Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
J Clin Invest. 2010 Jul;120(7):2280-91. doi: 10.1172/JCI41407.
The glycogen synthase kinase-3 (GSK-3) family of serine/threonine kinases consists of 2 highly related isoforms, alpha and beta. Although GSK-3beta has an important role in cardiac development, much remains unknown about the function of either GSK-3 isoform in the postnatal heart. Herein, we present what we believe to be the first studies defining the role of GSK-3alpha in the mouse heart using gene targeting. Gsk3a(-/-) mice over 2 months of age developed progressive cardiomyocyte and cardiac hypertrophy and contractile dysfunction. Following thoracic aortic constriction in young mice, we observed enhanced hypertrophy that rapidly transitioned to ventricular dilatation and contractile dysfunction. Surprisingly, markedly impaired beta-adrenergic responsiveness was found at both the organ and cellular level. This phenotype was reproduced by acute treatment of WT cardiomyocytes with a small molecule GSK-3 inhibitor, confirming that the response was not due to a chronic adaptation to LV dysfunction. Thus, GSK-3alpha appears to be the central regulator of a striking range of essential processes, including acute and direct positive regulation of beta-adrenergic responsiveness. In the absence of GSK-3alpha, the heart cannot respond effectively to hemodynamic stress and rapidly fails. Our findings identify what we believe to be a new paradigm of regulation of beta-adrenergic signaling and raise concerns given the rapid expansion of drug development targeting GSK-3.
糖原合酶激酶-3(GSK-3)家族是丝氨酸/苏氨酸激酶的两个高度相关的同工酶,分别为α和β。虽然 GSK-3β在心的发育中具有重要作用,但对于其在出生后的心脏中的任一同工酶的功能仍知之甚少。在此,我们首次使用基因靶向技术定义 GSK-3α在小鼠心脏中的作用。超过 2 个月大的 Gsk3a(-/-)小鼠逐渐出现心肌细胞和心脏肥大以及收缩功能障碍。在年轻小鼠的胸主动脉缩窄后,我们观察到心肌肥大迅速发展为心室扩张和收缩功能障碍。令人惊讶的是,在器官和细胞水平上均发现β-肾上腺素能反应显著受损。WT 心肌细胞的急性 GSK-3 抑制剂处理重现了该表型,证实该反应不是由于 LV 功能障碍的慢性适应所致。因此,GSK-3α似乎是包括β-肾上腺素能反应的急性和直接正调节在内的一系列基本过程的中央调节剂。在缺乏 GSK-3α的情况下,心脏不能有效地应对血流动力学应激,从而迅速衰竭。我们的发现确定了我们认为是β-肾上腺素能信号转导的新调节范式,并引起了人们的关注,因为靶向 GSK-3 的药物开发正在迅速扩展。