Potential treatments for autoimmune disorders in a RIG-I mutant mouse model (B22)

• Subject Area: Immunology
• Term: since 2018
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 369799452

Gain-of-function mutations in the viral sensor RIG-I are known to cause the autoimmune disease Singleton Merten syndrome (SMS), an autosomal-dominant multi-system disorder characterized by dental dysplasia, aortic calcification, skeletal abnormalities, glaucoma and psoriasis. However, the mechanisms by which constitutively active RIG-I causes the pathogenesis remain unclear and effective treatments for these autoimmune disorders are missing. We have established a mouse model expressing RIG-I SMS mutant with spontaneous SMS-like pathologies. We aim to reveal the pathophysiology of each of these clinical manifestations and develop treatments for patients by analysing this mouse model.

• DFG Programme: CRC/Transregios
• Subproject of: TRR 237: Nucleic Acid Immunity
• Applicant Institution: Ludwig-Maximilians-Universität München
• Project Head: Professor Dr. Hiroki Kato