New Paper - B20 Günther
20.02.2024
New publication from Claudia Günther's and Eva Bartok's group
Myotonic dystrophy is a repeat expansion disease that is associated with an increased prevalence of autoimmune disorders. The group was able to demonstrate that the accumulation of RNA repeats in the cell induces a chronic ER stress response, which is characterized by the activation of the ATF6 pathway. This response ensures survival at the expense of a tightly regulated mitochondrial stress response that leads to the release of mtDNA into the cytoplasm. mtDNA is a danger signal that, after stimulation of the cGAS-STING pathway, induces chronic activation of the type I interferon response. This predisposes affected patients to the development of autoimmunity. The exploration of this new pathogenic pathway opens new possibilities for targeted intervention strategies.
published in Nature Communications: Chronic endoplasmic reticulum stress in myotonic dystrophy type 2 promotes autoimmunity via mitochondrial DNA release.
Please check out the press release from the TU Dresden (German): https://tu-dresden.de/tu-dresden/newsportal/news/falscher-alarm-des-immunsystems-bei-muskelkrankheit