Developmental Expression of Calcium Activated Chloride Ion Channels Anoctamin 5 in Mouse Skeletal Muscle

Hai-yan Song, Yi-Min Zhang, Hui Lian, Li Zhou, Yue-Min Tian, Jin-Xia Zhu
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引用次数: 2

Abstract

Anoctamin 5 (Ano5), also named TMEM16E, belongs to the Anoctamin gene family. The mutations in the Ano5 gene cause Limb-girdle muscular dystrophy (LGMD) 2L type and Miyoshi muscular dystrophy (MMD3). Both patients showed sarcolemmal lesions. The studies showed that TMEM16E mRNA expressed in the somites during embryogenesis, particularly in the myotomal cells, and also in the muscle myotome-derived progenitor cells. However, no report has been done to examine Ano5 expression during mouse skeletal muscle development. In the present study, we investigated the distribution and quantification of Ano5 in the skeletal muscles of mice during their development, with the methods of immunofluorescence, Reverse transcription-polymerase chain reaction (RT-PCR) and Western blot analyses. The results indicated that Ano5 mRNA and protein are expressed in skeletal muscle of the mouse from 1 day to 6 months, but with development and aging, the expression of Ano5 reduced gradually. Taken together, our results demonstrate that Ano5 expression level decreased throughout development and aging, and this may explain why the musculardystrophy syndrome of Ano5 mutant patients only starts during the later stage of their lives.
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钙激活氯离子通道-氨基辛胺5在小鼠骨骼肌中的发育表达
Anoctamin 5 (Ano5)也被命名为TMEM16E,属于Anoctamin基因家族。Ano5基因的突变导致肢体带状肌营养不良(LGMD) 2L型和三良肌营养不良(MMD3)。两例患者均有肌上皮病变。研究表明,TMEM16E mRNA在胚胎发生过程中在体体中表达,特别是在肌瘤细胞中,也在肌瘤源性祖细胞中表达。然而,没有关于Ano5在小鼠骨骼肌发育过程中的表达的报道。本研究采用免疫荧光、逆转录聚合酶链反应(RT-PCR)和Western blot等方法研究了Ano5在小鼠发育过程中骨骼肌中的分布和定量。结果表明,Ano5 mRNA和蛋白在小鼠1 ~ 6月龄骨骼肌中有表达,但随着发育和衰老,Ano5的表达逐渐降低。综上所述,我们的研究结果表明,Ano5的表达水平在发育和衰老过程中下降,这可能解释了为什么Ano5突变患者的肌肉营养不良综合征只在他们生命的后期才开始。
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