The Wallerian degeneration slow (Wld(s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy.
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.
Rose
Ferrill F
FF
Department of Veterinary Pathobiology, Life Sciences Center, University of Missouri, Room 471G, Columbia, MO 65211, USA.
Meehan
Philip W
PW
Coady
Tristan H
TH
Garcia
Virginia B
VB
Garcia
Michael L
ML
Lorson
Christian L
CL
D000818:Animals; D000870:Anterior Horn Cells / Q000473:pathology; D004195:Disease Models, Animal; D018450:Disease Progression; D005799:Genes, Dominant; D051379:Mice; D008817:Mice, Mutant Strains; D009134:Muscular Atrophy, Spinal / Q000209:etiology* / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000235:genetics*; D012584:Sciatic Nerve / Q000473:pathology; D013126:Spinal Nerve Roots / Q000473:pathology; D014855:Wallerian Degeneration / Q000150:complications / Q000235:genetics*
D009419:Nerve Tissue Proteins; C442580:Wld protein, mouse