KID interferon project
We are pleased to share information about the "KID_interferon" project, provided by Lorenzo Lodi.
Brief rationale of the project:
Monogenic type I interferonopathies (T1I) (like COPA syndrome (COPA), STING-associated vasculopathy with onset in infancy (SAVI) or some monogenic lupus) may display renal involvement. These entities are characterized by a systemic upregulation of type I interferons (IFN-I) which is central to the disease pathogenesis. However, a clear documentation of IFN-I upregulation at tissue level is still lacking. Indeed, this would be crucial to understand the selective organ involvement encountered in these diseases. The demonstration of tissue IFN-I upregulation can be performed applying human myxovirus resistance protein 1 (MxA) immunostaining to renal biopsies. While this characterization has been assessed in a few patients with T1I-related kidney diseases, it has never been performed in SAVI, COPA and several others T1I.
Here you can find more details about the project.