Mutations in human caveolin-3 are known to underlie a range of myopathies. The cav-1 gene of Caenorhabditis elegans is a homologue of human caveolin-3 and is expressed in both neurons and body wall muscles. Within the body wall muscle CAV-1 localises adjacent to neurons, most likely at the neuromuscular junction (NMJ). Using fluorescently tagged CAV-1 and pre- and post-synaptic markers we demonstrate that CAV-1 co-localises with UNC-63, a post-synaptic marker, but not with several pre-synaptic markers. To establish a model for human muscular dystrophies caused by dominant-negative mutations in caveolin-3 we created transgenic animals carrying versions of cav-1 with homologous mutations. These animals had increased sensitivity to levamisole, suggesting a role for cav-1 at the NMJ. Animals carrying a deletion in cav-1 show a similar sensitivity. Sensitivity to levamisole and locomotion were also perturbed in animals carrying a dominant-negative cav-1 and a mutation in dynamin, which is a protein known to interact with caveolins. Thus, indicating an interaction between CAV-1 and dynamin at the NMJ and/or in neurons.

中文翻译：

---

肌营养不良相关的caveolin-1突变诱导秀丽隐杆线虫的神经传递和运动缺陷。

已知人类小窝蛋白 3 的突变是一系列肌病的基础。Caenorhabditis elegans 的 cav-1 基因是人类小窝蛋白 3 的同源物，在神经元和体壁肌肉中均有表达。在体壁肌肉内，CAV-1 定位于神经元附近，最有可能位于神经肌肉接头 (NMJ)。使用荧光标记的 CAV-1 和突触前和突触后标记，我们证明 CAV-1 与突触后标记 UNC-63 共定位，但不与几个突触前标记共定位。为了建立由caveolin-3 显性负突变引起的人类肌肉营养不良模型，我们创造了携带具有同源突变的cav-1 版本的转基因动物。这些动物对左旋咪唑的敏感性增加，表明 Cav-1 在 NMJ 中的作用。携带cav-1缺失的动物表现出类似的敏感性。携带显性阴性 cav-1 和 dynamin 突变的动物对左旋咪唑和运动的敏感性也受到干扰，dynamin 是一种已知与小窝蛋白相互作用的蛋白质。因此，表明 CAV-1 与 NMJ 和/或神经元中的动力蛋白之间的相互作用。