TMEM129 is a Derlin-1 associated ERAD E3 ligase essential for virus-induced degradation of MHC-I

Proc Natl Acad Sci U S A. 2014 Aug 5;111(31):11425-30. doi: 10.1073/pnas.1409099111. Epub 2014 Jul 16.

Abstract

The US11 gene product of human cytomegalovirus promotes viral immune evasion by hijacking the endoplasmic reticulum (ER)-associated degradation (ERAD) pathway. US11 initiates dislocation of newly translocated MHC I from the ER to the cytosol for proteasome-mediated degradation. Despite the critical role for ubiquitin in this degradation pathway, the responsible E3 ligase is unknown. In a forward genetic screen for host ERAD components hijacked by US11 in near-haploid KBM7 cells, we identified TMEM129, an uncharacterized polytopic membrane protein. TMEM129 is essential and rate-limiting for US11-mediated MHC-I degradation and acts as a novel ER resident E3 ubiquitin ligase. TMEM129 contains an unusual cysteine-only RING with intrinsic E3 ligase activity and is recruited to US11 via Derlin-1. Together with its E2 conjugase Ube2J2, TMEM129 is responsible for the ubiquitination, dislocation, and subsequent degradation of US11-associated MHC-I. US11 engages two degradation pathways: a Derlin-1/TMEM129-dependent pathway required for MHC-I degradation and a SEL1L/HRD1-dependent pathway required for "free" US11 degradation. Our data show that TMEM129 is a novel ERAD E3 ligase and the central component of a novel mammalian ERAD complex.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Biocatalysis
  • Cytosol / metabolism
  • Down-Regulation
  • Endoplasmic Reticulum / enzymology
  • Endoplasmic Reticulum-Associated Degradation*
  • Genes, Viral
  • Genetic Testing
  • Haploidy
  • Herpesvirus 1, Human / genetics
  • Herpesvirus 1, Human / physiology*
  • Histocompatibility Antigens Class I / metabolism*
  • Humans
  • Membrane Proteins / metabolism*
  • Molecular Sequence Data
  • Protein Binding
  • Protein Stability
  • Proteins / metabolism
  • Proteolysis*
  • RNA-Binding Proteins / genetics
  • RNA-Binding Proteins / metabolism
  • Ubiquitin-Conjugating Enzymes / metabolism
  • Ubiquitin-Protein Ligases / chemistry
  • Ubiquitin-Protein Ligases / metabolism*
  • Ubiquitination
  • Viral Proteins / genetics
  • Viral Proteins / metabolism

Substances

  • DERL1 protein, human
  • Histocompatibility Antigens Class I
  • Membrane Proteins
  • Proteins
  • RNA-Binding Proteins
  • SEL1L protein, human
  • US11 protein, herpesvirus
  • Viral Proteins
  • UBE2J2 protein, human
  • Ubiquitin-Conjugating Enzymes
  • SYVN1 protein, human
  • TMEM129 protein, human
  • Ubiquitin-Protein Ligases