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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Detalhes bibliográficos
Main Authors: Kemter, Elisabeth, Sklenak, Stefanie, Rathkolb, Birgit, Hrabě de Angelis, Martin, Wolf, Eckhard, Aigner, Bernhard, Wanke, Ruediger
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2014
Assuntos:
Molecular Bases of Disease
Acesso em linha: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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