A meta-analysis study of sod1-mutant mouse models of als to analyse the determinants of disease onset and progression

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SUMMARY

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterised by selective loss of both upper and lower motor neurons (MNs), leading to respiratory failure within 2-5 years from symptom onset. Recent studies suggest that the development of ALS is the final product of a step-bystep pathological process, supporting the idea that a number of sequential steps need to arise before patients manifest the disease. This hypothesis was tested by a recent population-based modelling study conducted on Italian and Irish ALS registers, concluding that patient cohorts with a known ALS-causing . . .

     

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