Axonal Transport and Local Translation of mRNA in Amyotrophic Lateral Sclerosis
Main Article Content
ABSTRACT
Since neurons have long neurites, especially axons, the transport of essential mRNAs, and their translation locally in axons, are essential to maintain the shape and function of the neurons. The RNA-binding protein TDP 43 (transactive response DNA binding protein 43) plays a crucial role in the transport and translation of mRNAs in neurons. In amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), TDP-43 and other RNA-binding proteins are mis-localized and abnormally deposited in neurons. Mutations of genes regulating these proteins have been identified in clinical cases. Impaired mRNA transport system may be a contributing factor of neurodegeneration in ALS/FTLD. In this chapter, we outline the role of RNA-binding proteins, with emphasis on TDP-43, in axonal transport and local translation of mRNAs in ALS/FTLD.
Downloads
Metrics
Article Details
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright of individual chapters belongs to the respective authors. The authors grant unrestricted publishing and distribution rights to the publisher. The electronic versions of the chapters are published under Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). Users are allowed to share and adapt the chapters for any non-commercial purposes as long as the authors and the publisher are explicitly identified and properly acknowledged as the original source. The books in their entirety are subject to copyright by the publisher. The reproduction, modification, republication and display of the books in their entirety, in any form, by anyone, for commercial purposes are strictly prohibited without the written consent of the publisher.