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obscurin缺失会损害斑马鱼胚胎发育过程中骨骼肌的组织形成。

Obscurin depletion impairs organization of skeletal muscle in developing zebrafish embryos.

作者信息

Raeker Maide Ö, Russell Mark W

机构信息

Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI 48109, USA.

出版信息

J Biomed Biotechnol. 2011;2011:479135. doi: 10.1155/2011/479135. Epub 2011 Nov 15.

Abstract

During development, skeletal myoblasts differentiate into myocytes and skeletal myotubes with mature contractile structures that are precisely oriented with respect to surrounding cells and tissues. Establishment of this highly ordered structure requires reciprocal interactions between the differentiating myocytes and the surrounding extracellular matrix to form correctly positioned and well-organized attachments from the skeletal muscle to the bony skeleton. Using the developing zebrafish embryo as a model, we examined the relationship between new myofibril assembly and the organization of the membrane domains involved in cell-extracellular matrix interactions. We determined that depletion of obscurin, a giant muscle protein, resulted in irregular cell morphology and disturbed extracellular matrix organization during skeletal muscle development. The resulting impairment of myocyte organization was associated with disturbance of the internal architecture of the myocyte suggesting that obscurin participates in organizing the internal structure of the myocyte and translating those structural cues to surrounding cells and tissues.

摘要

在发育过程中,骨骼肌成肌细胞分化为具有成熟收缩结构的肌细胞和骨骼肌肌管,这些收缩结构相对于周围细胞和组织精确排列。建立这种高度有序的结构需要分化中的肌细胞与周围细胞外基质之间的相互作用,以形成从骨骼肌到骨骼的正确定位和组织良好的附着。我们以发育中的斑马鱼胚胎为模型,研究了新的肌原纤维组装与参与细胞 - 细胞外基质相互作用的膜结构域组织之间的关系。我们确定, obscurin(一种巨大的肌肉蛋白)的缺失导致骨骼肌发育过程中细胞形态不规则和细胞外基质组织紊乱。由此导致的肌细胞组织受损与肌细胞内部结构紊乱有关,这表明obscurin参与组织肌细胞的内部结构,并将这些结构线索传递给周围的细胞和组织。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/028a/3228690/7302296a871b/JBB2011-479135.001.jpg

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