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...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Research paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6603805/
https://www.ncbi.nlm.nih.gov/pubmed/31078522
http://dx.doi.org/10.1016/j.ebiom.2019.04.062

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