The potential of obscurin as a therapeutic target in muscle disorders
The potential of obscurin as a therapeutic target in muscle disorders
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Obscurin 作为肌肉疾病治疗靶点的潜力
DOI：
10.1080/14728222.2017.1361931
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发表时间：
2017
影响因子：
5.8
通讯作者：
V. Sorrentino
中科院分区：
文献类型：
--
作者：
D. Randazzo;E. Pierantozzi;D. Rossi;V. Sorrentino
摘要引言：暗蛋白是横纹肌中的一种巨大蛋白质，在肌原纤维的组装和维持、肌肉蛋白降解和细胞内信号传导等过程中发挥着重要作用。因此，obscurin参与肌肉适应生理要求或与心脏和骨骼肌疾病相关的病理线索的机制。涵盖的领域：不同的obscurin异构体的结构确定到目前为止，它们的组织分布和最近的研究结果obscurin在无脊椎动物和哺乳动物将进行审查。我们将提供一个概要的obscurin和其他蛋白质之间的分子相互作用和生物学相关性，这些相互作用的横纹肌功能。蛋白质降解机制和细胞内信号的参与，也将讨论沿着的作用，obscurin在人类疾病的病理生理学的初步证据。专家意见：尽管仍有许多关于obscurin作为肌节的结构组分或作为肌细胞内信号传导途径的介体的作用有待发现，但可以设想，这种蛋白质代表了用于预防和治疗心脏和骨骼肌疾病的有趣的新型药理学靶点。
ABSTRACT Introduction: Obscurin, a giant protein of striated muscles, is emerging as an important player in a wide range of processes including myofibril assembly and maintenance, muscle protein degradation and intracellular signaling. Accordingly, obscurin participates to the mechanisms by which muscles adapt to physiological requirements or to pathological cues associated with cardiac and skeletal muscle diseases. Areas covered: The structure of the different obscurin isoforms identified so far, their tissue distribution and the most recent findings on obscurin in invertebrates and mammals will be reviewed. We will provide a synopsis of known molecular interactions between obscurin and other proteins and the biological relevance of these interactions for striated muscle function. The involvement of obscurin in protein degradation mechanisms and intracellular signaling will be also discussed along with initial evidence of a role of obscurin in the pathophysiology of human diseases. Expert opinion: Although still much remains to be discovered about the role of obscurin either as a structural component of the sarcomere or as a mediator of signaling pathways within muscle cells, it can be envisioned that this protein represents an interesting novel pharmacological target for the prevention and treatment of cardiac and skeletal muscle diseases.
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影响因子：
3.3
作者：
Ford-Speelman，Diana L.;Roche，Joseph A.;Bloch，Robert J.
通讯作者：
Bloch，Robert J.
斑马鱼 obscurin A RhoGEF 结构域的靶向删除会影响心脏、骨骼肌和大脑发育。
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：
Kontrogianni-Konstantopoulos, Aikaterini
影响因子：
20.1
作者：
M. Bang;T. Centner;F. Fornoff;A. J. Geach;M. Gotthardt;M. Mcnabb;C. Witt;D. Labeit;C. Gregorio;H. Granzier;S. Labeit
通讯作者：
M. Bang;T. Centner;F. Fornoff;A. J. Geach;M. Gotthardt;M. Mcnabb;C. Witt;D. Labeit;C. Gregorio;H. Granzier;S. Labeit
DOI：
--
发表时间：
2000
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
C. Hsieh;S. Fukumoto;M. Layne;K. Maemura;H. Charles;A. Patel;M. Perrella;M. -. Lee
通讯作者：
C. Hsieh;S. Fukumoto;M. Layne;K. Maemura;H. Charles;A. Patel;M. Perrella;M. -. Lee