Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis

  title={Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis},
  author={Stefan Br{\"a}uer and Vitaly L Zimyanin and Andreas Hermann},
  journal={Journal of Neural Transmission},
The hallmark of age-related neurodegenerative diseases is the appearance of cellular protein deposits and spreading of this pathology throughout the central nervous system. Growing evidence has shown the involvement and critical role of proteins with prion-like properties in the formation of these characteristic cellular aggregates. Prion-like domains of such proteins with their proposed function in the organization of membraneless organelles are prone for misfolding and promoting further… 

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Central nervous system in motor neuron disease

  • A. Eisen
  • Biology, Psychology
    Journal of Neurology, Neurosurgery, and Psychiatry
  • 2018
The 45 cases described is the second largest series with extensive clinical-pathological correlations reported up to 1970, and when taken together with sophisticated MRI imaging and threshold tracking using transcranial magnetic stimulation convincingly points to a cortical origin of ALS.



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