| Literature DB >> 10433959 |
D G Flood1, A G Reaume, J A Gruner, E K Hoffman, J D Hirsch, Y G Lin, K S Dorfman, R W Scott.
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.Entities:
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Year: 1999 PMID： 10433959 PMCID： PMC1866863 DOI： 10.1016/S0002-9440(10)65162-0
Source DB: PubMed Journal: Am J Pathol ISSN： 0002-9440 Impact factor: 4.307