Frederik Staels, Leoni Bücken, Leana De Vuyst, Mathijs Willemsen, Erika Van Nieuwenhove, Margaux Gerbaux, Julika Neumann, Vanshika Malviya, Lize Van Meerbeeck, Jeason Haughton, Laura Seldeslachts, Mieke Gouwy, Kimberly Martinod, Greetje Vande Velde, Paul Proost, Lidia Yshii, Susan Schlenner, Rik Schrijvers, Adrian Liston, Stephanie Humblet-Baron
Recently, OTULIN haploinsufficiency was linked to enhanced susceptibility to Staphylococcus aureus infections accompanied by local necrosis and systemic inflammation. The pathogenesis observed in haploinsufficient patients differs from the hyperinflammation seen in classical OTULIN-related autoinflammatory syndrome (ORAS) patients and is characterized by increased susceptibility of dermal fibroblasts to S. aureus alpha toxin-inflicted cytotoxic damage. Immunological abnormalities were not observed in OTULIN haploinsufficient patients, suggesting a non-hematopoietic basis...
2024: Frontiers in Immunology