Journal Article (Review Article)

DZNE-2020-06553

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Propagation and spread of pathogenic protein assemblies in neurodegenerative diseases.

Jucker, M. (First author)DZNE* ; Walker, L. C. (Last author)DZNE*

2018
Nature Publ. Group58142 London

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Please use a persistent id in citations: doi:10.1038/s41593-018-0238-6

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

Keyword(s): Animals (MeSH) ; Cell Communication (MeSH) ; Humans (MeSH) ; Neurodegenerative Diseases: metabolism (MeSH) ; Neurodegenerative Diseases: pathology (MeSH) ; Prions: metabolism (MeSH) ; Prions: pathogenicity (MeSH) ; Prions

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Contributing Institute(s):

1. Cell Biology of Neurologic Diseases (AG Jucker)
2. Tübingen Pre 2020 (Tübingen Pre 2020)

Research Program(s):

1. 342 - Disease Mechanisms and Model Systems (POF3-342) (POF3-342)

Appears in the scientific report 2018

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Database coverage:
; BIOSIS Previews ; BIOSIS Reviews Reports And Meetings ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; Ebsco Academic Search ; IF >= 30 ; JCR ; Nationallizenz ; SCOPUS ; Web of Science Core Collection

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