Journal
NEUROBIOLOGY OF DISEASE
Volume 34, Issue 1, Pages 155-162Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2009.01.005
Keywords
Amyotrophic lateral sclerosis; Mitochondria; Cytochrome c oxidase; Transgenic; Motor neuron; Paralysis
Categories
Funding
- NINDS [R01NS055315]
- Muscular Dystrophy Association
- The Horace C. Cabe Foundation
- The Judith and Jean Pape Adams Charitable Foundation
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Over-expression of CCS in G93A SOD1 mice accelerates neurological disease and enhances mitochondrial pathology. We studied the effect of CCS over-expression in transgenic mice expressing G37P, G86R or L126Z SOD1 mutations in order to understand factors which influence mitochondrial dysfunction. Over-expression of CCS markedly decreased survival and produced mitochondrial vacuolation in G37R SOD1 mice but not in G86R or L126Z SOD1 mice. Moreover, CCS/G37R SOD1 spinal cord showed specific reductions in mitochondrial complex IV subunits consistent with an isolated COX deficiency, while no such reductions were detected in CCS/G86R or CCS/L126Z SOD1 mice. CCS over-expression increased the ratio of reduced to oxidized SOD1 monomers in the spinal cords of G37R SOD1 as well as G93A SOD1 mice, but did not influence the redox state of G86R or L126Z SOD1 monomers. The effects of CCS on disease are SOD1 mutation dependent and correlate with SOD1 redox susceptibility. (C) 2009 Elsevier Inc. All rights reserved.
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