No Amelioration of Uromodulin Maturation and Trafficking Defect by Sodium 4-Phenylbutyrate in Vivo: STUDIES IN MOUSE MODELS OF UROMODULIN-ASSOCIATED KIDNEY DISEASE

Uromodulin (UMOD)-associated kidney disease (UAKD) belongs to the hereditary progressive ER storage diseases caused by maturation defects of mutant UMOD protein. Current treatments of UAKD patients are symptomatic and cannot prevent disease progression. Two in vitro studies reported a positive effec...

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Hlavní autoři: Kemter, Elisabeth, Sklenak, Stefanie, Rathkolb, Birgit, Hrabě de Angelis, Martin, Wolf, Eckhard, Aigner, Bernhard, Wanke, Ruediger
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Biochemistry and Molecular Biology 2014
Témata:
Molecular Bases of Disease
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC4036188/
https://ncbi.nlm.nih.gov/pubmed/24567330
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M113.537035
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