Literature DB >> 17888939

Canine X-linked muscular dystrophy in a family of Grand Basset Griffon Vendéen dogs.

S Klarenbeek1, M J Gerritzen-Bruning2, A J M Rozemuller3, J J van der Lugt4.   

Abstract

Three related Grand Basset Griffon Vendéen (GBGV) dogs, two male and one female, with poor locomotion and muscle pain on palpation, were humanely destroyed at approximately 2 months of age and submitted for necropsy. Histopathological examination of skeletal muscles showed hyaline hypereosinophilic myofibres, hypertrophy and atrophy, calcification, necrosis, and mild proliferation of endomyseal connective tissue, as well as small basophilic fibres with internalized nuclei in rows, indicating regeneration. Immunohistochemical labelling for the carboxy-terminal domain of dystrophin, performed on skeletal muscle from one of the male dogs, was negative, whereas it was positive in skeletal muscle from a normal control dog. Both parents were clinically unaffected. These findings confirmed the diagnosis of canine X-linked muscular dystrophy (CXMD). To the authors' knowledge, this is the first report of CXMD in the GBGV breed, and one of very few cases in a female dog.

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Year:  2007        PMID: 17888939     DOI: 10.1016/j.jcpa.2007.06.007

Source DB:  PubMed          Journal:  J Comp Pathol        ISSN: 0021-9975            Impact factor:   1.311


  5 in total

Review 1.  Dystrophin-deficient large animal models: translational research and exon skipping.

Authors:  Xinran Yu; Bo Bao; Yusuke Echigoya; Toshifumi Yokota
Journal:  Am J Transl Res       Date:  2015-08-15       Impact factor: 4.060

2.  Duchenne muscular dystrophy gene therapy: Lost in translation?

Authors:  Dongsheng Duan
Journal:  Res Rep Biol       Date:  2011-03

Review 3.  Duchenne muscular dystrophy gene therapy in the canine model.

Authors:  Dongsheng Duan
Journal:  Hum Gene Ther Clin Dev       Date:  2015-02-24       Impact factor: 5.032

4.  An intronic LINE-1 element insertion in the dystrophin gene aborts dystrophin expression and results in Duchenne-like muscular dystrophy in the corgi breed.

Authors:  Bruce F Smith; Yongping Yue; Philip R Woods; Joe N Kornegay; Jin-Hong Shin; Regina R Williams; Dongsheng Duan
Journal:  Lab Invest       Date:  2010-08-16       Impact factor: 5.662

5.  X-linked muscular dystrophy in a Labrador Retriever strain: phenotypic and molecular characterisation.

Authors:  Inès Barthélémy; Nadège Calmels; Robert B Weiss; Laurent Tiret; Adeline Vulin; Nicolas Wein; Cécile Peccate; Carole Drougard; Christophe Beroud; Nathalie Deburgrave; Jean-Laurent Thibaud; Catherine Escriou; Isabel Punzón; Luis Garcia; Jean-Claude Kaplan; Kevin M Flanigan; France Leturcq; Stéphane Blot
Journal:  Skelet Muscle       Date:  2020-08-07       Impact factor: 4.912

  5 in total

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