The chemical chaperone, PBA, reduces ER stress and autophagy and increases collagen IV a5 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 cult...
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Main Authors: | , , , , , , , , |
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格式: | Article |
语言: | English |
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Elsevier
2017
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在线阅读: | http://irep.iium.edu.my/62219/1/mardhiah2017.pdf http://irep.iium.edu.my/62219/ http://www.kireports.org/article/S2468-0249(17)30058-X/pdf http://dx.doi.org/10.1016/j.ekir.2017.03.004 |
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