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Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa
Author(s): Woodley DT, Keene DR, Atha T, Huang Y, Lipman K, Li W, Chen M
Source: NATURE MEDICINE    Volume: 10    Issue: 7    Pages: 693-695    Published: JUL 2004  
Times Cited: 21     References: 15     
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.
Document Type: Article
Language: English
Reprint Address: Chen, M (reprint author), Univ So Calif, Div Dermatol, Dept Med, CRL 204,1303 Mission Rd, Los Angeles, CA 90033 USA
Addresses:
1. Univ So Calif, Div Dermatol, Dept Med, Los Angeles, CA 90033 USA
2. Shriners Hosp Children, Portland, OR 97201 USA
Publisher: NATURE PUBLISHING GROUP, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 USA
Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental
IDS Number: 835DM
ISSN: 1078-8956
DOI: 10.1038/nm1063
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