Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
Science
; 302(5642): 113-7, 2003 Oct 03.
Article
in En
| MEDLINE
| ID: mdl-14526083
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Spinal Cord
/
Superoxide Dismutase
/
Amyotrophic Lateral Sclerosis
/
Motor Neurons
Limits:
Animals
/
Humans
Language:
En
Journal:
Science
Year:
2003
Document type:
Article
Affiliation country:
United States