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

Diana Ferro, Stephen Yao, Daniela C Zarnescu

Research output: Contribution to journalArticle

2 Scopus citations

Abstract

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.

Original languageEnglish (US)
JournalBrain Research
DOIs
Publication statusAccepted/In press - Jan 1 2018

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Keywords

  • Amyotrophic lateral sclerosis
  • FMRP
  • Fragile X syndrome
  • Frontotemporal dementia
  • mRNA targets
  • RNA binding proteins
  • TDP-43

ASJC Scopus subject areas

  • Neuroscience(all)
  • Molecular Biology
  • Clinical Neurology
  • Developmental Biology

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