Grisoni Karine, Gieseler Kathrin, Mariol Marie Christine, Martin Edwige, Carre-Pierrat Maité, Moulder Gary, Barstead Robert, Ségalat Laurent
CGMC, CNRS-UMR 5534, Université Lyon-1, 43 Bid du 11 Novembre, 69622, Villeurbanne, cedex, France.
J Mol Biol. 2003 Oct 3;332(5):1037-46. doi: 10.1016/j.jmb.2003.08.021.
Syntrophins are a family of PDZ domain-containing adaptor proteins required for receptor localization. Syntrophins are also associated with the dystrophin complex in muscles. We report here the molecular and functional characterization of the Caenorhabditis elegans gene stn-1 (F30A10.8), which encodes a syntrophin with homology to vertebrate alpha and beta-syntrophins. stn-1 is expressed in neurons and in muscles of C.elegans. stn-1 mutants resemble dystrophin (dys-1) and dystrobrevin (dyb-1) mutants: they are hyperactive, bend their heads when they move forward, tend to hypercontract, and are hypersensitive to the acetylcholinesterase inhibitor aldicarb. These phenotypes are suppressed when stn-1 is expressed under the control of a muscular promoter, indicating that they are caused by the absence of stn-1 in muscles. These results suggest that the role of syntrophin is linked to dystrophin function in C.elegans.
肌养蛋白是一类含PDZ结构域的衔接蛋白家族，是受体定位所必需的。肌养蛋白也与肌肉中的肌营养不良蛋白复合体相关。我们在此报告秀丽隐杆线虫基因stn-1（F30A10.8）的分子和功能特征，该基因编码一种与脊椎动物α和β-肌养蛋白具有同源性的肌养蛋白。stn-1在秀丽隐杆线虫的神经元和肌肉中表达。stn-1突变体类似于肌营养不良蛋白（dys-1）和肌萎缩蛋白（dyb-1）突变体：它们活动亢进，向前移动时会低头，易于过度收缩，并且对乙酰胆碱酯酶抑制剂涕灭威高度敏感。当stn-1在肌肉启动子的控制下表达时，这些表型会受到抑制，这表明它们是由肌肉中stn-1的缺失引起的。这些结果表明，在秀丽隐杆线虫中，肌养蛋白的作用与肌营养不良蛋白的功能相关。
