C. elegans dystroglycan DGN-1 functions in epithelia and neurons, but not muscle, and independently of dystrophin
C. elegans dystroglycan DGN-1 functions in epithelia and neurons, but not muscle, and independently of dystrophin
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DOI：
10.1242/dev.02363
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发表时间：
2006-05-15
期刊：
影响因子：
4.6
通讯作者：
Kramer, JM
中科院分区：
文献类型：
--
作者：
Johnson, RP;Kang, SH;Kramer, JM
线虫营养不良聚糖(DG)同源物DGN-1在上皮和神经元中表达，定位于基底膜(BM)表面。与脊椎动物DG不同，DGN-1在肌肉中不表达，也不是肌肉功能所必需的。DGN-1缺失突变体是可存活的，但由于体细胞性腺上皮严重紊乱而不育，并且在外阴和排泄细胞上皮以及运动神经元轴突引导方面存在缺陷。这些缺陷类似于epi-1层粘连蛋白αB突变体的缺陷，表明DGN-1是层粘连蛋白的受体。DGN-1(0)/+动物可以生育，但除了纯合子的缺陷外，还表现出性腺迁移缺陷，这表明DGN-1的功能是剂量敏感的。表型分析表明，DGN-1和dystrophin相关蛋白复合体(DAPC)具有不同的独立功能，这与脊椎动物肌肉中的情况相反。DGN-1在上皮和神经元中的DAPC非依赖性功能表明，脊椎动物DG也可能独立于非肌肉组织中的dystrophin/utroin发挥作用。
The C. elegans dystroglycan (DG) homolog DGN-1 is expressed in epithelia and neurons, and localizes to basement membrane ( BM) surfaces. Unlike vertebrate DG, DGN-1 is not expressed in muscle or required for muscle function. dgn-1 null mutants are viable but sterile owing to severe disorganization of the somatic gonad epithelium, and show defects in vulval and excretory cell epithelia and in motoneuron axon guidance. The defects resemble those of epi-1 laminin alpha B mutants, suggesting that DGN-1 serves as a receptor for laminin. dgn-1(0)/+ animals are fertile but show gonad migration defects in addition to the defects seen in homozygotes, indicating that DGN-1 function is dosage sensitive. Phenotypic analyses show that DGN-1 and dystrophin-associated protein complex ( DAPC) components have distinct and independent functions, in contrast to the situation in vertebrate muscle. The DAPC-independent functions of DGN-1 in epithelia and neurons suggest that vertebrate DG may also act independently of dystrophin/utrophin in non-muscle tissues.