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

INTRODUCTION: 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 cultu...

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Detalles Bibliográficos
Autores principales: Wang, Dongmao, Mohammad, Mardhiah, Wang, Yanyan, Tan, Rachel, Murray, Lydia S., Ricardo, Sharon, Dagher, Hayat, van Agtmael, Tom, Savige, Judy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2017
Materias:
Translational Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5678609/
https://www.ncbi.nlm.nih.gov/pubmed/29142990
http://dx.doi.org/10.1016/j.ekir.2017.03.004

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5678609/
https://www.ncbi.nlm.nih.gov/pubmed/29142990
http://dx.doi.org/10.1016/j.ekir.2017.03.004

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