肌营养不良缺失的肌管显示出不同膜区室的连接，其特征为 TMEM16E 和 DHPRα。
Dysferlin-deficient myotubes show tethering of different membrane compartments characterized by TMEM16E and DHPRα.
机构信息
Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, 734-8553, Japan; Department of Oral Surgery, National Hospital Organization Kure Medical Center, Kure, 737-0023, Japan.
Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, 734-8553, Japan.
出版信息
Biochem Biophys Res Commun. 2020 Aug 27;529(3):720-725. doi: 10.1016/j.bbrc.2020.06.079. Epub 2020 Jul 18.
TMEM16E deficiency has been shown to be responsible for human limb-girdle muscular dystrophy LGMD2L. We found that endogenous TMEM16E co-localized with caveolin-3 at cytoplasmic vesicular compartments in a myotube from C2C12 cells (C2C12 myotube) without forming a molecular complex. In contrast, a myotube from murine myoblastic dysferlin-deficient GREG cells (GREG myotube) showed not only co-localization but also constitutive association of caveolin-3 and TMEM16E. GREG myotubes also displayed constitutive association of TMEM16E with DHPRα, which reside in different membrane compartments, indicating increased contact of the different vesicular membrane compartments. Τhese results suggest that a dynamic tethering of different membrane compartments might represent a distorted membrane damage repairing process in the absence of dysferlin.
TMEM16E 缺乏被证明是导致人类肢带型肌肉营养不良症 LGMD2L 的原因。我们发现，内源性 TMEM16E 在 C2C12 细胞的肌管（C2C12 肌管）中与 caveolin-3 共同定位于细胞质囊泡区室中，而不形成分子复合物。相比之下，来自鼠源成肌细胞营养不良症缺失 dysferlin 的 GREG 细胞的肌管（GREG 肌管）不仅显示出 caveolin-3 和 TMEM16E 的共定位，还显示出它们的组成性关联。GREG 肌管还显示出 TMEM16E 与 DHPRα 的组成性关联，DHPRα 位于不同的膜区室中，表明不同的囊泡膜区室之间的接触增加。这些结果表明，在没有 dysferlin 的情况下，不同膜区室的动态连接可能代表了一种扭曲的膜损伤修复过程。
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