Zennaro Cristina, Mariotti Massimo, Carraro Michele, Pasqualetti Sara, Corbelli Alessandro, Armelloni Silvia, Li Min, Ikehata Masami, Clai Milan, Artero Mary, Messa Piergiorgio, Boscutti Giuliano, Rastaldi Maria Pia
Department of Medical, Surgery and Health Sciences, Università degli Studi di Trieste, Trieste, Italy; Renal Research Laboratory, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico & Fondazione D'Amico per la Ricerca sulle Malattie Renali, Milano, Italy.
Department of Biomedical, Surgical and Dental Sciences, University of Milano & IRCCS Orthopedic Institute, Milano, Italy.
PLoS One. 2014 May 20;9(5):e98131. doi: 10.1371/journal.pone.0098131. eCollection 2014.
The zebrafish pronephros is gaining popularity in the nephrology community, because embryos are easy to cultivate in multiwell plates, allowing large number of experiments to be conducted in an in vivo model. In a few days, glomeruli reach complete development, with a structure that is similar to that of the mammalian counterpart, showing a fenestrated endothelium and a basement membrane covered by the multiple ramifications of mature podocytes. As a further advantage, zebrafish embryos are permeable to low molecular compounds, and this explains their extensive use in drug efficacy and toxicity experiments. Here we show that low concentrations of adriamycin (i.e. 10 and 20 µM), when dissolved in the medium of zebrafish embryos at 9 hours post-fertilization and removed after 48 hours (57 hpf), alter the development of podocytes with subsequent functional impairment, demonstrated by onset of pericardial edema and reduction of expression of the podocyte proteins nephrin and wt1. Podocyte damage is morphologically confirmed by electron microscopy and functionally supported by increased clearance of microinjected 70 kDa fluorescent dextran. Importantly, besides pericardial edema and glomerular damage, which persist and worsen after adriamycin removal from the medium, larvae exposed to adriamycin 10 and 20 µM do not show any myocardiocyte alterations nor vascular changes. The only extra-renal effect is a transient delay of cartilage formation that rapidly recovers once adriamycin is removed. In summary, this low dose adriamycin model can be applied to analyze podocyte developmental defects, such as those observed in congenital nephrotic syndrome, and can be taken in consideration for pharmacological studies of severe early podocyte injury.
斑马鱼前肾在肾脏病学界越来越受欢迎,因为胚胎易于在多孔板中培养,这使得在体内模型中能够进行大量实验。在几天内,肾小球就能发育完全,其结构与哺乳动物的肾小球相似,具有有孔的内皮和由成熟足细胞的多个分支覆盖的基底膜。另一个优势是,斑马鱼胚胎对低分子化合物具有通透性,这解释了它们在药物疗效和毒性实验中的广泛应用。我们在此表明,低浓度的阿霉素(即10和20 μM),在受精后9小时溶解于斑马鱼胚胎培养基中,并在48小时(57 hpf)后去除,会改变足细胞的发育,随后导致功能受损,表现为心包水肿的出现以及足细胞蛋白nephrin和wt1表达的降低。足细胞损伤通过电子显微镜在形态学上得到证实,并且通过微注射的70 kDa荧光葡聚糖清除率的增加在功能上得到支持。重要的是,除了心包水肿和肾小球损伤(在从培养基中去除阿霉素后持续存在并恶化)之外,暴露于10和20 μM阿霉素的幼虫未显示任何心肌细胞改变或血管变化。唯一的肾外效应是软骨形成的短暂延迟,一旦去除阿霉素,这种延迟会迅速恢复。总之,这种低剂量阿霉素模型可用于分析足细胞发育缺陷,例如在先天性肾病综合征中观察到的缺陷,并且可用于严重早期足细胞损伤的药理学研究。