| 18680723 |
Rose FF Jr, Meehan PW, Coady TH, Garcia VB, Garcia ML, Lorson CL: The Wallerian degeneration slow (Wld (s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy. Biochem Biophys Res Commun. 2008 Oct 10;375(1):119-23. Epub 2008 Aug 3.
Spinal muscular atrophy (SMA) is a severe neuromuscular disease characterized by loss of spinal alpha-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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