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Article: Tissue-specific expression and long-term deposition of human collagen VII in the skin of transgenic mice: Implications for gene therapy

TitleTissue-specific expression and long-term deposition of human collagen VII in the skin of transgenic mice: Implications for gene therapy
Authors
Issue Date2000
PublisherNature Publishing Group. The Journal's web site is located at http://www.nature.com/gt
Citation
Gene Therapy, 2000, v. 7 n. 19, p. 1631-1639 How to Cite?
AbstractWe report the isolation of a cosmid clone containing the entire human COL7A1 gene in one piece. The ability of the genomic sequences within this clone to direct tissue-specific expression of human collagen VII in transgenic mice was tested. The data show that the gene construct is capable of directing expression of collagen VII in the skin of fetal and neonatal transgenic mice. Expression of COL7A1 in these mice was widespread, in a pattern consistent with that found in human tissues and was in parallel with that of the endogenous mouse gene. Immunostaining, using human-specific antibodies, showed that human collagen VII protein was present at the skin basement membrane zone of the transgenic mice. Dermal extracts from 19-month-old transgenic mice contained mature human collagen VII protein, and fibroblasts derived from skin biopsies of these mice actively synthesized human collagen VII. The demonstration of successful and stable expression of human collagen VII in in vivo gene transfer is the first step towards the future development of therapeutic protocols for the rescue of keratinocyte function in severe blistering diseases such as dystrophic epidermolysis bullosa.
Persistent Identifierhttp://hdl.handle.net/10722/147446
ISSN
2015 Impact Factor: 3.242
2015 SCImago Journal Rankings: 1.519
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorSat, Een_US
dc.contributor.authorLeung, KHen_US
dc.contributor.authorBrucknerTuderman, Len_US
dc.contributor.authorCheah, KSEen_US
dc.date.accessioned2012-05-29T06:03:46Z-
dc.date.available2012-05-29T06:03:46Z-
dc.date.issued2000en_US
dc.identifier.citationGene Therapy, 2000, v. 7 n. 19, p. 1631-1639en_US
dc.identifier.issn0969-7128en_US
dc.identifier.urihttp://hdl.handle.net/10722/147446-
dc.description.abstractWe report the isolation of a cosmid clone containing the entire human COL7A1 gene in one piece. The ability of the genomic sequences within this clone to direct tissue-specific expression of human collagen VII in transgenic mice was tested. The data show that the gene construct is capable of directing expression of collagen VII in the skin of fetal and neonatal transgenic mice. Expression of COL7A1 in these mice was widespread, in a pattern consistent with that found in human tissues and was in parallel with that of the endogenous mouse gene. Immunostaining, using human-specific antibodies, showed that human collagen VII protein was present at the skin basement membrane zone of the transgenic mice. Dermal extracts from 19-month-old transgenic mice contained mature human collagen VII protein, and fibroblasts derived from skin biopsies of these mice actively synthesized human collagen VII. The demonstration of successful and stable expression of human collagen VII in in vivo gene transfer is the first step towards the future development of therapeutic protocols for the rescue of keratinocyte function in severe blistering diseases such as dystrophic epidermolysis bullosa.en_US
dc.languageengen_US
dc.publisherNature Publishing Group. The Journal's web site is located at http://www.nature.com/gten_US
dc.relation.ispartofGene Therapyen_US
dc.subject.meshAnimalsen_US
dc.subject.meshBlotting, Southernen_US
dc.subject.meshCollagen - Analysis - Biosynthesis - Geneticsen_US
dc.subject.meshEpidermolysis Bullosa Dystrophica - Therapyen_US
dc.subject.meshFibroblasts - Metabolismen_US
dc.subject.meshGene Expressionen_US
dc.subject.meshGene Therapy - Methodsen_US
dc.subject.meshGene Transfer Techniquesen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunohistochemistryen_US
dc.subject.meshMiceen_US
dc.subject.meshMice, Transgenicen_US
dc.subject.meshRestriction Mappingen_US
dc.subject.meshReverse Transcriptase Polymerase Chain Reactionen_US
dc.subject.meshSkin - Chemistry - Metabolismen_US
dc.subject.meshTime Factorsen_US
dc.titleTissue-specific expression and long-term deposition of human collagen VII in the skin of transgenic mice: Implications for gene therapyen_US
dc.typeArticleen_US
dc.identifier.emailLeung, KH:keithlee@hku.hken_US
dc.identifier.emailCheah, KSE:hrmbdkc@hku.hken_US
dc.identifier.authorityLeung, KH=rp00298en_US
dc.identifier.authorityCheah, KSE=rp00342en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid11083471-
dc.identifier.scopuseid_2-s2.0-0033759279en_US
dc.identifier.hkuros114934-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0033759279&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume7en_US
dc.identifier.issue19en_US
dc.identifier.spage1631en_US
dc.identifier.epage1639en_US
dc.identifier.isiWOS:000089770200003-
dc.publisher.placeUnited Kingdomen_US
dc.identifier.scopusauthoridSat, E=6506589776en_US
dc.identifier.scopusauthoridLeung, KH=7401860467en_US
dc.identifier.scopusauthoridBrucknerTuderman, L=7006538168en_US
dc.identifier.scopusauthoridCheah, KSE=35387746200en_US

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