McKeown Caroline R, Han Hsiao-Fen, Beckerle Mary C
Huntsman Cancer Institute, Department of Biology, University of Utah, Salt Lake City, Utah 84112, USA.
Dev Dyn. 2006 Feb;235(2):530-8. doi: 10.1002/dvdy.20633.
Members of the ALP/Enigma family of PDZ-LIM proteins play a role in cytoskeletal anchorage and mutations in at least one member of this family are associated with human cardiomyopathy. Here, we describe the analysis of the Caenorhabditis elegans alp-1 gene. alp-1 is predicted to encode the entire nematode ALP/Enigma protein family, consisting of one ALP-related protein with a single LIM domain and three Enigma-like proteins containing four LIM domains. We demonstrate that the ALP-1 proteins are expressed in muscle cells, where they localize to actin anchorage and muscle attachment sites. We show that the PDZ domain of the ALP-1 proteins is sufficient to target the protein to the dense bodies, which are important actin anchorage sites in C. elegans body wall muscle. We demonstrate that the C. elegans ALP/Enigma proteins are also localized to cell-cell junctions and to both epithelial and muscle cell nuclei. These findings suggest new roles for the ALP/Enigma protein family that may lead to the understanding of their involvement in cardiomyopathy.
PDZ-LIM蛋白家族的ALP/Enigma成员在细胞骨架锚定中发挥作用,该家族至少一个成员的突变与人类心肌病相关。在此,我们描述了秀丽隐杆线虫alp-1基因的分析。alp-1预计编码整个线虫ALP/Enigma蛋白家族,该家族由一个具有单个LIM结构域的ALP相关蛋白和三个含有四个LIM结构域的类Enigma蛋白组成。我们证明ALP-1蛋白在肌肉细胞中表达,它们定位于肌动蛋白锚定和肌肉附着位点。我们表明ALP-1蛋白的PDZ结构域足以将该蛋白靶向致密体,致密体是秀丽隐杆线虫体壁肌肉中重要的肌动蛋白锚定位点。我们证明秀丽隐杆线虫的ALP/Enigma蛋白也定位于细胞间连接以及上皮细胞核和肌肉细胞核。这些发现提示了ALP/Enigma蛋白家族的新作用,可能有助于理解它们在心肌病中的作用。