Cystic canal mutants in Caenorhabditis elegans are defective in the apical membrane domain of the renal (excretory) cell
Cystic canal mutants in Caenorhabditis elegans are defective in the apical membrane domain of the renal (excretory) cell
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DOI：
10.1006/dbio.1999.9398
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发表时间：
1999-10-01
影响因子：
2.7
通讯作者：
Hedgecock, EM
中科院分区：
文献类型：
--
作者：
Buechner, M;Hall, DH;Hedgecock, EM
排泄细胞沿着表皮的基底外侧表面沿着延伸管状突起或管，形成线虫肾上皮。这种细胞在分离的细胞培养物中可以进行正常的小管形成。12个基因的突变导致秀丽隐杆线虫排泄道囊肿。遗传相互作用和它们相似的表型表明这些基因可能编码功能相关的蛋白质。根据基因型和个别的管道，缺陷范围从局灶性囊肿，两侧正常宽度的部分，区域囊肿涉及整个小管。通常，扩大的区域是旋绕的或部分分隔的。在具有非常大的囊肿的突变体中，肾功能可测量地受损。根据组织学和超微结构，根管囊肿可能是由于根尖膜域的缺陷。这些突变体提供了一个没有增生或基底膜异常的小管囊性疾病模型。类似的顶端机制可以调节脊椎动物肾单位的管状形态。(C)北京：科学出版社.
The excretory cell extends a tubular process, or canal, along the basolateral surface of the epidermis to form the nematode renal epithelium. This cell can undergo normal tubulogenesis in isolated cell culture. Mutations in 12 genes cause excretory canal cysts in Caenorhabditis elegans. Genetic interactions, and their similar phenotypes, suggest these genes may encode functionally related proteins. Depending upon genotype and individual canal, defects range from focal cysts, flanked by normal width segments, to regional cysts involving the entire tubule. Oftentimes the enlarged regions are convoluted or partially septated. In mutants with very large cysts, renal function is measurably impaired. Based on histology and ultrastructure, canal cysts likely result from defects of the apical membrane domain. These mutants provide a model of tubulocystic disease without hyperplasia or basement membrane abnormalities. Similar apical mechanisms could regulate tubular morphology of vertebrate nephrons. (C) 1999 Academic Press.