Vanslambrouck Jessica M, Wilson Sean B, Tan Ker Sin, Groenewegen Ella, Rudraraju Rajeev, Neil Jessica, Lawlor Kynan T, Mah Sophia, Scurr Michelle, Howden Sara E, Subbarao Kanta, Little Melissa H
Murdoch Children's Research Institute, Flemington Rd, Parkville, VIC, Australia.
Department of Paediatrics, The University of Melbourne, VIC, Australia.
bioRxiv. 2022 May 27:2021.10.14.464320. doi: 10.1101/2021.10.14.464320.
While pluripotent stem cell-derived kidney organoids are now being used to model renal disease, the proximal nephron remains immature with limited evidence for key functional solute channels. This may reflect early mispatterning of the nephrogenic mesenchyme and/or insufficient maturation. Here we show that enhanced specification to metanephric nephron progenitors results in elongated and radially aligned proximalised nephrons with distinct S1 - S3 proximal tubule cell types. Such PT-enhanced organoids possess improved albumin and organic cation uptake, appropriate KIM-1 upregulation in response to cisplatin, and improved expression of SARS-CoV-2 entry factors resulting in increased viral replication. The striking proximo-distal orientation of nephrons resulted from localized WNT antagonism originating from the organoid stromal core. PT-enhanced organoids represent an improved model to study inherited and acquired proximal tubular disease as well as drug and viral responses.
虽然多能干细胞衍生的肾类器官目前正被用于模拟肾脏疾病,但近端肾单位仍然不成熟,关键功能性溶质通道的证据有限。这可能反映了肾发生间充质的早期模式错误和/或成熟不足。在这里,我们表明,增强向后肾肾单位祖细胞的特化会导致近端化的肾单位拉长并呈放射状排列,具有不同的S1 - S3近端小管细胞类型。这种PT增强的类器官具有改善的白蛋白和有机阳离子摄取、对顺铂有适当的KIM-1上调,以及改善的SARS-CoV-2进入因子表达,导致病毒复制增加。肾单位显著的近端到远端方向是由源自类器官基质核心的局部WNT拮抗作用引起的。PT增强的类器官代表了一种改进的模型,用于研究遗传性和获得性近端肾小管疾病以及药物和病毒反应。