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| Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue |
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| Author(s): Ortiz-Urda S, Lin Q, Green CL, Keene DR, Marinkovich MP, Khavari PA |
| Source: JOURNAL OF CLINICAL INVESTIGATION Volume: 111 Issue: 2 Pages: 251-255 Published: JAN 2003 |
| Times Cited: 45 References: 25 |
| Abstract: Current therapeutic strategies for genetic skin disorders rely on the complex process of grafting genetically engineered tissue to recipient wound beds. Because fibroblasts synthesize and secrete extracellular matrix, we explored their utility in recessive dystrophic epidermolysis bullosa (RDEB), a blistering disease due to defective extracellular type VII collagen. Intradermal injection of RDEB fibroblasts overexpressing type VII collagen into intact RDEB skin stably restored correctly localized type VII collagen expression in vivo and normalized hallmark RDEB disease features, including subepidermal blistering and anchoring fibril defects. Fibroblast injection thus provides a simplified approach to correcting human disorders of secreted matrix proteins. |
| Document Type: Article |
| Language: English |
| Reprint Address: Khavari, PA (reprint author), Stanford Univ, Sch Med, Program Epithelial Biol, 269 Campus Dr,Room 2145, Stanford, CA 94305 USA |
Addresses:
1. Stanford Univ, Sch Med, Program Epithelial Biol, Stanford, CA 94305 USA
2. Vet Affairs Palo Alto Healthcare Syst, Palo Alto, CA USA
3. Stanford Univ, Sch Med, Stanford, CA USA
4. Shriners Hosp Children, Portland, OR 97201 USA |
| Publisher: AMER SOC CLINICAL INVESTIGATION INC, 35 RESEARCH DR, STE 300, ANN ARBOR, MI 48103 USA |
| Subject Category: Medicine, Research & Experimental |
| IDS Number: 640DT |
| ISSN: 0021-9738 |
| DOI: 10.1172/JCI.200317193 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |