Effects of Alexander disease–associated mutations on the assembly and organization of GFAP intermediate filaments

Author:

Yang Ai-Wen1,Lin Ni-Hsuan1,Yeh Ting-Hung2,Snider Natasha3,Perng Ming-Der12

Affiliation:

1. Institute of Molecular Medicine, National Tsing Hua University, Hsinchu 30013, Taiwan

2. Department of Medical Science, College of Life Sciences, National Tsing Hua University, Hsinchu 30013, Taiwan

3. Department of Cell Biology and Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599

Abstract

The effects of the GFAP rod end mutations are dominant, changing the assembly process in a way that promotes GFAP aggregation and decreases GFAP solubility. Using Alexander disease brain tissues, clinically relevant evidence is provided that links GFAP assembly defects to disease pathology at the tissue level.

Publisher

American Society for Cell Biology (ASCB)

Subject

Cell Biology,Molecular Biology

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