Immunobiological analysis of cardiac muscle type ryanodine receptor genetic mutation mice

在这项研究中，我们研究了RyR 2的R420 W突变是否与胸腺淋巴状态的表型特征相关，通过产生携带突变的敲入小鼠模型。与野生型小鼠相比，纯合子（RyR 2 R420 W/R420 W）小鼠的胸腺和脾脏重量显著增加。此外，在RyR 2 R420 W/R420 W小鼠中诱导了各种类型的心律失常，以响应咖啡因和肾上腺素的给药;在野生型小鼠中未观察到相同的结果。这些发现表明RyR 2 R420 W/R420 W小鼠表现出胸腺淋巴状态样表型。这种小鼠模型可能有助于研究这种疾病的病理生理学。RyR 2 R420 W/R420 W小鼠胸腺和脾脏中mmu-miR-291 b-5 p的量减少。这些结果提示mmu-miR-291 b-5 p可能参与了导致淋巴器官肥大的病理生理过程。

In this study, we investigated whether the R420W mutation of RyR2 is associated with the phenotypic characteristics of status thymicolymphaticus by generating a knock-in mouse model carrying the mutation. Homozygous (RyR2R420W/R420W) mice showed significant increases in thymus and spleen weights, when compared with wild-type mice. In addition, various types of arrhythmias were induced in RyR2R420W/R420W mice in response to the administration of caffeine and adrenaline; the same results were not observed in wild-type mice. These findings suggest that RyR2R420W/R420W mice exhibit status thymicolymphaticus-like phenotypes. This mouse model may be useful for studying the pathophysiology of this condition. The amount of mmu-miR-291b-5p from the thymus and spleen decreased in the RyR2R420W/R420W mice. These results suggested that mmu-miR-291b-5p possible may be associated with the pathophysiological process leading to the hypertrophy of lymphoid organs.