Literature DB >> 9349347

Non-lethal junctional epidermolysis bullosa in a dog.

M Nagata1, T Iwasaki, H Masuda, H Shimizu.   

Abstract

We report a 4-year-old female mongrel dog with a history since birth of erosions and atrophic skin, with pigmentation and alopecia on the face, trunk and extremities, together with dystrophic nails. Light microscopy revealed subepidermal blisters with minimal inflammation and electron microscopy confirmed that the ultrastructural site of separation site was at the lamina lucida. Indirect immunofluorescence of the dog's skin detected the positive expression of laminin 5, BPAG2, integrin-alpha 6 and type VII collagen. These clinical, ultrastructural and immunohistochemical features suggested that the dog had a non-lethal subtype of junctional epidermolysis bullosa (JEB). This is the first confirmed case of non-lethal JEB in a dog and presents a possible candidate for an animal model of gene therapy. Further study should provide important information of the phenotype, pathophysiology and prognosis of non-lethal JEB in humans.

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Mesh:

Year:  1997        PMID: 9349347

Source DB:  PubMed          Journal:  Br J Dermatol        ISSN: 0007-0963            Impact factor:   9.302


  3 in total

1.  A mouse model of generalized non-Herlitz junctional epidermolysis bullosa.

Authors:  Jason A Bubier; Thomas J Sproule; Lydia M Alley; Cameron M Webb; Jo-David Fine; Derry C Roopenian; John P Sundberg
Journal:  J Invest Dermatol       Date:  2010-03-25       Impact factor: 8.551

2.  A frameshift mutation within LAMC2 is responsible for Herlitz type junctional epidermolysis bullosa (HJEB) in black headed mutton sheep.

Authors:  Stefanie Mömke; Andrea Kerkmann; Anne Wöhlke; Miriam Ostmeier; Marion Hewicker-Trautwein; Martin Ganter; James Kijas; Ottmar Distl
Journal:  PLoS One       Date:  2011-05-04       Impact factor: 3.240

3.  A COL7A1 Variant in a Litter of Neonatal Basset Hounds with Dystrophic Epidermolysis Bullosa.

Authors:  Teresa Maria Garcia; Sarah Kiener; Vidhya Jagannathan; Duncan S Russell; Tosso Leeb
Journal:  Genes (Basel)       Date:  2020-12-04       Impact factor: 4.096

  3 in total

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