Enhanced interleukin-1β and interleukin-18 release in a patient with chronic infantile neurologic, cutaneous, articular syndrome
Enhanced interleukin-1β and interleukin-18 release in a patient with chronic infantile neurologic, cutaneous, articular syndrome
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DOI：
10.1002/art.20494
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发表时间：
2004-10-01
影响因子：
--
通讯作者：
van Dissel, JT
中科院分区：
文献类型：
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作者：
Janssen, R;Verhard, E;van Dissel, JT
慢性婴儿神经、皮肤、关节（CINCA）综合征是一种严重的炎症性疾病，最近与CIAS1基因突变有关。据推测，这些突变可能导致炎症反应增强。在此，我们提供证据证明CINCA综合征炎症的特征是白细胞介素-1 β (il -1 β)和IL-18的释放在刺激血细胞时增加，并表明这种释放依赖于caspase I。
Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome is a severe inflammatory disease that recently was associated with mutations in CIAS1. It was hypothesized that these mutations may lead to enhanced inflammatory responses. Herein, we provide evidence that inflammation in the CINCA syndrome is characterized by enhanced interleukin-1beta (IL-1beta) and IL-18 release upon stimulation of blood cells and show that this release is caspase I dependent.