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The Wallerian degeneration slow (Wld(s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy |
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| Authors: | Rose Ferrill F Meehan Philip W Coady Tristan H Garcia Virginia B Garcia Michael L Lorson Christian L |
| Institution: | a Department of Veterinary Pathobiology, Life Sciences Center, University of Missouri, Room 471G, Columbia, MO 65211, USA b Department of Molecular Microbiology and Immunology, University of Missouri, Columbia, MO, USA c Department of Biology, University of Missouri, Columbia, MO, USA |
| Abstract: | Spinal muscular atrophy (SMA) is a severe neuromuscular disease characterized by loss of spinal α-motor neurons, resulting in the paralysis of skeletal muscle. SMA is caused by deficiency of survival motor neuron (SMN) protein levels. Recent evidence has highlighted an axon-specific role for SMN protein, raising the possibility that axon degeneration may be an early event in SMA pathogenesis. The Wallerian degeneration slow (Wlds) gene is a spontaneous dominant mutation in mice that delays axon degeneration by approximately 2-3 weeks. We set out to examine the effect of Wlds on the phenotype of a mouse model of SMA. We found that Wlds does not alter the SMA phenotype, indicating that Wallerian degeneration does not directly contribute to the pathogenesis of SMA development. |
| Keywords: | Wlds Spinal muscular atrophy Axon Wallerian degeneration Survival motor neuron SMN |
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