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