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

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...

詳細記述

保存先:
書誌詳細
出版年:EBioMedicine
主要な著者: Spörrer, Marina, Prochnicki, Ania, Tölle, Regine C., Nyström, Alexander, Esser, Philipp R., Homberg, Melanie, Athanasiou, Ioannis, Zingkou, Eleni, Schilling, Achim, Gerum, Richard, Thievessen, Ingo, Winter, Lilli, Bruckner-Tuderman, Leena, Fabry, Ben, Magin, Thomas M., Dengjel, Jörn, Schröder, Rolf, Kiritsi, Dimitra
フォーマット: Artigo
言語:Inglês
出版事項: Elsevier 2019
主題:
Research paper
オンライン･アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC6603805/
https://ncbi.nlm.nih.gov/pubmed/31078522
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ebiom.2019.04.062
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