The Chemical Chaperone, PBA, Reduces ER Stress and Autophagy and Increases Collagen IV α5 Expression in Cultured Fibroblasts From Men With X-Linked Alport Syndrome and Missense Mutations

X-linked Alport syndrome (OMIM 301050) is caused by COL4A5 missense variants in 40% of families. This study examined the effects of chemical chaperone treatment (sodium 4-phenylbutyrate) on fibroblast cell lines derived from men with missense mutations. Methods: Dermal fibroblast cultures were estab...

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Principais autores:	Dongmao Wang, Mardhiah Mohammad, Yanyan Wang, Rachel Tan, Lydia S. Murray, Sharon Ricardo, Hayat Dagher, Tom van Agtmael, Judy Savige
Formato:	Artigo
Idioma:	Inglês
Publicado:	Elsevier 2017-07-01
Series:	Kidney International Reports
Assuntos:	autophagy chaperones ER stress missense mutations unfolded protein response X-linked Alport syndrome
Acceso en liña:	http://www.sciencedirect.com/science/article/pii/S246802491730058X
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