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Inherited Dystrophic Epidermolysis Bullosa in Inbred Dogs: A Spontaneous Animal Model for Somatic Gene Therapy  Xavier Palazzi, Thierry Marchal, Luc Chabanne,

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Presentation on theme: "Inherited Dystrophic Epidermolysis Bullosa in Inbred Dogs: A Spontaneous Animal Model for Somatic Gene Therapy  Xavier Palazzi, Thierry Marchal, Luc Chabanne,"— Presentation transcript:

1 Inherited Dystrophic Epidermolysis Bullosa in Inbred Dogs: A Spontaneous Animal Model for Somatic Gene Therapy  Xavier Palazzi, Thierry Marchal, Luc Chabanne, Anne Spadafora, Guerrino Meneguzzi  Journal of Investigative Dermatology  Volume 115, Issue 1, Pages (July 2000) DOI: /j x Copyright © 2000 The Society for Investigative Dermatology, Inc Terms and Conditions

2 Figure 1 Oral erosions in Golden Retriever dogs suffering from a mild form of dystrophic EB. The dystrophic EB dogs (individuals II-7 and II-8) are products of two distinct inbred unions within a strain of animals with a previous history of inherited skin blistering disease. Dog 1-4 had ancestors common to dogs I-3 and I-5 (A). Ulcerations and hemorragic blisters of the oral mucosa and lips in the affected female and the male dog (B and C, respectively). Histologic examination of the blistered skin detects a subepidermal cleavage (D). Journal of Investigative Dermatology  , DOI: ( /j x) Copyright © 2000 The Society for Investigative Dermatology, Inc Terms and Conditions

3 Figure 2 Altered expression of collagen type VII in canine dystrophic EB epithelia. Immunofluorescence analysis of frozen cryostat sections of epithelia specimens obtained from a healthy unrelated control (A, D, G), the dystrophic EB female (B, E, H) and male (C, F, I) dogs were stained with monoclonal antibody LH7:2 specific to collagen type VII. Compared with the control skin (A), non-lesional skin of the dystrophic EB female dog revealed a slightly reduced reactivity (B) while non-lesional skin of the diseased male was weakly immunoreactive (C). Reactivity of the oral and esophageal epithelia (E, F and H, I, respectively) appeared strongly reduced in the two affected animals. Labeling of the basement membrane was continuous at the roof of the blisters (F, H). Basilar, suprabasilar, and intradermal staining of collagen type VII was also observed. Blistered areas are indicated (arrows). Scale Bar: 20μm. Journal of Investigative Dermatology  , DOI: ( /j x) Copyright © 2000 The Society for Investigative Dermatology, Inc Terms and Conditions

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