期刊
DEVELOPMENTAL CELL
卷 22, 期 3, 页码 515-529出版社
CELL PRESS
DOI: 10.1016/j.devcel.2011.12.008
关键词
-
资金
- Boehringer Ingelheim
- European Commission
- ZF-Health
- Association Francaise contre lea Myopathies
Muscle cells have a remarkable capability to repair plasma membrane lesions. Mutations in dysferlin (dye) are known to elicit a progressive myopathy in humans, probably due to impaired sarcolemmal repair. We show here that loss of Dysf and annexin A6 (Anxa6) function lead to myopathy in zebrafish. By use of high-resolution imaging of myofibers in intact animals, we reveal sequential phases in sarcolemmal repair. Initially, membrane vesicles enriched in Dysf together with cytoplasmic Anxa6 form a tight patch at the lesion independently of one another. In the subsequent steps, annexin A2a (Anxa2a) followed by annexin A1a (Anxa1a) accumulate at the patch; the recruitment of these annexins depends on Dysf and Anxa6. Thus, sarcolemmal repair relies on the ordered assembly of a protein-membrane scaffold. Moreover, we provide several lines of evidence that the membrane for sarcolemmal repair is derived from a specialized plasma membrane co,partment.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据