Obscure functions: the location–function relationship of obscurins
Obscure functions: the location–function relationship of obscurins
复制标题
隐匿功能：隐匿蛋白的位置-功能关系
作者：
H. Manring;Olivia A. Carter;Maegen A. Ackermann
多肽的obscurin家族对于正常横纹肌功能是必需的，并且有助于致命疾病（包括心肌病和癌症）的发病机制。单个哺乳动物obscurin基因OBSCN产生由串联粘附和信号基序组成的巨大（约800 kDa）和较小（约40-500 kDa）蛋白质。哺乳动物obscurin蛋白在多种细胞类型中表达，包括横纹肌，并定位于不同的亚细胞区室，在那里它们有助于不同的细胞过程。在秀丽隐杆线虫和果蝇中的Obscurin同系物具有相似的结构域结构，并且也在横纹肌中表达。长期以来一直在寻找的问题，“蒙昧是做什么的？”是复杂的，并且如果不考虑这些蛋白质的亚细胞分布和局部同种型浓度就不能解决。在此，我们提出了一个概述的功能obscurin和开始，以确定其在横纹肌的亚细胞分布和功能之间的复杂关系。
The obscurin family of polypeptides is essential for normal striated muscle function and contributes to the pathogenesis of fatal diseases, including cardiomyopathies and cancers. The single mammalian obscurin gene, OBSCN, gives rise to giant (∼800 kDa) and smaller (∼40–500 kDa) proteins that are composed of tandem adhesion and signaling motifs. Mammalian obscurin proteins are expressed in a variety of cell types, including striated muscles, and localize to distinct subcellular compartments where they contribute to diverse cellular processes. Obscurin homologs in Caenorhabditis elegans and Drosophila possess a similar domain architecture and are also expressed in striated muscles. The long sought after question, “what does obscurin do?” is complex and cannot be addressed without taking into consideration the subcellular distribution of these proteins and local isoform concentration. Herein, we present an overview of the functions of obscurins and begin to define the intricate relationship between their subcellular distributions and functions in striated muscles.
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影响因子：
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作者：
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通讯作者：
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Kontrogianni-Konstantopoulos A;Ackermann MA;Bowman AL;Yap SV;Bloch RJ
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3.3
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影响因子：
2.7
作者：
Raeker,MaideO;Bieniek,AshleyN;Ryan,AlisonS;Tsai,Huai-Jen;Zahn,KatelinM;Russell,MarkW
通讯作者：
Russell,MarkW
影响因子：
3.3
作者：
Ackermann, Maegen A.;Hu, Li-Yen R.;Kontrogianni-Konstantopoulos, Aikaterini
通讯作者：
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