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

被引:7
作者
Klarenbeek, S. [1 ]
Gerritzen-Bruning, M. J.
Rozemuller, A. J. M.
van der Lugt, J. J.
机构
[1] Univ Utrecht, Fac Vet Med, Dept Pathobiol, Utrecht, Netherlands
[2] Univ Utrecht, Fac Vet Med, Dept Clin Sci Compan Anim, Utrecht, Netherlands
[3] Acad Med Ctr, Dept Neuropathol, Amsterdam, Netherlands
关键词
dog; muscular dystrophy;
D O I
10.1016/j.jcpa.2007.06.007
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Three related Grand Basset Griffon Vendeen (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. (C) 2007 Elsevier Ltd. All rights reserved.
引用
收藏
页码:249 / 252
页数:4
相关论文
共 17 条
[1]   Duchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies [J].
Collins, CA ;
Morgan, JE .
INTERNATIONAL JOURNAL OF EXPERIMENTAL PATHOLOGY, 2003, 84 (04) :165-172
[2]   The muscular dystrophies [J].
Emery, AEH .
LANCET, 2002, 359 (9307) :687-695
[3]   EXPRESSION OF DELETION-CONTAINING DYSTROPHINS IN MDX MUSCLE - IMPLICATIONS FOR GENE-THERAPY AND DYSTROPHIN FUNCTION [J].
FRITZ, JD ;
DANKO, I ;
ROBERDS, SL ;
CAMPBELL, KP ;
LATENDRESSE, JS ;
WOLFF, JA .
PEDIATRIC RESEARCH, 1995, 37 (06) :693-700
[4]   DYSTROPHIN - THE PROTEIN PRODUCT OF THE DUCHENNE MUSCULAR-DYSTROPHY LOCUS [J].
HOFFMAN, EP ;
BROWN, RH ;
KUNKEL, LM .
CELL, 1987, 51 (06) :919-928
[5]   IS THE CARBOXYL-TERMINUS OF DYSTROPHIN REQUIRED FOR MEMBRANE ASSOCIATION - A NOVEL, SEVERE CASE OF DUCHENNE MUSCULAR-DYSTROPHY [J].
HOFFMAN, EP ;
GARCIA, CA ;
CHAMBERLAIN, JS ;
ANGELINI, C ;
LUPSKI, JR ;
FENWICK, R .
ANNALS OF NEUROLOGY, 1991, 30 (04) :605-610
[6]   Molecular pathophysiology and targeted therapeutics for muscular dystrophy [J].
Hoffman, EP ;
Dressman, D .
TRENDS IN PHARMACOLOGICAL SCIENCES, 2001, 22 (09) :465-470
[7]   Muscular dystrophy with truncated dystrophin in a family of Japanese Spitz dogs [J].
Jones, BR ;
Brennan, S ;
Mooney, CT ;
Callanan, JJ ;
McAllister, H ;
Guo, LT ;
Martin, PT ;
Engvall, E ;
Shelton, GD .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 2004, 217 (02) :143-149
[8]   MUSCULAR-DYSTROPHY IN A LITTER OF GOLDEN RETRIEVER DOGS [J].
KORNEGAY, JN ;
TULER, SM ;
MILLER, DM ;
LEVESQUE, DC .
MUSCLE & NERVE, 1988, 11 (10) :1056-1064
[9]   Adeno-associated virus-mediated microdystrophin expression protects young mdx muscle from contraction-induced injury [J].
Liu, MJ ;
Yue, YP ;
Harper, SQ ;
Grange, RW ;
Chamberlain, JS ;
Duan, DS .
MOLECULAR THERAPY, 2005, 11 (02) :245-256
[10]   Dystrophin-glycoprotein complex: Post-translational processing and dystroglycan function [J].
Michele, DE ;
Campbell, KP .
JOURNAL OF BIOLOGICAL CHEMISTRY, 2003, 278 (18) :15457-15460