Type of uromodulin mutation and allelic status influence onset and severity of uromodulin-associated kidney disease in mice.
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Protein trafficking defects in inherited kidney diseasesUromodulin retention in thick ascending limb of Henle's loop affects SCD1 in neighboring proximal tubule: renal transcriptome studies in mouse models of uromodulin-associated kidney diseaseGeneration and Standardized, Systemic Phenotypic Analysis of Pou3f3L423P Mutant MiceA mouse model for inherited renal fibrosis associated with endoplasmic reticulum stress.Standardized, systemic phenotypic analysis of Umod(C93F) and Umod(A227T) mutant mice.Missense Mutation of POU Domain Class 3 Transcription Factor 3 in Pou3f3L423P Mice Causes Reduced Nephron Number and Impaired Development of the Thick Ascending Limb of the Loop of Henle.Mutant uromodulin expression leads to altered homeostasis of the endoplasmic reticulum and activates the unfolded protein response.Mitochondrial Dysregulation Secondary to Endoplasmic Reticulum Stress in Autosomal Dominant Tubulointerstitial Kidney Disease - UMOD (ADTKD-UMOD)Uromodulin: from physiology to rare and complex kidney disorders.No amelioration of uromodulin maturation and trafficking defect by sodium 4-phenylbutyrate in vivo: studies in mouse models of uromodulin-associated kidney disease.Early involvement of cellular stress and inflammatory signals in the pathogenesis of tubulointerstitial kidney disease due to UMOD mutations.Standardized, systemic phenotypic analysis reveals kidney dysfunction as main alteration of Kctd1 I27N mutant mice.Elevated urinary CRELD2 is associated with endoplasmic reticulum stress-mediated kidney disease.
P2860
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P2860
Type of uromodulin mutation and allelic status influence onset and severity of uromodulin-associated kidney disease in mice.
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name
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@en
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@nl
type
label
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@en
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@nl
prefLabel
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@en
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@nl
P2093
P2860
P50
P356
P1476
Type of uromodulin mutation an ...... ciated kidney disease in mice.
@en
P2093
Birgit Rathkolb
Helmut Fuchs
Petra Prueckl
Stefanie Sklenak
Valérie Gailus-Durner
Wolfgang Hans
P2860
P304
P356
10.1093/HMG/DDT263
P577
2013-06-06T00:00:00Z