Department of Biochemistry, Panjab University, Chandigarh, India.
J Environ Pathol Toxicol Oncol. 2012;31(3):285-93. doi: 10.1615/jenvironpatholtoxicoloncol.v31.i3.90.
Polycystic kidney disease (PKD) is associated with alterations in developmental processes that severely affect kidney integrity, often leading to fatal consequences. It has been suggested that dysfunctional calcium (Ca2+) regulation associated with the PKD phenotype is consequent to mutations affecting the pkd1 gene. Previously, it has been observed that blocking calcium along with cAMP allowed tubular epithelial cells to enter the proliferative phase that culminated in a cyst-like phenotype. In this regard, mouse metanephroi, (embryonic day 13.5, E13.5) were used to study morphological and ultrastructural effects of calcium replenishment on 8-bromocyclic 3'5'cyclic adenosine monophosphate (8-Br-cAMP)-induced cyst-like tubular dilations. Phase contrast microscopy of 8-Br-cAMP-treated metanephroi exhibited numerous dilated tubules that continued to increase in size for 4 days in culture. The effects of 8-Br-cAMP on renal tubular epithelia were assessed by histopathological and electron microscopic analyses. Transmission electron microscopy revealed changes such as increased vacuolation, swollen mitochondria, chromatin condensation, and disrupted cell membrane in tubular epithelia of 8-Br-cAMP-treated metanephroi. Concurrent treatments with calcium-channel agonists (calcium ionophore A23187 and phorbol-12-myristate-13-acetate) and 8-Br-cAMP abolished cAMP-induced morphometric and ultrastructural alterations. Calcium replenishment rescued tubular epithelial cells from mitogenic effects of cAMP and restored normal morphology at cellular and sub-cellular levels as verified by histopathological and ultrastructural examinations.
多囊肾病 (PKD) 与发育过程中的改变有关,这些改变严重影响肾脏的完整性,经常导致致命的后果。有人认为,与 PKD 表型相关的功能失调的钙 (Ca2+) 调节是由于影响 pkd1 基因的突变所致。此前,人们观察到,阻断钙和 cAMP 可以使肾小管上皮细胞进入增殖期,最终形成囊泡样表型。在这方面,使用小鼠后肾原基(胚胎第 13.5 天,E13.5)研究了钙补充对 8-溴环磷酸腺苷(8-Br-cAMP)诱导的囊泡样管状扩张的形态和超微结构的影响。8-Br-cAMP 处理的后肾原基的相差显微镜观察显示,许多扩张的小管在培养中继续增大,持续 4 天。通过组织病理学和电子显微镜分析评估 8-Br-cAMP 对肾小管上皮细胞的影响。透射电子显微镜显示,8-Br-cAMP 处理的后肾原基的肾小管上皮细胞发生了变化,如空泡化增加、线粒体肿胀、染色质浓缩和细胞膜破裂。钙通道激动剂(钙离子载体 A23187 和佛波醇 12-肉豆蔻酸 13-乙酸酯)和 8-Br-cAMP 的联合处理消除了 cAMP 诱导的形态计量和超微结构改变。钙补充使管状上皮细胞免受 cAMP 的有丝分裂作用的影响,并通过组织病理学和超微结构检查证实,在细胞和亚细胞水平恢复了正常形态。