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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.
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