Toll-like receptor 4 antagonist TAK-242 inhibits autoinflammatory symptoms in DITRA

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作   者：

Shibata, Akitaka;  Sugiura, Kazumitsu;  Furuta, Yasuhide;  Mukumoto, Yoshiko;  Kaminuma, Osamu;  Akiyama, Masashi; 

作者机构：

Kobe;  Allergy & Immunol Project;  Tokyo;  Nagoya;  Tokyo Metropolitan Inst Med Sci;  Anim Resource Dev Unit;  Nagoya Univ;  Aichi;  Japan;  Dept Genome Med;  Grad Sch Med;  Hyogo;  Ctr Life Sci Technol;  RIKEN;  Dept Dermatol; 

关键词：

Autoinflammation;  Toll-like receptor 4;  Deficiency of IL-36 receptor antagonist;  Generalized pustular psoriasis;  TAK-242;  LPS; 

期刊名称：

Journal of Autoimmunity

i s s n：

0896-8411

年卷期：

2017 年 80 卷

页   码：

28-38

页   码：

摘   要：

Background: IL36RN encodes the IL-36 receptor antagonist (IL-36Ra), and loss-of-function mutations in IL36RN define a recessively inherited autoinflammatory disease named "deficiency of IL-36Ra" (DITRA). DITRA causes systemic autoinflammatory diseases, including generalized pustular psoriasis (GPP), an occasionally life-threatening disease that is characterized by widespread sterile pustules on the skin, fever and other systemic symptoms. GPP can present at any age, and provocative factors include various infections, medicines and pregnancy.