Propagation and spread of pathogenic protein assemblies in neurodegenerative diseases.

作者: Mathias Jucker , Lary C. Walker

DOI: 10.1038/S41593-018-0238-6

关键词: ExtracellularDiseaseAmyotrophic lateral sclerosisNervous systemExtramuralIntracellularProtein foldingCell signalingBiologyNeuroscience

摘要: Many neurodegenerative diseases, such as Alzheimer's disease, Parkinson's and amyotrophic lateral sclerosis, are characterized by the progressive appearance of abnormal proteinaceous assemblies in nervous system. Studies experimental systems indicate that originate from prion-like seeded aggregation specific misfolded proteins proliferate amass to form intracellular and/or extracellular lesions typical each disorder. The host which proteopathic seeds arise provides biochemical physiological environment either supports or restricts their emergence, proliferation, self-assembly, spread. Multiple mechanisms influence spatiotemporal spread nature resulting lesions, one is cellular uptake, release, transport along neural pathways networks. characteristics cells regions affected network govern vulnerability thereby neuropathological clinical attributes disease. propagation pathogenic protein within system thus determined interaction agent milieu.

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