Journal Articles - Health - 2020

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Dysferlin-deficient myotubes show tethering of different membrane compartments characterized by TMEM16E and DHPRα

( 2020)
Kazumi Kubozono
;
Kuniko Mizuta
;
Shinichi Fujimoto
;
Ta To Tran
;
Nobuyuki Kamata
;
Kei Tobiume

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.