Li Sienna R, Gulieva Ramila E, Helms Louisa, Cruz Nelly M, Vincent Thomas, Fu Hongxia, Himmelfarb Jonathan, Freedman Benjamin S
Division of Nephrology, University of Washington School of Medicine, Seattle, WA, 98109, USA.
Kidney Research Institute, University of Washington School of Medicine, Seattle, WA, 98109, USA.
Nat Commun. 2022 Dec 23;13(1):7918. doi: 10.1038/s41467-022-35537-2.
In polycystic kidney disease (PKD), fluid-filled cysts arise from tubules in kidneys and other organs. Human kidney organoids can reconstitute PKD cystogenesis in a genetically specific way, but the mechanisms underlying cystogenesis remain elusive. Here we show that subjecting organoids to fluid shear stress in a PKD-on-a-chip microphysiological system promotes cyst expansion via an absorptive rather than a secretory pathway. A diffusive static condition partially substitutes for fluid flow, implicating volume and solute concentration as key mediators of this effect. Surprisingly, cyst-lining epithelia in organoids polarize outwards towards the media, arguing against a secretory mechanism. Rather, cyst formation is driven by glucose transport into lumens of outwards-facing epithelia, which can be blocked pharmacologically. In PKD mice, glucose is imported through cysts into the renal interstitium, which detaches from tubules to license expansion. Thus, absorption can mediate PKD cyst growth in human organoids, with implications for disease mechanism and potential for therapy development.
在多囊肾病(PKD)中,充满液体的囊肿起源于肾脏和其他器官的肾小管。人类肾脏类器官可以以基因特异性方式重建PKD囊肿形成过程,但囊肿形成的潜在机制仍不清楚。在这里,我们表明,在PKD芯片微生理系统中对类器官施加流体剪切应力可通过吸收而非分泌途径促进囊肿扩张。扩散静态条件部分替代了流体流动,这表明体积和溶质浓度是这种效应的关键介质。令人惊讶的是,类器官中的囊肿内衬上皮细胞向外朝着培养基极化,这与分泌机制相悖。相反,囊肿形成是由葡萄糖转运到朝外上皮细胞的管腔中驱动的,这一过程可被药物阻断。在PKD小鼠中,葡萄糖通过囊肿进入肾间质,肾间质与肾小管分离从而促进囊肿扩张。因此,吸收可介导人类类器官中PKD囊肿的生长,这对疾病机制及治疗开发潜力具有重要意义。