Zhi-Ying Liu

Abstract Autosomal dominant tubulointerstitial kidney disease -UMOD is characterized by progressive renal interstitial inflammation and fibrosis. However, its underlying mechanisms remain unclear. Here, we identify a large ADTKD pedigree harboring a novel UMOD p.H36Y mutation. Using CRISPR/Cas9 technology, we generated a UmodH36Y/+ mouse model that recapitulates the key phenotypes observed in affected individuals, including renal dysfunction, cyst formation, and interstitial inflammation.

CONFLICT OF INTEREST STATEMENT The authors declare no conflict of interest. Supporting Information Filename Description pce14709-sup-0001-Supplemental_File.docx4.8 MB Supporting information. REFERENCES , , , , , et al. (2014) A conserved network of transcriptional activators and repressors regulates anthocyanin pigmentation in eudicots. The Plant Cell, 26, 962–980. , & (2008) MYB transcription factors that colour our fruit. Trends in Plant Science, 13, 99–102.

Research Article Dr. An is fully responsible for the distributions of all materials associated with this article.) Received Apr. 22, 2023; Accepted June 5, 2023; Edited by Deyu Xie, North Carolina State University, USA This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record.