Membrane-myofibril cross-talk in myofibrillogenesis and in muscular dystrophy pathogenesis: lessons from the zebrafish.
Membrane-myofibril cross-talk in myofibrillogenesis and in muscular dystrophy pathogenesis: lessons from the zebrafish.
复制标题
DOI：
10.3389/fphys.2014.00014
复制
发表时间：
2014
影响因子：
4
通讯作者：
Russell MW
中科院分区：
文献类型：
--
作者：
Raeker MÖ;Shavit JA;Dowling JJ;Michele DE;Russell MW
横纹肌具有高度有序的结构，其中参与力传递（costameres）和兴奋-收缩耦合（T小管）的细胞膜的专门区域以及肌浆网的内部膜组织在肌节的特定区域上。最佳的肌肉功能依赖于这种高水平的组织，但它是如何建立和维持的还不是很清楚。由于其体外发育和透明性，斑马鱼胚胎是研究发育过程中细胞内和细胞间动态关系的优秀脊椎动物模型。转基因模型可以描述骨骼肌细胞分化过程中的细胞迁移和复杂的形态重排，以及新肌原纤维的组装和组织。基因和转录本的分子靶向已经允许鉴定肌原纤维组装和组织的关键要求。随着基因编辑方法的最新进展，斑马鱼将成为研究人类肌病和肌肉萎缩症的越来越重要的模型。它的高繁殖力和小体积使它非常适合于高通量筛选，以确定新的药理学和分子疗法，用于治疗广泛的神经肌肉疾病。在这篇综述中，我们研究了从斑马鱼模型中获得的关于肌节和肌膜之间复杂相互作用的经验教训，并讨论了斑马鱼作为模型系统的潜力，以检查人类肌病和肌肉萎缩症的病理生理学，并确定新的治疗方法。
Striated muscle has a highly ordered structure in which specialized domains of the cell membrane involved in force transmission (costameres) and excitation-contraction coupling (T tubules) as well as the internal membranes of the sarcoplasmic reticulum are organized over specific regions of the sarcomere. Optimal muscle function is dependent on this high level of organization but how it established and maintained is not well understood. Due to its ex utero development and transparency, the zebrafish embryo is an excellent vertebrate model for the study of dynamic relationships both within and between cells during development. Transgenic models have allowed the delineation of cellular migration and complex morphogenic rearrangements during the differentiation of skeletal myocytes and the assembly and organization of new myofibrils. Molecular targeting of genes and transcripts has allowed the identification of key requirements for myofibril assembly and organization. With the recent advances in gene editing approaches, the zebrafish will become an increasingly important model for the study of human myopathies and muscular dystrophies. Its high fecundity and small size make it well suited to high-throughput screenings to identify novel pharmacologic and molecular therapies for the treatment of a broad range of neuromuscular conditions. In this review, we examine the lessons learned from the zebrafish model regarding the complex interactions between the sarcomere and the sarcolemma that pattern the developing myocyte and discuss the potential for zebrafish as a model system to examine the pathophysiology of, and identify new treatments for, human myopathies and muscular dystrophies.
登录
查看更多内容
影响因子：
3.5
作者：
Gupta, Vandana;Kawahara, Genri;Beggs, Alan H.
通讯作者：
Beggs, Alan H.
影响因子：
16.6
作者：
通讯作者：
--
影响因子：
10.5
作者：
Durbin, L;Brennan, C;Holder, N
通讯作者：
Holder, N
影响因子：
2
作者：
Blackburn, Patrick R.;Campbell, Jarryd M.;Ekker, Stephen C.
通讯作者：
Ekker, Stephen C.
DOI：
10.1111/febs.12412
发表时间：
2013-09
期刊：
The FEBS journal
影响因子：
--
作者：
Gibbs EM;Horstick EJ;Dowling JJ
通讯作者：
Dowling JJ