Pluripotent stem cell models of Blau syndrome reveal an IFN-γ-dependent inflammatory response in macrophages


Our data support the significance of ligand-independent autoinflammation in the pathophysiology of Blau syndrome. Our comprehensive isogenic disease-specific iPSC panel provides a useful platform for probing therapeutic and diagnostic clues for the treatment of patients with Blau syndrome. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Authors Takada S, Kambe N, Kawasaki Y, Niwa A, Honda-Ozaki F, Kobayashi K, Osawa M, Nagahashi A, Semi K, Hotta A, Asaka I, Yamada Y, Nishikomori R, Heike T, Matsue H, Nakahata T, Saito MK
Journal The Journal of allergy and clinical immunology
Publication Date 2018 Jan;141(1):339-349.e11
PubMed 28587749
DOI 10.1016/j.jaci.2017.04.013

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