Cilia-related disease model and use thereof
|Appl. No. (filing date)||PCT/JP2020/028474 (2020/07/22)|
|Pub. No. (Pub. date)||WO2021/15245 (2021/01/28)|
|Priority claim No. (Priority date)||
|Inventor||OSAFUNE Kenji, etc.|
|Title of Invention||Cilia-related disease model and use thereof|
The invention relates to a renal cyst-forming model obtained from a renal organoid induced from an iPS cell derived from a patient with a cilia-related disease such as autosomal dominant polycystic kidney (ADPKD), and a method for screening or evaluating a therapeutic agent for cilia-related diseases using the same, wherein the cyst-formation is promoted by culturing in the presence of forskolin or Blebbistatin, etc. The renal cyst-forming model of the invention recapitulates the cilia-related disease phenotype such as cyst formation and decreased calcium release, and thus enables an efficient and highly accurate screening and evaluation of candidate therapeutic agents for cilia-related diseases such as ADPKD by using as indices the morphology of renal cysts and calcium kinetics.