MCC950 blocks enhanced interleukin-1β production in patients with NLRP3 low penetrance variants.

E Schuh,C J Groß, D Wagner, M Schlüter,O Groß,T Kümpfel

Clinical Immunology（2019）

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摘要

PBMC of several patients with NLRP3 low penetrance variants and CNS manifestation showed increased NLRP3-specific IL-1β release upon stimulation and elevated NLRP3-independent IL-6 and TNF-α levels as those were not suppressed by MCC950. Our data suggest that beside the possible causal involvement of the NLRP3 inflammasome additional, yet unidentified genetic or environmental factors may contribute to the multi-organ inflammation in our patients and explain the partial response to IL-1 targeting therapies.

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关键词

NLRP3 low penetrance mutations,IL-1β,MCC950,Autoinflammation,Autoimmunity

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