The NSP3 protein of SARS-CoV-2 binds fragile X mental retardation proteins to disrupt UBAP2L interactions

EMBO Rep. 2024 Feb;25(2):902-926. doi: 10.1038/s44319-023-00043-z. Epub 2024 Jan 2.

Abstract

Viruses interact with numerous host factors to facilitate viral replication and to dampen antiviral defense mechanisms. We currently have a limited mechanistic understanding of how SARS-CoV-2 binds host factors and the functional role of these interactions. Here, we uncover a novel interaction between the viral NSP3 protein and the fragile X mental retardation proteins (FMRPs: FMR1, FXR1-2). SARS-CoV-2 NSP3 mutant viruses preventing FMRP binding have attenuated replication in vitro and reduced levels of viral antigen in lungs during the early stages of infection. We show that a unique peptide motif in NSP3 binds directly to the two central KH domains of FMRPs and that this interaction is disrupted by the I304N mutation found in a patient with fragile X syndrome. NSP3 binding to FMRPs disrupts their interaction with the stress granule component UBAP2L through direct competition with a peptide motif in UBAP2L to prevent FMRP incorporation into stress granules. Collectively, our results provide novel insight into how SARS-CoV-2 hijacks host cell proteins and provides molecular insight into the possible underlying molecular defects in fragile X syndrome.

Keywords: Fragile X Syndrome; NSP3; SARS-CoV-2; Stress Granules; UBAP2L.

Publication types

  • Case Reports

MeSH terms

  • COVID-19*
  • Fragile X Mental Retardation Protein / genetics
  • Fragile X Mental Retardation Protein / metabolism
  • Fragile X Syndrome* / genetics
  • Fragile X Syndrome* / metabolism
  • Humans
  • Peptides / metabolism
  • RNA-Binding Proteins / genetics
  • SARS-CoV-2

Substances

  • FMR1 protein, human
  • Fragile X Mental Retardation Protein
  • FXR1 protein, human
  • Peptides
  • RNA-Binding Proteins