Aggregatin is a mitochondrial regulator of MAVS activation to drive innate immunity

J Immunol. 2025 Feb 1;214(2):238-252. doi: 10.1093/jimmun/vkae019.

Abstract

Mitochondrial antiviral-signaling protein (MAVS) is a key adapter protein required for inducing type I interferons (IFN-Is) and other antiviral effector molecules. The formation of MAVS aggregates on mitochondria is essential for its activation; however, the regulatory mitochondrial factor that mediates the aggregation process is unknown. Our recent work has identified the protein Aggregatin as a critical seeding factor for β-amyloid peptide aggregation. Here we show that Aggregatin serves as a cross-seed for MAVS aggregates on mitochondria to orchestrate innate immune signaling. Aggregatin is primarily localized to mitochondria in the cytosol and has the ability to induce MAVS aggregation and MAVS-dependent IFN-I responses alone in both HEK293 cells and human leukemia monocytic THP-1 cells. Mitochondrial Aggregatin level increases upon viral infection. Also, Aggregatin knockout suppresses viral infection-induced MAVS aggregation and IFN-I signal cascade activation. Nemo-like kinase is further identified as a kinase phosphorylating Aggregatin at Ser59 to regulate its stability and cross-seeding activity. Collectively, our finding reveals an important physiological function of Aggregatin in innate immunity by cross-seeding MAVS aggregation.

Keywords: Aggregatin; MAVS; innate immunity; interferon; mitochondria; viral infection.

MeSH terms

  • Adaptor Proteins, Signal Transducing* / immunology
  • Adaptor Proteins, Signal Transducing* / metabolism
  • HEK293 Cells
  • Humans
  • Immunity, Innate* / immunology
  • Interferon Type I / immunology
  • Interferon Type I / metabolism
  • Mitochondria* / immunology
  • Mitochondria* / metabolism
  • Signal Transduction / immunology
  • THP-1 Cells

Substances

  • MAVS protein, human
  • Adaptor Proteins, Signal Transducing
  • Interferon Type I