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Article

Correction of Recessive Dystrophic Epidermolysis Bullosa by Transposon-Mediated Integration of COL7A1 in Transplantable Patient-Derived Primary Keratinocytes.

The Journal of investigative dermatology - 1 Apr 2017

Latella Maria Carmela, Cocchiarella Fabienne, De Rosa Laura, Turchiano Giandomenico, Gonçalves Manuel A F V, Larcher Fernando, De Luca Michele, Recchia Alessandra

Abstract excerpt

Recessive dystrophic epidermolysis bullosa (RDEB) is caused by defects in type-VII collagen (C7), a protein encoded by the COL7A1 gene and essential for anchoring fibril formation at the dermal-epidermal junction. Gene therapy of RDEB is based on transplantation of autologous epidermal grafts generated from gene-corrected keratinocytes sustaining C7 deposition at the dermal-epidermal junction. Transfer of the...

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Share to Collagen Type VII
Share to Epidermolysis Bullosa Dystrophica

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Fluorescent Antibody Technique

Share to Genetic Therapy

Share to Keratinocytes

Share to Mice, Mutant Strains

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Share to Transplantation, Heterologous

Source

Identifiers

PMID: 28027893
DOI: 10.1016/j.jid.2016.11.038

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Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Correction of Recessive Dystrophic Epidermolysis Bullosa by Transposon-Mediated Integration of COL7A1 in Transplantable Patient-Derived Primary Keratinocytes.PMID 28027893Read ↗

Gene Therapy for Dystrophic Epidermolysis Bullosa.Semantic proximity Read ↗

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Gene editing toward the use of autologous therapies in recessive dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Correction of dog dystrophic epidermolysis bullosa by transplantation of genetically modified epidermal autografts.Semantic proximity Read ↗

Deletion of a Pathogenic Mutation-Containing Exon of COL7A1 Allows Clonal Gene Editing Correction of RDEB Patient Epidermal Stem CellsSemantic proximity Read ↗

Lentiviral Engineered Fibroblasts Expressing Codon-Optimized COL7A1 Restore Anchoring Fibrils in RDEBSemantic proximity Read ↗

Targeted Exon Skipping Restores Type VII Collagen Expression and Anchoring Fibril Formation in an In Vivo RDEB Model.Semantic proximity Read ↗

Functional correction of type VII collagen expression in dystrophic epidermolysis bullosa.Semantic proximity Read ↗

Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis BullosaSemantic proximity Read ↗

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