| 000 | 03774 am a22006613u 4500 | ||
|---|---|---|---|
| 042 | _adc | ||
| 100 | 1 | 0 |
_aXu, Yaoxian _eauthor _91567 |
| 700 | 1 | 0 |
_aKuppe, Christoph _eauthor _91568 |
| 700 | 1 | 0 |
_aPerales-Patón, Javier _eauthor _91569 |
| 700 | 1 | 0 |
_aHayat, Sikander _eauthor _91570 |
| 700 | 1 | 0 |
_aKranz, Jennifer _eauthor _91571 |
| 700 | 1 | 0 |
_aAbdallah, Ali T. _eauthor _91572 |
| 700 | 1 | 0 |
_aNagai, James _eauthor _91573 |
| 700 | 1 | 0 |
_aLi, Zhijian _eauthor _91574 |
| 700 | 1 | 0 |
_aPeisker, Fabian _eauthor _91575 |
| 700 | 1 | 0 |
_aSaritas, Turgay _eauthor _91576 |
| 700 | 1 | 0 |
_aHalder, Maurice _eauthor _91577 |
| 700 | 1 | 0 |
_aMenzel, Sylvia _eauthor _91578 |
| 700 | 1 | 0 |
_aHoeft, Konrad _eauthor _91579 |
| 700 | 1 | 0 |
_aKenter, Annegien _eauthor _91580 |
| 700 | 1 | 0 |
_aKim, Hyojin _eauthor _91581 |
| 700 | 1 | 0 |
_avan Roeyen, Claudia R. C. _eauthor _91582 |
| 700 | 1 | 0 |
_aLehrke, Michael _eauthor _91583 |
| 700 | 1 | 0 |
_aMoellmann, Julia _eauthor _91584 |
| 700 | 1 | 0 |
_aSpeer, Thimoteus _eauthor _91585 |
| 700 | 1 | 0 |
_aBuhl, Eva M. _eauthor _91586 |
| 700 | 1 | 0 |
_aHoogenboezem, Remco _eauthor _91587 |
| 700 | 1 | 0 |
_aBoor, Peter _eauthor _91588 |
| 700 | 1 | 0 |
_aJansen, Jitske _eauthor _91589 |
| 700 | 1 | 0 |
_aKnopp, Cordula _eauthor _91590 |
| 700 | 1 | 0 |
_aKurth, Ingo _eauthor _91591 |
| 700 | 1 | 0 |
_aSmeets, Bart _eauthor _91592 |
| 700 | 1 | 0 |
_aBindels, Eric _eauthor _91593 |
| 700 | 1 | 0 |
_aReinders, Marlies E. J. _eauthor _91594 |
| 700 | 1 | 0 |
_aBaan, Carla _eauthor _91595 |
| 700 | 1 | 0 |
_aGribnau, Joost _eauthor _91596 |
| 700 | 1 | 0 |
_aHoorn, Ewout J. _eauthor _91597 |
| 700 | 1 | 0 |
_aSteffens, Joachim _eauthor _91598 |
| 700 | 1 | 0 |
_aHuber, Tobias B _eauthor _91599 |
| 700 | 1 | 0 |
_aCosta, Ivan _eauthor _91600 |
| 700 | 1 | 0 |
_aFloege, Jürgen _eauthor _91601 |
| 700 | 1 | 0 |
_aSchneider, Rebekka K. _eauthor _91602 |
| 700 | 1 | 0 |
_aSaez-Rodriguez, Julio _eauthor _91603 |
| 700 | 1 | 0 |
_aFreedman, Benjamin S. _eauthor _91604 |
| 700 | 1 | 0 |
_aKramann, Rafael _eauthor _91605 |
| 245 | 0 | 0 | _aAdult human kidney organoids originate from CD24(+) cells and represent an advanced model for adult polycystic kidney disease |
| 260 | _c2022-11. | ||
| 500 | _a/pmc/articles/PMC7613830/ | ||
| 500 | _a/pubmed/36303074 | ||
| 520 | _aAdult kidney organoids have been described as strictly tubular epithelial and termed tubuloids. While the cellular origin of tubuloids has remained elusive, here we report that they originate from a distinct CD24(+) epithelial subpopulation. Long-term-cultured CD24(+)-derived tubuloids represent a functional human kidney tubule. We show that kidney tubuloids can be used to model the most common inherited kidney disease, namely autosomal dominant polycystic kidney disease (ADPKD), reconstituting the phenotypic hallmark of this disease with cyst formation. Single-cell RNA sequencing of CRISPR/Cas9 gene-edited PKD1 and PKD2 knockout tubuloids and human ADPKD and control tissue show similarities in upregulation of disease-driving genes. Furthermore, in a proof of concept, we demonstrate that tolvaptan, the only approved drug for ADPKD, shows a significant effect on cyst size in tubuloids but no effect in a pluripotent-stem-cell-derived model. Thus, tubuloids derive from a tubular epithelial subpopulation and represent an advanced model for ADPKD disease modeling. | ||
| 540 | _a | ||
| 540 | _ahttps://www.springernature.com/gp/open-research/policies/accepted-manuscript-termsUsers may view, print, copy, and download text and data-mine the content in such documents, for the purposes of academic research, subject always to the full Conditions of use: https://www.springernature.com/gp/open-research/policies/accepted-manuscript-terms | ||
| 546 | _aen | ||
| 690 | _aArticle | ||
| 655 | 7 |
_aText _2local |
|
| 786 | 0 | _nNat Genet | |
| 856 | 4 | 1 |
_uhttp://dx.doi.org/10.1038/s41588-022-01202-z _zConnect to this object online. |
| 999 |
_c1716 _d1716 |
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