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Author Notes:

Correspondence: mpinter@emory.edu, Martin J. Pinter

Conceived and designed the experiments: DC MP.

Performed the experiments: DC KS.

Analyzed the data: DC EB MP.

Wrote the paper: MP.

The authors wish to thank Drs. Mark Rich, Robert W. Burgess and Gregory A. Cox for helpful discussions while the manuscript was in preparation and Kate Miers for Western blot analysis.

The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

The authors have declared that no competing interests exist.

Subjects:

Research Funding:

This work was supported by National Institutes of Health (NIH) grant NS31621 (M.J.P.) and by generous funding provided by the ALS Association (K.L.S.).

Nerve Terminal Degeneration Is Independent of Muscle Fiber Genotype in SOD1G93A Mice

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Journal Title:

PLoS ONE

Volume:

Volume 5, Number 3

Publisher:

, Pages e9802-e9802

Type of Work:

Article | Final Publisher PDF

Abstract:

Background Motor neuron degeneration in SOD1G93A transgenic mice begins at the nerve terminal. Here we examine whether this degeneration depends on expression of mutant SOD1 in muscle fibers. Methodology/Principal Findings Hindlimb muscles were transplanted between wild-type and SOD1G93A transgenic mice and the innervation status of neuromuscular junctions (NMJs) was examined after 2 months. The results showed that muscles from SOD1G93A mice did not induce motor terminal degeneration in wildtype mice and that muscles from wildtype mice did not prevent degeneration in SOD1G93A transgenic mice. Control studies demonstrated that muscles transplanted from SOD1G93A mice continued to express mutant SOD1 protein. Experiments on wildtype mice established that the host supplied terminal Schwann cells (TSCs) at the NMJs of transplanted muscles. Conclusions/Significance These results indicate that expression of the mutant protein in muscle is not needed to cause motor terminal degeneration in SOD1G93A transgenic mice and that a combination of motor terminals, motor axons and Schwann cells, all of which express mutant protein may be sufficient.

Copyright information:

Copyright Carrasco et al.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 3.0 Unported License (http://creativecommons.org/licenses/by/3.0/).

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