An NLRP3 Mutation Causes Arthropathy and Osteoporosis in Humanized Mice

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• 作者：John N. Snouwaert¹ ; ⁵ ; MyTrang Nguyen¹ ; ⁵ ; Peter W. Repenning¹ ; Rebecca Dye¹ ; Eric W. Livingston⁴ ; Martina Kovarova¹ ; Sheryl S. Moy² ; Brian E. Brigman³ ; Ted A. Bateman⁴ ; Jenny P.-Y. Ting¹ ; Beverly H. Koller¹ ; ⁶ ; ^{bkoller@correspondence.unc.edu}
• 关键词：syntenic replacement ; cryopyrinopathies ; inflammasome ; meningitis ; endotoxic shock ; autoinflammatory ; IL-18 ; arthritis ; familial cold autoinflammatory syndrome ; Muckle-Wells syndrome ; NOMID
• 刊名：Cell Reports
• 出版年：2016
• 出版时间：13 December 2016
• 年：2016
• 卷：17
• 期：11
• 页码：3077-3088
• 全文大小：3716 K
• 卷排序：17

文摘

Syntenic replacement humanizes the NLRP3 inflammasome in mice The common NLRP3 gene replaces the function of the excised mouse gene An NLRP3 disease allele causes arthritis, osteoporosis, and endotoxin hypersensitivity NLRP3-humanized mice provide a platform for evaluating anti-NLRP3 therapies