Roles for RNA-binding proteins in development and disease

Brain Res. 2016 Sep 15:1647:1-8. doi: 10.1016/j.brainres.2016.02.050. Epub 2016 Mar 10.

Abstract

RNA-binding protein activities are highly regulated through protein levels, intracellular localization, and post-translation modifications. During development, mRNA processing of specific gene sets is regulated through manipulation of functional RNA-binding protein activities. The impact of altered RNA-binding protein activities also affects human diseases in which there are either a gain-of-function or loss-of-function causes pathogenesis. We will discuss RNA-binding proteins and their normal developmental RNA metabolism and contrast how their function is disrupted in disease. This article is part of a Special Issue entitled SI:RNA Metabolism in Disease.

Keywords: Development; Neurodegenerative disease; Protein aggregate; Protein mis-regulation; RNA processing; RNA-binding protein.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / metabolism*
  • Animals
  • Brain / growth & development
  • Brain / metabolism
  • CELF1 Protein / metabolism
  • Cell Nucleus / metabolism
  • DNA-Binding Proteins / metabolism
  • Growth and Development*
  • Heart / growth & development
  • Humans
  • Muscle, Skeletal / metabolism
  • Myocardium / metabolism
  • Myotonic Dystrophy / metabolism*
  • RNA / metabolism
  • RNA, Messenger / metabolism
  • RNA-Binding Protein FUS / metabolism
  • RNA-Binding Proteins / metabolism*

Substances

  • CELF1 Protein
  • DNA-Binding Proteins
  • FUS protein, human
  • MBNL1 protein, human
  • RNA, Messenger
  • RNA-Binding Protein FUS
  • RNA-Binding Proteins
  • TARDBP protein, human
  • RNA