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(American Journal of Pathology. 1999;155:663-672.)
© 1999 American Society for Investigative Pathology

Animal Model

Hindlimb Motor Neurons Require Cu/Zn Superoxide Dismutase for Maintenance of Neuromuscular Junctions

Dorothy G. Flood*, Andrew G. Reaume*, John A. Gruner{dagger}, Eric K. Hoffman*, James D. Hirsch*, Yin-Guo Lin*, Karen S. Dorfman* and Richard W. Scott*

From the Departments of Molecular Biology*
and Pharmacology,{dagger}
Cephalon, Inc., West Chester, Pennsylvania

The role of oxidative damage in neurodegenerative disease was investigated in mice lacking cytoplasmic Cu/Zn superoxide dismutase (SOD), created by deletion of the SOD1 gene (SOD1-/-). SOD1-/- mice developed a chronic peripheral hindlimb axonopathy. Mild denervation of muscle was detected at 2 months, and behavioral and physiological motor deficits were present at 5–7 months of age. Ventral root axons were shrunken but were normal in number. The somatosensory system in SOD1-/- mice was mildly affected. SOD1-/- mice expressing Cu/Zn SOD only in brain and spinal cord were generated using transgenic mice expressing mouse SOD1 driven by the neuron-specific synapsin promoter. Neuron-specific expression of Cu/Zn SOD in SOD1-/- mice rescued motor neurons from the neuropathy. Therefore, Cu/Zn SOD is not required for normal motor neuron survival, but is necessary for the maintenance of normal neuromuscular junctions by hindlimb motor neurons.




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Copyright © 1999 by the American Society for Investigative Pathology.