Article
Pluripotent stem cell models of Blau syndrome reveal an IFN-γ-dependent inflammatory response in macrophages.
The Journal of allergy and clinical immunology - 1 Jan 2018
Takada Sanami, Kambe Naotomo, Kawasaki Yuri, Niwa Akira, Honda-Ozaki Fumiko, Kobayashi Kazuki, Osawa Mitsujiro, Nagahashi Ayako, Semi Katsunori, Hotta Akitsu, Asaka Isao, Yamada Yasuhiro, Nishikomori Ryuta, Heike Toshio, Matsue Hiroyuki, Nakahata Tatsutoshi, Saito Megumu K
Abstract excerpt
BACKGROUND: Blau syndrome, or early-onset sarcoidosis, is a juvenile-onset systemic granulomatosis associated with a mutation in nucleotide-binding oligomerization domain 2 (NOD2). The underlying mechanisms of Blau syndrome leading to autoinflammation are still unclear, and there is currently no effective specific treatment for Blau syndrome. OBJECTIVES: To elucidate the mechanisms of autoinflammation in patients...
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