Department of Cardiovascular and Metabolic Sciences, Lerner Research Institute, and.
Department of Cardiology, National Defense Medical College, Tokorozawa, Japan, 359-8513.
Mol Biol Cell. 2021 Apr 1;32(7):622-633. doi: 10.1091/mbc.E20-06-0394. Epub 2021 Feb 3.
Dysregulation of immune responses has been linked to the generation of immunoglobulin G (IgG) autoantibodies that target human β1ARs and contribute to deleterious cardiac outcomes. Given the benefits of β-blockers observed in patients harboring the IgG3 subclass of autoantibodies, we investigated the role of these autoantibodies in human β1AR function. Serum and purified IgG3(+) autoantibodies from patients with onset of cardiomyopathy were tested using human embryonic kidney (HEK) 293 cells expressing human β1ARs. Unexpectedly, pretreatment of cells with IgG3(+) serum or purified IgG3(+) autoantibodies impaired dobutamine-mediated adenylate cyclase (AC) activity and cyclic adenosine monophosphate (cAMP) generation while enhancing biased β-arrestin recruitment and Extracellular Regulated Kinase (ERK) activation. In contrast, the β-blocker metoprolol increased AC activity and cAMP in the presence of IgG3(+) serum or IgG3(+) autoantibodies. Because IgG3(+) autoantibodies are specific to human β1ARs, non-failing human hearts were used as an endogenous system to determine their ability to bias β1AR signaling. Consistently, metoprolol increased AC activity, reflecting the ability of the IgG3(+) autoantibodies to bias β-blocker toward G-protein coupling. Importantly, IgG3(+) autoantibodies are specific toward β1AR as they did not alter β2AR signaling. Thus, IgG3(+) autoantibody biases β-blocker toward G-protein coupling while impairing agonist-mediated G-protein activation but promoting G-protein-independent ERK activation. This phenomenon may underlie the beneficial outcomes observed in patients harboring IgG3(+) β1AR autoantibodies.
免疫反应失调与产生针对人β1AR 的免疫球蛋白 G (IgG) 自身抗体有关,这些自身抗体导致心脏不良后果。鉴于β受体阻滞剂在携带 IgG3 自身抗体亚类的患者中观察到的益处,我们研究了这些自身抗体在人β1AR 功能中的作用。使用表达人β1AR 的人胚肾 (HEK) 293 细胞测试了来自心肌病发病患者的血清和纯化的 IgG3(+)自身抗体。出乎意料的是,用 IgG3(+)血清或纯化的 IgG3(+)自身抗体预处理细胞会损害多巴酚丁胺介导的腺苷酸环化酶 (AC) 活性和环腺苷酸 (cAMP) 的产生,同时增强偏向β-抑制蛋白募集和细胞外调节激酶 (ERK) 激活。相比之下,β受体阻滞剂美托洛尔在存在 IgG3(+)血清或 IgG3(+)自身抗体的情况下增加 AC 活性和 cAMP。由于 IgG3(+)自身抗体特异性针对人β1AR,因此使用非衰竭人心作为内源性系统来确定它们偏向β1AR 信号的能力。一致地,美托洛尔增加了 AC 活性,反映了 IgG3(+)自身抗体将β受体阻滞剂偏向 G 蛋白偶联的能力。重要的是,IgG3(+)自身抗体特异性针对β1AR,因为它们不会改变β2AR 信号。因此,IgG3(+)自身抗体使β受体阻滞剂偏向 G 蛋白偶联,同时损害激动剂介导的 G 蛋白激活,但促进 G 蛋白非依赖性 ERK 激活。这种现象可能是携带 IgG3(+)β1AR 自身抗体的患者观察到的有益结果的基础。