Structural and functional analysis of the three MIF4G domains of nonsense-mediated decay factor UPF2

Nucleic Acids Res. 2014 Feb;42(4):2673-86. doi: 10.1093/nar/gkt1197. Epub 2013 Nov 23.

Abstract

Nonsense-mediated decay (NMD) is a eukaryotic quality control pathway, involving conserved proteins UPF1, UPF2 and UPF3b, which detects and degrades mRNAs with premature stop codons. Human UPF2 comprises three tandem MIF4G domains and a C-terminal UPF1 binding region. MIF4G-3 binds UPF3b, but the specific functions of MIF4G-1 and MIF4G-2 are unknown. Crystal structures show that both MIF4G-1 and MIF4G-2 contain N-terminal capping helices essential for stabilization of the 10-helix MIF4G core and that MIF4G-2 interacts with MIF4G-3, forming a rigid assembly. The UPF2/UPF3b/SMG1 complex is thought to activate the kinase SMG1 to phosphorylate UPF1 in vivo. We identify MIF4G-3 as the binding site and in vitro substrate of SMG1 kinase and show that a ternary UPF2 MIF4G-3/UPF3b/SMG1 complex can form in vitro. Whereas in vivo complementation assays show that MIF4G-1 and MIF4G-2 are essential for NMD, tethering assays reveal that UPF2 truncated to only MIF4G-3 and the UPF1-binding region can still partially accomplish NMD. Thus UPF2 MIF4G-1 and MIF4G-2 appear to have a crucial scaffolding role, while MIF4G-3 is the key module required for triggering NMD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • HeLa Cells
  • Humans
  • Models, Molecular
  • Nonsense Mediated mRNA Decay*
  • Phosphatidylinositol 3-Kinases / metabolism
  • Protein Structure, Secondary
  • Protein Structure, Tertiary
  • RNA-Binding Proteins / metabolism
  • Transcription Factors / chemistry*
  • Transcription Factors / metabolism

Substances

  • RNA-Binding Proteins
  • Transcription Factors
  • UPF2 protein, human
  • Phosphatidylinositol 3-Kinases

Associated data

  • PDB/4CEK
  • PDB/4CEM