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

  title={Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis},
  author={S. Br{\"a}uer and V. Zimyanin and A. Hermann},
  journal={Journal of Neural Transmission},
  • S. Bräuer, V. Zimyanin, A. Hermann
  • Published 2018
  • Biology, Medicine
  • 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… CONTINUE READING
    9 Citations

    Topics from this paper.

    Paper Mentions

    The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD
    • 30
    • PDF
    Human Spinal Motor Neurons Are Particularly Vulnerable to Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients
    • 2
    • PDF
    Defining novel functions for cerebrospinal fluid in ALS pathophysiology
    Central nervous system in motor neuron disease
    • A. Eisen
    • Medicine
    • Journal of Neurology, Neurosurgery, and Psychiatry
    • 2018
    • 1
    • PDF
    The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?
    • 40
    ODiNPred: comprehensive prediction of protein order and disorder


    Prion-like activity of Cu/Zn superoxide dismutase
    • 51
    • Highly Influential
    The Seeds of Neurodegeneration: Prion-like Spreading in ALS
    • 267
    • Highly Influential
    • PDF
    Serial propagation of distinct strains of Aβ prions from Alzheimer’s disease patients
    • 210
    • PDF
    In vitro prion-like behaviour of TDP-43 in ALS
    • 60
    • Highly Influential
    • PDF
    RNA-binding proteins with prion-like domains in ALS and FTLD-U
    • 127
    • PDF
    Structural and functional characterization of two alpha-synuclein strains
    • 455
    • PDF
    Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
    • 409
    • PDF
    Amyotrophic lateral sclerosis—a model of corticofugal axonal spread
    • 278
    • Highly Influential