Article
Patient iPSC-Derived Macrophages to Study Inborn Errors of the IFN-γ Responsive Pathway.
Cells - 19 Feb 2020
Haake Kathrin, Neehus Anna-Lena, Buchegger Theresa, Kühnel Mark Philipp, Blank Patrick, Philipp Friederike, Oleaga-Quintas Carmen, Schulz Ansgar, Grimley Michael, Goethe Ralph, Jonigk Danny, Kalinke Ulrich, Boisson-Dupuis Stéphanie, Casanova Jean-Laurent, Bustamante Jacinta, Lachmann Nico
Abstract excerpt
Interferon γ (IFN-γ) was shown to be a macrophage activating factor already in 1984. Consistently, inborn errors of IFN-γ immunity underlie Mendelian Susceptibility to Mycobacterial Disease (MSMD). MSMD is characterized by genetic predisposition to disease caused by weakly virulent mycobacterial species. Paradoxically, macrophages from patients with MSMD were little tested. Here, we report a disease modeling...
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