Document Detail


Fibroblast-based cell therapy strategy for recessive dystrophic epidermolysis bullosa.
MedLine Citation:
PMID:  20447505     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in the COL7A1 gene which result in reduced, truncated, or absent type VII collagen, and anchoring fibrils at the dermal-epidermal junction (DEJ). Because no topical wound-healing agents have shown unequivocal benefit in the treatment of DEB, alternative approaches are needed. The purpose of cell therapy for recessive DEB is to increase the amount of collagen VII in the basement membrane zone in order to heal wounds and prevent further wound formation. Fibroblast-based cell therapy is safe and easy to work with, has few side effects, can dramatically restore stable collagen VII at the DEJ, and can normalize the substructure changes of DEB for at least a few months. Even though the mechanism and the duration of newly produced collagen VII at the DEJ are still unknown, this form of cell therapy provides a new effective approach to the treatment of recessive DEB.
Authors:
W F Yan; Dédée F Murrell
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Dermatologic clinics     Volume:  28     ISSN:  1558-0520     ISO Abbreviation:  Dermatol Clin     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-05-07     Completed Date:  2010-08-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8300886     Medline TA:  Dermatol Clin     Country:  United States    
Other Details:
Languages:  eng     Pagination:  367-70, xii     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Department of Dermatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia.
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MeSH Terms
Descriptor/Qualifier:
Animals
Cell Transplantation / methods*,  trends*
Epidermolysis Bullosa Dystrophica / therapy*
Fibroblasts / transplantation*
Humans

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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