A conserved isoleucine in the binding pocket of RIG-I controls immune tolerance to mitochondrial RNA

Nucleic Acids Res. 2023 Nov 27;51(21):11893-11910. doi: 10.1093/nar/gkad835.

Abstract

RIG-I is a cytosolic receptor of viral RNA essential for the immune response to numerous RNA viruses. Accordingly, RIG-I must sensitively detect viral RNA yet tolerate abundant self-RNA species. The basic binding cleft and an aromatic amino acid of the RIG-I C-terminal domain(CTD) mediate high-affinity recognition of 5'triphosphorylated and 5'base-paired RNA(dsRNA). Here, we found that, while 5'unmodified hydroxyl(OH)-dsRNA demonstrated residual activation potential, 5'-monophosphate(5'p)-termini, present on most cellular RNAs, prevented RIG-I activation. Determination of CTD/dsRNA co-crystal structures and mutant activation studies revealed that the evolutionarily conserved I875 within the CTD sterically inhibits 5'p-dsRNA binding. RIG-I(I875A) was activated by both synthetic 5'p-dsRNA and endogenous long dsRNA within the polyA-rich fraction of total cellular RNA. RIG-I(I875A) specifically interacted with long, polyA-bearing, mitochondrial(mt) RNA, and depletion of mtRNA from total RNA abolished its activation. Altogether, our study demonstrates that avoidance of 5'p-RNA recognition is crucial to prevent mtRNA-triggered RIG-I-mediated autoinflammation.

MeSH terms

  • DEAD Box Protein 58* / chemistry
  • DEAD Box Protein 58* / genetics
  • DEAD Box Protein 58* / metabolism
  • Humans
  • Immune Tolerance
  • Isoleucine* / genetics
  • RNA, Double-Stranded / genetics
  • RNA, Mitochondrial / genetics
  • RNA, Mitochondrial / metabolism
  • RNA, Viral / genetics
  • RNA, Viral / metabolism
  • Receptors, Immunologic* / chemistry
  • Receptors, Immunologic* / genetics
  • Receptors, Immunologic* / metabolism

Substances

  • DEAD Box Protein 58
  • Isoleucine
  • RNA, Double-Stranded
  • RNA, Mitochondrial
  • RNA, Viral
  • RIGI protein, human
  • Receptors, Immunologic