Dynamic duo - FMRP and TDP-43: Regulating common targets, causing different diseases.

Brain Res

Department of Molecular and Cellular Biology, University of Arizona, Tucson, AZ, United States; Department of Neuroscience, University of Arizona, Tucson, AZ, United States; Department of Neurology, University of Arizona, Tucson AZ, United States. Electronic address:

Published: August 2018

RNA binding proteins play essential roles during development and aging, and are also involved in disease pathomechanisms. RNA sequencing and omics analyses have provided a window into systems level alterations in neurological disease, and have identified RNA processing defects among notable disease mechanisms. This review focuses on two seemingly distinct neurological disorders, the RNA binding proteins they are linked to, and their newly discovered functional relationship. When deficient, Fragile X Mental Retardation Protein (FMRP) causes developmental deficits and autistic behaviors while TAR-DNA Binding Protein (TDP-43) dysregulation causes age dependent neuronal degeneration. Recent findings that FMRP and TDP-43 associate in ribonuclear protein particles and share mRNA targets in neurons highlight the critical importance of translation regulation in synaptic plasticity and provide new perspectives on neuronal vulnerability during lifespan.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5997554PMC
http://dx.doi.org/10.1016/j.brainres.2018.04.034DOI Listing

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