Effects of Alexander disease–associated mutations on the assembly and organization of GFAP intermediate filaments-Reference-Cited by-同舟云学术

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

Published:2022-07-01 Issue:8 Volume:33 Page:
ISSN:1059-1524
Container-title:Molecular Biology of the Cell
language:en
Short-container-title:MBoC

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

Reference69 articles.

1. A toolbox of IgG subclass-switched recombinant monoclonal antibodies for enhanced multiplex immunolabeling of brain

2. Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages

3. Helix geometry in proteins

4. Alexander disease with serial MRS and a new mutation in the glial fibrillary acidic protein gene

5. Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander disease severity

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