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 ( Wld s ) 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 Wld s on the phenotype of a mouse model of SMA. We found that Wld s does not alter the SMA phenotype, indicating that Wallerian degeneration does not directly contribute to the pathogenesis of SMA development.
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More From: Biochemical and Biophysical Research Communications
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