Loss of USP18 in microglia induces white matter pathology
Schwabenland M, Mossad O, Peres AG, Kessler F, Maron FJM, Harsan LA, Bienert T, von Elverfeldt D, Knobeloch KP, Staszewski O, Heppner FL, Meuwissen MEC, Mancini GMS, Prinz M, Blank T.
Acta Neuropathol Commun. online article
The Ubiquitin specific protease 18 (USP18) is a major negative regulator of the type 1 interferon (IFN) pathway. In a recent publication we showed that USP18 is a key molecule imposing microglial quiescence specifically in the white matter. USP18 is a negative regulator of the type 1 interferon (IFN) pathway. Microglia lacking Usp18 exhibited constitutive activation of type I IFN signaling pathways resulting in markedly elevated expression of multiple interferon-stimulated genes (ISGs). That is why we now characterized conditional myeloid specific Usp18 deficient mice in more detail.