Rat Model for Dominant Dystrophic Epidermolysis Bullosa: Glycine Substitution Reduces Collagen VII Stability and Shows Gene-Dosage Effect

Dystrophic epidermolysis bullosa, a severely disabling hereditary skin fragility disorder, is caused by mutations in the gene coding for collagen VII, a specialized adhesion component of the dermal-epidermal junction zone. Both recessive and dominant forms are known; the latter account for about 40%...

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Detalles Bibliográficos
Autores principales: Nyström, Alexander, Buttgereit, Jens, Bader, Michael, Shmidt, Tatiana, Özcelik, Cemil, Hausser, Ingrid, Bruckner-Tuderman, Leena, Kern, Johannes S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3662756/
https://www.ncbi.nlm.nih.gov/pubmed/23717576
http://dx.doi.org/10.1371/journal.pone.0064243

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