title: Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa.
abstract: Dystrophic epidermolysis bullosa (DEB) is a family of inherited mechano-bullous disorders that are caused by mutations in the type VII collagen gene and for which ex vivo gene therapy has been considered. To develop a simpler approach for treating DEB, we evaluated the feasibility of protein-based therapy by intradermally injecting human recombinant type VII collagen into mouse skin and a DEB human skin equivalent transplanted onto mice. The injected collagen localized to the basement membrane zone of both types of tissues, was organized into human anchoring fibril structures and reversed the features of DEB disease in the DEB skin equivalent.
authors.affiliation: Department of Medicine, Division of Dermatology, University of Southern California, CRL 204, 1303 Mission Road, Los Angeles, California 90033, USA.
authors.forename: David T
authors.initials: DT
authors.lastname: Woodley
authors.forename: Douglas R
authors.initials: DR
authors.lastname: Keene
authors.forename: Tom
authors.initials: T
authors.lastname: Atha
authors.forename: Yi
authors.initials: Y
authors.lastname: Huang
authors.forename: Kathi
authors.initials: K
authors.lastname: Lipman
authors.forename: Wei
authors.initials: W
authors.lastname: Li
authors.forename: Mei
authors.initials: M
authors.lastname: Chen
mesh_terms: D000818:Animals; D024143:Collagen Type VII / Q000008:administration & dosage* / Q000235:genetics / Q000502:physiology; D016108:Epidermolysis Bullosa Dystrophica / Q000188:drug therapy*; D006801:Humans; D051379:Mice; D008812:Mice, Hairless; D011994:Recombinant Proteins / Q000008:administration & dosage
chemical_list: D024143:Collagen Type VII; D011994:Recombinant Proteins