Department of Immunology, UConn Health, 263 Farmington Ave, Farmington, CT, 06030, USA.
Department of Neuroscience, UConn Health, 263 Farmington Ave, Farmington, CT, 06030, USA.
Sci Rep. 2024 Jul 16;14(1):16393. doi: 10.1038/s41598-024-63567-x.
The search for dementia treatments, including treatments for neuropsychiatric lupus (NPSLE), has not yet uncovered useful therapeutic targets that mitigate underlying inflammation. Currently, NPSLE's limited treatment options are often accompanied by severe toxicity. Blocking toll-like receptor (TLR) and IL-1 receptor signal transduction by inhibiting interleukin-1 receptor-associated kinase 4 (IRAK4) offers a new pathway for intervention. Using a pre-clinical NPSLE model, we compare lupus-like B6.MRL-Faslpr (MRL) mice with B6.MRL-Faslpr-IRAK4 kinase-dead (MRL-IRAK4-KD) mice, which are were less prone to 'general' lupus-like symptoms. We demonstrate that lupus-prone mice with a mutation in the kinase domain of IRAK4 no longer display typical lupus hallmarks such as splenomegaly, inflammation, production of hormones, and anti-double-stranded (ds)DNA antibody. water maze behavioral testing, which measures contextual associative learning, revealed that mice without functional IRAK4 displayed a recovery in memory acquisition deficits. RNA-seq approach revealed that cytokine and hormone signaling converge on the JAK/STAT pathways in the mouse hippocampus. Ultimately, the targets identified in this work may result in broad clinical value that can fill the significant scientific and therapeutic gaps precluding development of cures for dementia.
目前,针对神经精神性狼疮(NPSLE)的治疗方法有限,且往往伴随着严重的毒性。通过抑制白细胞介素-1 受体相关激酶 4(IRAK4)来阻断 toll 样受体(TLR)和白细胞介素-1 受体信号转导,为干预提供了新途径。我们使用临床前 NPSLE 模型,比较了 B6.MRL-Faslpr(MRL)小鼠和 B6.MRL-Faslpr-IRAK4 激酶失活(MRL-IRAK4-KD)小鼠,后者更不容易出现“全身性”狼疮样症状。我们发现,IRAK4 激酶结构域发生突变的狼疮易感小鼠不再表现出典型的狼疮特征,如脾肿大、炎症、激素产生和抗双链(ds)DNA 抗体。水迷宫行为测试,用于测量情景联想学习,表明没有功能性 IRAK4 的小鼠在记忆获取缺陷方面有恢复。RNA-seq 方法揭示了细胞因子和激素信号在小鼠海马体中汇聚到 JAK/STAT 通路。最终,这项工作中确定的靶点可能具有广泛的临床价值,可以填补阻止痴呆症治疗方法发展的重大科学和治疗空白。