Wellstone Center for FSHD; Department of Cell and Developmental Biology
Amyotrophic Lateral Sclerosis; Animals; 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
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.
Rights and Permissions
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/).
DOI of Published Version
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 site
The Journal of cell biology
Li, Yun R.; King, Oliver D.; Shorter, James; and Gitler, Aaron D., "Stress granules as crucibles of ALS pathogenesis" (2013). Wellstone Center for FSHD Publications. 6.