Characterization of the pathophysiologie of uromodulin-associated kidney disease
Final Report Abstract
Each objective of the research grant were successfully adressed and numerous new so far unknown pathomachanisms of UAKD were identified. Both in vivo UAKD models of two independent Umod mutant mouse lines revealed to be of important value. For instance, so far commonly used in vitro models for this disease were recently described to be not suitbale to analyze potential disease mechanisms in UAKD. This is in line with the findings of the studies of this DFG-funded project in testing the effect of 4-PBA to influence ER trafficking defect of mutant UMOD. A putative therapeutic use of 4-PBA was proposed by two independent research groups due to their in vitro results, but in the studies of this DFG-funded project, even a deterioration of kidney function in UAKD in vivo was detected. Within this funding period, new objectives were evolved, for instance what is the mechanisms why UAKD affected kidneys seemed to be protected against acute ischemic reperfusion injury.
Publications
- Role of NF-κB in Uromodulin-associated kidney disease. 51th ERA-EDTA Congress, Amsterdam, The Netherlands, 31.05.-03.05.2014
Kemter E, Aigner B, Wolf E, Wanke R
- Pathophysiology of Uromodulin-associated kidney disease: what we can learn from Umod-mutant mice. 29th meeting of the European Society of Veterinary Pathology (ESVP) and 9th European Congress of Toxicologic Pathology of the European Society of Toxicologic Pathology (ESTP). Uppsala, Sweden, 7.-10.09.2011
Kemter E, Sklenak S, Prueckl P, Rathkolb R, Hrabé de Angelis M, Wolf E, Aigner B, and Wanke R
- Progression der Nierenveränderungen in mutanten Mausmodellen für die Uromodulin-assoziierte Nierenerkrankung. 4. Jahrestagung der Deutschen Gesellschaft für Nephrologie, 06.-09.10.2012, Hamburg. DGfN Heft 04/2012, Seite 51
Kemter E, Sklenak S, Prueckl P, Rathkolb R, Habermann F, Hrabé de Angelis M, Wolf E, Aigner B, und Wanke R
- Progressive kidney lesions in mutant mouse models for uromodulinassociated kidney disease. 96. Jahrestagung der Deutschen Gesellschaft für Pathologie, 31.05. -03.06.2012, Berlin. Der Pathologe, Band 33, Sonderheft 1, Mai 2012, Seite 84
Kemter E, Sklenak S, Prueckl P, Rathkolb R, Habermann F, Hrabé de Angelis M, Wolf E, Aigner B, and Wanke R
- No amelioration of uromodulin maturation and trafficking defect by sodium-4-phenylbutyrate in vivo: studies in a mouse model of uromodulin-associated kidney disease. 50th ERA-EDTA Congress, 18.-21.05.2013, Istanbul, Turkey
Kemter E, Sklenak S, Aigner B, und Wanke R
- Standardized, systemic phenotypic analysis of UmodC93F and UmodA227T mutant mice. PLOS One 8(10):e78337 (2013)
Kemter E, Prückl P, Rathkolb R, Micklich K, Adler T, Becker L, Beckers J, Busch DH, Götz AA, Hans W, Horsch M, Ivandic B, Klingenspor M, Klopstock T, Rozman J, Schrewe A, Schulz H, Fuchs H, Gailus-Durner V, Hrabé de Angelis M, Wolf E, Aigner B
(See online at https://doi.org/10.1371/journal.pone.0078337) - Type of uromodulin mutation and allelic status influence onset and severity of uromodulin-associated kidney disease in mice. Hum Mol Genet 22(20):4148-63 (2013)
Kemter E, Sklenak S, Prueckl P, Rathkolb R, Habermann FA, Hans W, Gailus-Durner V, Fuchs H, Hrabé de Angelis M, Wolf E, Aigner B, und Wanke R
(See online at https://doi.org/10.1093/hmg/ddt263) - No Amelioration of Uromodulin Maturation and Trafficking Defect by Sodium-4-Phenylbutyrate in vivo: Studies in Mouse Models of Uromodulin-associated Kidney Disease. J Biol Chem. 289, 10715-10726 (2014)
Kemter E, Sklenak S, Rathkolb R, Hrabě de Angelis M, Wolf E, Aigner B, and Wanke R
(See online at https://doi.org/10.1074/jbc.M113.537035) - Uromodulin 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 disease. PLoS One 9(11):e113125 (2014)
Horsch M, Beckers J, Fuchs H, Gailus-Durner V, Hrabě de Angelis M, Rathkolb R, Wolf E, Aigner B, Kemter E
(See online at https://doi.org/10.1371/journal.pone.0113125) - Durch ENU-Mutagenese generierte murine Nephropathiemodelle. Nova Acta Leopoldina NF 119, Nr. 402, 147-156 (2015)
Wanke R, Wolf E, Aigner B, und Kemter E
