Article
Gentamicin induces functional type VII collagen in recessive dystrophic epidermolysis bullosa patients.
The Journal of clinical investigation - 1 Aug 2017
Woodley David T, Cogan Jon, Hou Yingping, Lyu Chao, Marinkovich M Peter, Keene Douglas, Chen Mei
Abstract excerpt
BACKGROUND: Recessive dystrophic epidermolysis bullosa (RDEB) is an incurable disease caused by mutations in the gene encoding type VII collagen, the major component of anchoring fibrils (AF). We previously demonstrated that gentamicin produced functional type VII collagen in RDEB cells harboring nonsense mutations. Herein, we determined whether topical or intradermal gentamicin administration induces type VII...
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