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PMID:10433959
| Citation |
Flood, DG, Reaume, AG, Gruner, JA, Hoffman, EK, Hirsch, JD, Lin, YG, Dorfman, KS and Scott, RW (1999) Hindlimb motor neurons require Cu/Zn superoxide dismutase for maintenance of neuromuscular junctions. Am. J. Pathol. 155:663-72 |
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| Abstract |
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. |
| Links |
PubMed PMC1866863 Online version:10.1016/S0002-9440(10)65162-0 |
| Keywords |
Animals; Axons/enzymology; Axons/physiology; Behavior, Animal; Copper/physiology; Culture Techniques; Disease Models, Animal; Electrophysiology; Hindlimb/innervation; Mice; Mice, Knockout; Models, Genetic; Motor Neurons/enzymology; Motor Neurons/physiology; Muscles/anatomy & histology; Muscles/metabolism; Neural Conduction; Neuromuscular Junction/enzymology; Neuromuscular Junction/physiology; Perfusion; Peripheral Nerves/physiology; Promoter Regions, Genetic; Silver Staining; Superoxide Dismutase/physiology; Time Factors; Tissue Distribution; Zinc/physiology |
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Significance
Annotations
| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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