TDP-43 accelerates deadenylation of target mRNAs by recruiting Caf1 deadenylase

FEBS Lett. 2019 Feb;593(3):277-287. doi: 10.1002/1873-3468.13310. Epub 2019 Jan 25.

Abstract

TAR DNA-binding protein 43 (TDP-43) is an RNA-binding protein, whose loss-of-function mutation causes amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Recent studies demonstrated that TDP-43 binds to the 3' untranslated region (UTR) of target mRNAs to promote mRNA instability. Here, we show that TDP-43 recruits Caf1 deadenylase to mRNA targets and accelerates their deadenylation. Tethering TDP-43 to the mRNA 3'UTR recapitulates destabilization of the mRNA, and TDP-43 accelerates their deadenylation. This accelerated deadenylation is inhibited by a dominant negative mutant of Caf1. We find that TDP-43 physically interacts with Caf1. In addition, we provide evidence that TDP-43 regulates poly(A) tail length of endogenous Progranulin (GRN) mRNA. These results may shed light on the link between dysregulation of TDP-43-mediated mRNA deadenylation and pathogenesis of neurodegenerative diseases.

Keywords: ALS; Caf1; TDP-43; deadenylation; mRNA decay; translation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 3' Untranslated Regions*
  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Exoribonucleases / genetics
  • Exoribonucleases / metabolism*
  • HEK293 Cells
  • HeLa Cells
  • Humans
  • Progranulins / biosynthesis*
  • Progranulins / genetics
  • RNA Stability*

Substances

  • 3' Untranslated Regions
  • DNA-Binding Proteins
  • GRN protein, human
  • Progranulins
  • TARDBP protein, human
  • Exoribonucleases
  • poly(A)-specific ribonuclease