Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
Howard Hughes Medical Institute · Ludwig Cancer Research · +4 more institutions
Abstract
The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1). In chimeric mice that are mixtures of normal and SOD1 mutant-expressing cells, toxicity to motor neurons is shown to require damage from mutant SOD1 acting within nonneuronal cells. Normal motor neurons in SOD1 mutant chimeras develop aspects of ALS pathology. Most important, nonneuronal cells that do not express mutant SOD1 delay degeneration and significantly extend survival of mutant-expressing motor neurons.
Citation impact
- FWCI
- 43.97
- Percentile
- 100%
- References
- 36
Authors
14- AMAlbrecht M. ClementCorresponding
Howard Hughes Medical Institute, Ludwig Cancer Research, Montreal General Hospital, University of California San Diego, Ludwig Cancer Research
- MDMinh Dang NguyenCorresponding
Howard Hughes Medical Institute, Ludwig Cancer Research, Montreal General Hospital, University of California San Diego, Ludwig Cancer Research
- EAElizabeth A. Roberts
Howard Hughes Medical Institute, Ludwig Cancer Research, Montreal General Hospital, University of California San Diego, Ludwig Cancer Research
- MLMichael L. Garcia
Howard Hughes Medical Institute, Ludwig Cancer Research, Montreal General Hospital, University of California San Diego, Ludwig Cancer Research
- SBSéverine Boillée
Howard Hughes Medical Institute, Ludwig Cancer Research, Montreal General Hospital, University of California San Diego, Ludwig Cancer Research
Topics & keywords
- SOD1
- Amyotrophic lateral sclerosis
- Mutant
- Motor neuron
- Cell biology
- Biology
- Superoxide dismutase
- Wild type
- Good health and well-being