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Treatment of keratinocytes with 4-phenylbutyrate in epidermolysis bullosa: Lessons for therapies in keratin disordersResearch in context

Background: Missense mutations in keratin 5 and 14 genes cause the severe skin fragility disorder epidermolysis bullosa simplex (EBS) by collapsing of the keratin cytoskeleton into cytoplasmic protein aggregates. Despite intense efforts, no molecular therapies are available, mostly due to the comple...

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主要な著者: Marina Spörrer, Ania Prochnicki, Regine C. Tölle, Alexander Nyström, Philipp R. Esser, Melanie Homberg, Ioannis Athanasiou, Eleni Zingkou, Achim Schilling, Richard Gerum, Ingo Thievessen, Lilli Winter, Leena Bruckner-Tuderman, Ben Fabry, Thomas M. Magin, Jörn Dengjel, Rolf Schröder, Dimitra Kiritsi
フォーマット: Artigo
言語:Inglês
出版事項: Elsevier 2019-06-01
シリーズ:EBioMedicine
オンライン・アクセス:http://www.sciencedirect.com/science/article/pii/S2352396419303019
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