FKBP12.6从RyR2解离导致通道构象及功能改变。Ca2+从SR漏出使SR的Ca2+负荷减少,Ca2+瞬变减少,舒缩时RyR2耦联障碍,最终引起心肌功能异常。而且RyR2对CICR敏感性提高导致心肌迟后去极诱发心律失常。通过过表达或增加FKBP12.6对RyR2亲和力可以改善通道缺陷,从而使心脏功能及心律失常得到改善。 Dissociation of FKBP12.6 from RyR2 results in conformational and functional alterations of the channel. Leakage of Ca2 + from the SR reduces the Ca2 + load of SR, decreases the transient of Ca2 +, and couples RyR2 during relaxation and contraction, finally resulting in myocardial dysfunction. And RyR2 increased sensitivity to CICR leading to delayed myocardial depolarization induced arrhythmia. By over-expressing or increasing the affinity of FKBP12.6 for RyR2 can improve channel defects, resulting in improved cardiac function and arrhythmia.