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. doi: 10.1016/j.bbrc.2008.07.130. Epub 2008 Aug 3.

Abstract

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Animals
  • Anterior Horn Cells / pathology
  • Disease Models, Animal
  • Disease Progression
  • Genes, Dominant*
  • Mice
  • Mice, Mutant Strains
  • Muscular Atrophy, Spinal / etiology*
  • Muscular Atrophy, Spinal / pathology
  • Nerve Tissue Proteins / genetics*
  • Sciatic Nerve / pathology
  • Spinal Nerve Roots / pathology
  • Wallerian Degeneration / complications
  • Wallerian Degeneration / genetics*

Substances

  • Nerve Tissue Proteins
  • Wld protein, mouse