A mouse model for inherited renal fibrosis associated with endoplasmic reticulum stress
Renal fibrosis is a common feature of renal failure resulting from multiple etiologies,
including diabetic nephropathy, hypertension and inherited renal disorders. However, the …
including diabetic nephropathy, hypertension and inherited renal disorders. However, the …
Allelic effects on uromodulin aggregates drive autosomal dominant tubulointerstitial kidney disease
G Schiano, J Lake, M Mariniello… - EMBO molecular …, 2023 - embopress.org
Missense mutations in the uromodulin (UMOD) gene cause autosomal dominant
tubulointerstitial kidney disease (ADTKD), one of the most common monogenic kidney …
tubulointerstitial kidney disease (ADTKD), one of the most common monogenic kidney …
Early involvement of cellular stress and inflammatory signals in the pathogenesis of tubulointerstitial kidney disease due to UMOD mutations
M Trudu, C Schaeffer, M Riba, M Ikehata… - Scientific Reports, 2017 - nature.com
Autosomal dominant tubulointerstitial kidney disease (ADTKD) is an inherited disorder that
causes progressive kidney damage and renal failure. Mutations in the UMOD gene …
causes progressive kidney damage and renal failure. Mutations in the UMOD gene …
Uromodulin-related autosomal-dominant tubulointerstitial kidney disease—pathogenetic insights based on a case
J Reindl, HJ Gröne, G Wolf, M Busch - Clinical Kidney Journal, 2019 - academic.oup.com
Uromodulin-related autosomal-dominant tubulointerstitial kidney disease (ADTKD-UMOD) is
a rare monogenic disorder that is characterized by tubulointerstitial fibrosis and progression …
a rare monogenic disorder that is characterized by tubulointerstitial fibrosis and progression …
[HTML][HTML] Upregulation of C/EBP homologous protein induced by ER stress mediates epithelial to Myofibroblast transformation in ADTKD-UMOD
D Wang, Y Qiu, J Fan, Y Liu, W Chen, Z Li… - … Journal of Medical …, 2022 - ncbi.nlm.nih.gov
Autosomal dominant tubulointerstitial kidney disease due to UMOD mutations (ADTKD-
UMOD) results in chronic interstitial nephritis, which gradually develops into end-stage renal …
UMOD) results in chronic interstitial nephritis, which gradually develops into end-stage renal …
A transgenic mouse model for uromodulin-associated kidney diseases shows specific tubulo-interstitial damage, urinary concentrating defect and renal failure
I Bernascone, S Janas, M Ikehata… - Human molecular …, 2010 - academic.oup.com
Uromodulin-associated kidney diseases (UAKD) are autosomal-dominant disorders
characterized by alteration of urinary concentrating ability, tubulo-interstitial fibrosis …
characterized by alteration of urinary concentrating ability, tubulo-interstitial fibrosis …
Mutant uromodulin expression leads to altered homeostasis of the endoplasmic reticulum and activates the unfolded protein response
C Schaeffer, S Merella, E Pasqualetto, D Lazarevic… - PLoS …, 2017 - journals.plos.org
Uromodulin is the most abundant urinary protein in physiological conditions. It is exclusively
produced by renal epithelial cells lining the thick ascending limb of Henle's loop (TAL) and it …
produced by renal epithelial cells lining the thick ascending limb of Henle's loop (TAL) and it …
Uromodulin p. Cys147Trp mutation drives kidney disease by activating ER stress and apoptosis
BG Johnson, LT Dang, G Marsh… - The Journal of …, 2017 - Am Soc Clin Investig
Uromodulin-associated kidney disease (UAKD) is caused by mutations in the uromodulin
(UMOD) gene that result in a misfolded form of UMOD protein, which is normally secreted by …
(UMOD) gene that result in a misfolded form of UMOD protein, which is normally secreted by …
Mitochondrial Dysregulation Secondary to Endoplasmic Reticulum Stress in Autosomal Dominant Tubulointerstitial Kidney Disease – UMOD (ADTKD-UMOD)
Abstract 'Autosomal dominant tubulointerstitial kidney disease–UMOD'(ADTKD-UMOD) is
caused by impaired maturation and secretion of mutant uromodulin (UMOD) in thick …
caused by impaired maturation and secretion of mutant uromodulin (UMOD) in thick …
[HTML][HTML] Autosomal dominant tubulointerstitial kidney disease—uromodulin misclassified as focal segmental glomerulosclerosis or hereditary glomerular disease
Introduction Focal segmental glomerulosclerosis (FSGS) is a histopathologically defined
kidney lesion. FSGS can be observed with various underlying causes, including highly …
kidney lesion. FSGS can be observed with various underlying causes, including highly …