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- Name: A—without Myo—muscle Trophic—nourishment Lateral—side
(of the spinal cord) Sclerosis—hardening or scarring
- Selective death of large caliber axons
- Pathology:
- Neurofilament accumulation
- Genetics
- 2% of cases have SOD1 mutations that probably act through a toxic
property, not loss of activity
- maybe due to misfolding of SOD1
- transfer of toxicity
- mutant cells can transfer toxicity
- wt cells can help mutant cells live longer
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