Increased number of caveolae and caveolin-3 overexpression in Duchenne muscular dystrophy.
Increased number of caveolae and caveolin-3 overexpression in Duchenne muscular dystrophy.
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DOI：
10.1006/bbrc.1999.1055
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发表时间：
1999-08
影响因子：
3.1
通讯作者：
Silvia Repetto;M. Bado;P. Broda;G. Lucania;Emiliana Masetti;F. Sotgia;Ilaria Carbone;Antonio Pavan;Eduardo Bonilla;Giuseppe Cordone;M. Lisanti;Carlo Minetti
中科院分区：
文献类型：
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作者：
Silvia Repetto;M. Bado;P. Broda;G. Lucania;Emiliana Masetti;F. Sotgia;Ilaria Carbone;Antonio Pavan;Eduardo Bonilla;Giuseppe Cordone;M. Lisanti;Carlo Minetti
小泡是位于质膜上的小口袋或内陷。小泡蛋白是小泡的主要蛋白质成分，在小泡膜的形成中起着重要的结构作用。本研究采用冷冻断裂和免疫技术研究了杜氏肌营养不良（DMD）肌纤维中肌细胞质膜小窝的空间组织和小窝蛋白-3的表达。在DMD肌肉中，通过免疫组织化学和Western blot分析，我们发现肌膜上的小窝数量增加，这与小窝蛋白-3的过度表达相对应。这些发现提示小泡和小泡蛋白-3可能在DMD的发病机制中起作用。
Caveolae are small pockets or invaginations localized at the plasma membrane. Caveolins are the principal protein components of caveolae and play an important structural role in the formation of caveolae membranes. Here, we studied by freeze fracture and immunological techniques the spatial organization of caveolae at the muscle cell plasma membrane and the expression of caveolin-3 in Duchenne muscular dystrophy (DMD) muscle fibers. In DMD muscle, we found an increased number of caveolae at the sarcolemma that corresponds to an overexpression of caveolin-3 by immunohistochemistry and by Western blot analysis. These findings suggest a possible role for caveolae and caveolin-3 in the pathogenesis of DMD.