title: Canine X-linked muscular dystrophy in a family of Grand Basset Griffon Vendéen dogs.
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.
authors: Departments of Pathobiology. Electronic address: s.klarenbeek@vet.uu.nl.
authors: S
authors: S
authors: Klarenbeek
authors: Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Netherlands.
authors: M J
authors: MJ
authors: Gerritzen-Bruning
authors: Department of Neuropathology, Academic Medical Center, Amsterdam, Netherlands.
authors: A J M
authors: AJM
authors: Rozemuller
authors: Departments of Pathobiology.
authors: J J
authors: JJ
authors: van der Lugt
mesh_terms: D000818:Animals; D004283:Dog Diseases / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D004285:Dogs; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics; D005260:Female; D008040:Genetic Linkage; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009137:Muscular Dystrophy, Animal / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D014960:X Chromosome
chemical_list: D016189:Dystrophin