J Clin Invest. 2024 May 15;134(10):e180482. doi: 10.1172/JCI180482.
Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiac condition characterized by cardiac remodeling and life-threatening ventricular arrhythmias. In this issue of the JCI, Chelko, Penna, and colleagues mechanistically addressed the intricate contribution of immune-mediated injury in ACM pathogenesis. Inhibition of nuclear factor κ-B (NF-κB) and infiltration of monocyte-derived macrophages expressing C-C motif chemokine receptor-2 (CCR2) alleviated the phenotypic ACM features (i.e., fibrofatty replacement, contractile dysfunction, and ventricular arrhythmias) in desmoglein 2-mutant (Dsg2mut/mut) mice. These findings pave the way for efficacious and targetable immune therapy for patients with ACM.
致心律失常性心肌病（ACM）是一种遗传性心脏病，其特征为心脏重构和危及生命的室性心律失常。在本期 JCI 中，Chelko、Penna 和同事从机制上探讨了免疫介导损伤在 ACM 发病机制中的复杂作用。核因子 κ-B（NF-κB）抑制和表达 C-C 基序趋化因子受体-2（CCR2）的单核细胞来源的巨噬细胞浸润减轻了桥粒蛋白 2 突变（Dsg2mut/mut）小鼠的 ACM 表型特征（即纤维脂肪替代、收缩功能障碍和室性心律失常）。这些发现为 ACM 患者的有效和靶向免疫治疗铺平了道路。
