Stress granules as crucibles of ALS pathogenesis

Author:

Li Yun R.12,King Oliver D.3,Shorter James1,Gitler Aaron D.2

Affiliation:

1. Medical Scientist Training Program and Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104

2. Department of Genetics, Stanford University School of Medicine, Stanford, CA 94305

3. Department of Cell and Developmental Biology, University of Massachusetts Medical School, Worcester, MA 01655

Abstract

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.

Publisher

Rockefeller University Press

Subject

Cell Biology

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