Dataset Information


Transcriptional profiling of a mouse model of Uromodulin Associated Kidney Disease (UAKD)

ABSTRACT: Uromodulin (UMOD) is a secreted glycoprotein exclusively expressed by the cells lining the thick ascending limb of the loop of henle and the early distal tubule of the kidney nephron. Mutations in UMOD that interfere with proper folding of the protein are responsible for a progressive form of interstitial fibrotic kidney disease that leads to end stage renal disease, referred to as Uromodulin Associated Kidney Disease (UAKD). To assess key transcriptional changes associated with the progression of UAKD, we generated a knock-in mouse model harboring the mouse equivalent of the human mutation C148W. We profiled both the whole kidney tissue, as well as the specific UMOD+ cell populaitons in mutant and wild type mice. Analysis of differentially expressed genes in whole tissue and UMOD+ cells revealed a strong TNF-signaling signature, as well as TRIB3 upregulation, which is a key mediator of the intrinsic ER-stress mediated cell death pathway. Overall design: Uromodulin mutant and wild type littermate controls were aged to 6, 12, 24, and 30 weeks of age. Total RNA was isolated from whole kidney tissue and from isolated, Uromodulin positive cells at each timepoint.

INSTRUMENT(S): Illumina HiSeq 2500 (Mus musculus)

SUBMITTER: Suzanne Szak 

PROVIDER: GSE102566 | GEO | 2017-09-01



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