,13. Here, in direct contrast to this hypothesis, we show that two neurodegenerative disease models in the mouse, dystonia musculorum (dt)14,15 and a superoxide dismutase 1 (SOD1)-mediated form of human motor neuron disease (amyotrophic lateral sclerosis, ALS)16,17, progress with little or no abatement on a transgenic background in which neurofilaments are withheld from the axonal compartment18. By specifically excluding a necessary role for axonal neurofilaments, our observations redefine the components of the pathogenic pathway leading to axon disruption in these two degenerative diseases.

Date: 1998
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DOI: 10.1038/35378

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