News Releases & Research Results Elucidation of the molecular onset mechanism of incurable COPA syndrome - Development of new therapeutic agents for autoimmune/autoinflammatory diseases -

The results of research conducted by Assistant Professor Mukai and Professor Taguchi of the Graduate School of Life Sciences, Tohoku University.

• Using a mouse model of COPA syndrome*that causes arthritis and interstitial pneumonia, the inflammatory conditions of COPA syndrome were demonstrated to be caused by abnormal activation of innate immune signals in the STING (stimulators of interferon genes) pathway.
  *Autosomal dominantly inherited autoimmune/autoinflammatory disease caused by mutations in the COPA (coatomer protein alpha) gene, an intractable disease characterized by arthritis and interstitial pneumonia.
• Treatment with STING inhibitors improved the constitutive inflammatory response in cells collected from patients with COPA syndrome.
• The STING inhibitors used in the study should serve as drug discovery seeds for COPA syndrome.

This program was conducted with the support of the Advanced Research & Development Programs for Medical Innovation (PRIME) by AMED.

The results of this research project were published online in the Journal of Experimental Medicine on July 28.

Zimu Deng., et al. A defect in COPI-mediated transport of STING causes immune dysregulation in COPA syndrome Journal of Experimental Medicine
DOI：10.1084/jem.20201045