Wild-type uromodulin prevents NFkB activation in kidney cells, while mutant uromodulin, causing FJHU nephropathy, does not

• Published in: Journal of nephrology Vol. 27; no. 3; pp. 257 - 264
• Main Authors: Dinour, Dganit, Ganon, Liat, Nomy, Levin-Iaina, Ron, Rotem, Holtzman, Eliezer J.
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.06.2014
• Subjects: Active Transport, Cell Nucleus; Animals; Endoplasmic Reticulum - metabolism; Gout - genetics; Gout - metabolism; HEK293 Cells; Humans; Hyperuricemia - genetics; Hyperuricemia - metabolism; I-kappa B Proteins - metabolism; Interleukin-1 Receptor-Associated Kinases - metabolism; Interleukin-1beta - pharmacology; Kidney - drug effects; Kidney - metabolism; Kidney Diseases - genetics; Kidney Diseases - metabolism; Medicine; Medicine & Public Health; Mice; Mutation; Nephrology; NF-KappaB Inhibitor alpha; Original Article; Signal Transduction; Transcription Factor RelA - metabolism; Transfection; Urology; Uromodulin - genetics; Uromodulin - metabolism; Tamm-Horsfall protein; NFkB; Uric acid; Uromodulin; IL-1; Juvenile hyperuricemic nephropathy

Background Uromodulin (Tamm-Horsfall protein) is the most abundant urinary protein in healthy individuals. Despite 60 years of research, its physiological role remains rather elusive. Familial juvenile hyperuricemic nephropathy and medullary cystic kidney disease Type 2 are autosomal dominant tubulo...