Document Type
Journal ArticlePublication Date
2013-04-29Keywords
Amyotrophic Lateral SclerosisAnimals
Cytoplasmic Granules
DNA-Binding Proteins
Environmental Exposure
Humans
Nerve Tissue Proteins
Prions
Protein Structure, Tertiary
RNA-Binding Protein FUS
Stress, Physiological
Cell Biology
Developmental Biology
Molecular Biology
Molecular Genetics
Musculoskeletal Diseases
Nervous System Diseases
Metadata
Show full item recordAbstract
Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the degenerating motor neurons of ALS patients, and mutations in the genes encoding these proteins cause some forms of ALS. TDP-43 and FUS and several related RNA-binding proteins harbor aggregation-promoting prion-like domains that allow them to rapidly self-associate. This property is critical for the formation and dynamics of cellular ribonucleoprotein granules, the crucibles of RNA metabolism and homeostasis. Recent work connecting TDP-43 and FUS to stress granules has suggested how this cellular pathway, which involves protein aggregation as part of its normal function, might be coopted during disease pathogenesis.Source
Li YR, King OD, Shorter J, Gitler AD. Stress granules as crucibles of ALS pathogenesis. J Cell Biol. 2013 Apr 29;201(3):361-72. doi: 10.1083/jcb.201302044. Link to article on publisher's siteDOI
10.1083/jcb.201302044Permanent Link to this Item
http://hdl.handle.net/20.500.14038/50576PubMed ID
23629963Related Resources
Link to Article in PubMedRights
Copyright 2013 Li et al. This article is distributed under the terms of an Attribution–Noncommercial– Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).
ae974a485f413a2113503eed53cd6c53
10.1083/jcb.201302044