ArticleGene therapy1999
BP180 gene delivery in junctional epidermolysis bullosa.
Article in Gene therapy, 1999. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
13 citing papers in PubMed, 77 citations in OpenAlex.
- Ideal Living Skin Equivalents, From Old Technologies and Models to Advanced Ones: The Prospects for an Integrated Approach.BioMed research international · 2024Review
- Future Perspectives of Oral Delivery of Next Generation Therapies for Treatment of Skin Diseases.Pharmaceutics · 2021Review
- Collagen XVII (BP180) modulates keratinocyte expression of the proinflammatory chemokine, IL-8.Experimental dermatology · 2012Article
- Type XVII collagen (BP180) can function as a cell-matrix adhesion molecule via binding to laminin 332.Matrix biology : journal of the International Society for Matrix Biology · 2011Article
- Long-term type VII collagen restoration to human epidermolysis bullosa skin tissue.Human gene therapy · 2010Article
- Bone marrow transplantation restores epidermal basement membrane protein expression and rescues epidermolysis bullosa model mice.Proceedings of the National Academy of Sciences of the United States of America · 2010Article
- Gene transfer in human skin with different pseudotyped HIV-based vectors.Gene therapy · 2007Article
- Elevated expression and release of tissue-type, but not urokinase-type, plasminogen activator after binding of autoantibodies to bullous pemphigoid antigen 180 in cultured human keratinocytes.Clinical and experimental immunology · 2004Article
- Sustainable correction of junctional epidermolysis bullosa via transposon-mediated nonviral gene transfer.Gene therapy · 2003Article
- In vivo restoration of laminin 5 beta 3 expression and function in junctional epidermolysis bullosa.Proceedings of the National Academy of Sciences of the United States of America · 2001Article
- Tissue-specific expression and long-term deposition of human collagen VII in the skin of transgenic mice: implications for gene therapy.Gene therapy · 2000Article
- Stable integration of large (>100 kb) PAC constructs in HaCaT keratinocytes using an integrin-targeting peptide delivery system.Gene therapy · 2000Article
- Gene Therapy and its Application in Dermatology.Indian journal of dermatologyReview
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
Abstract
Epidermolysis bullosa (EB) comprises a family of inherited blistering skin diseases for which current therapy is only palliative. Junctional EB (JEB) involves dissociation of the dermal-epidermal junction and results from mutations in a number of genes that encode vital structural proteins, including BP180 (type XVII collagen/BPAG2). In order to develop a model of corrective gene delivery for JEB, we produced a retroviral expression vector for wild-type human BP180 and used it to restore BP180 protein expression to primary keratinocytes from BP180-negative patients with generalized atrophic JEB. Restoration of full-length BP180 protein expression was associated with adhesion parameter normalization of primary JEB keratinocytes in vitro. These cells were then used to regenerate human skin on immune-deficient mice. BP180 gene-transduced tissue demonstrated restoration of BP180 gene expression at the dermal-epidermal junction in vivo while untransduced regenerated JEB skin entirely lacked BP180 expression. These findings provide a basis for future efforts to achieve gene delivery in human EB skin tissue.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.