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. 1989 Oct;135(4):671–678.

Development of Duchenne-type cardiomyopathy. Morphologic studies in a canine model.

B A Valentine 1, J F Cummings 1, B J Cooper 1
PMCID: PMC1880020  PMID: 2679113

Abstract

The development of cardiac lesions was studied in xmd dogs aged from 1 day to 6 years. Cardiac lesions were not present in dystrophic dogs up to 3 months of age. Foci of mineralization were first seen at 6.5 months. A 1-year-old dog had foci of myocyte hypercontraction. Linear and anastomosing fibrosis was present in all dogs 1 year of age or older, most prominently and most consistently within the subepicardial region of the left ventricular (LV) free wall, the LV papillary muscles, and the right ventricular (RV) aspect of the septum. Ultrastructurally, endomysial fibrosis, decreased myofibrillar density, and prominence of mitochondria were consistent features. Severe degenerative changes were present in two dogs and included prominent intracytoplasmic myelin figures, lipid droplets, and lipofuscin. Immunocytochemical studies of an affected dog confirmed the absence of dystrophin in LV myocardium. Characteristic late-onset cardiac lesions, similar to those occurring in Duchenne dystrophy, are a consistent feature of canine X-linked muscular dystrophy (CXMD).

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Arahata K., Ishiura S., Ishiguro T., Tsukahara T., Suhara Y., Eguchi C., Ishihara T., Nonaka I., Ozawa E., Sugita H. Immunostaining of skeletal and cardiac muscle surface membrane with antibody against Duchenne muscular dystrophy peptide. Nature. 1988 Jun 30;333(6176):861–863. doi: 10.1038/333861a0. [DOI] [PubMed] [Google Scholar]
  2. Bridges L. R. The association of cardiac muscle necrosis and inflammation with the degenerative and persistent myopathy of MDX mice. J Neurol Sci. 1986 Feb;72(2-3):147–157. doi: 10.1016/0022-510x(86)90003-1. [DOI] [PubMed] [Google Scholar]
  3. Campbell K. P., Kahl S. D. Association of dystrophin and an integral membrane glycoprotein. Nature. 1989 Mar 16;338(6212):259–262. doi: 10.1038/338259a0. [DOI] [PubMed] [Google Scholar]
  4. Cardinet G. H., 3rd, Holliday T. A. Neuromuscular diseases of domestic animals: a summary of muscle biopsies from 159 cases. Ann N Y Acad Sci. 1979;317:290–313. [PubMed] [Google Scholar]
  5. Carnwath J. W., Shotton D. M. Muscular dystrophy in the mdx mouse: histopathology of the soleus and extensor digitorum longus muscles. J Neurol Sci. 1987 Aug;80(1):39–54. doi: 10.1016/0022-510x(87)90219-x. [DOI] [PubMed] [Google Scholar]
  6. Cooper B. J., Valentine B. A., Wilson S., Patterson D. F., Concannon P. W. Canine muscular dystrophy: confirmation of X-linked inheritance. J Hered. 1988 Nov-Dec;79(6):405–408. doi: 10.1093/oxfordjournals.jhered.a110543. [DOI] [PubMed] [Google Scholar]
  7. Cooper B. J., Winand N. J., Stedman H., Valentine B. A., Hoffman E. P., Kunkel L. M., Scott M. O., Fischbeck K. H., Kornegay J. N., Avery R. J. The homologue of the Duchenne locus is defective in X-linked muscular dystrophy of dogs. Nature. 1988 Jul 14;334(6178):154–156. doi: 10.1038/334154a0. [DOI] [PubMed] [Google Scholar]
  8. Cullen M. J., Mastaglia F. L. Morphological changes in dystrophic muscle. Br Med Bull. 1980 May;36(2):145–122. doi: 10.1093/oxfordjournals.bmb.a071630. [DOI] [PubMed] [Google Scholar]
  9. Frankel K. A., Rosser R. J. The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis. Hum Pathol. 1976 Jul;7(4):375–386. doi: 10.1016/s0046-8177(76)80053-6. [DOI] [PubMed] [Google Scholar]
  10. Hoffman E. P., Brown R. H., Jr, Kunkel L. M. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell. 1987 Dec 24;51(6):919–928. doi: 10.1016/0092-8674(87)90579-4. [DOI] [PubMed] [Google Scholar]
  11. Hoffman E. P., Hudecki M. S., Rosenberg P. A., Pollina C. M., Kunkel L. M. Cell and fiber-type distribution of dystrophin. Neuron. 1988 Jul;1(5):411–420. doi: 10.1016/0896-6273(88)90191-2. [DOI] [PubMed] [Google Scholar]
  12. Hunsaker R. H., Fulkerson P. K., Barry F. J., Lewis R. P., Leier C. V., Unverferth D. V. Cardiac function in Duchenne's muscular dystrophy. Results of 10-year follow-up study and noninvasive tests. Am J Med. 1982 Aug;73(2):235–238. doi: 10.1016/0002-9343(82)90184-x. [DOI] [PubMed] [Google Scholar]
  13. Hunter S. The heart in muscular dystrophy. Br Med Bull. 1980 May;36(2):133–134. doi: 10.1093/oxfordjournals.bmb.a071627. [DOI] [PubMed] [Google Scholar]
  14. JAMES T. N. Observations on the cardiovascular involvement, including the cardiac conduction system, in progressive muscular dystrophy. Am Heart J. 1962 Jan;63:48–56. doi: 10.1016/0002-8703(62)90219-3. [DOI] [PubMed] [Google Scholar]
  15. Koenig M., Monaco A. P., Kunkel L. M. The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell. 1988 Apr 22;53(2):219–228. doi: 10.1016/0092-8674(88)90383-2. [DOI] [PubMed] [Google Scholar]
  16. MEIER H. Myopathies in the dog. Cornell Vet. 1958 Jul;48(3):313–330. [PubMed] [Google Scholar]
  17. Moser H. Duchenne muscular dystrophy: pathogenetic aspects and genetic prevention. Hum Genet. 1984;66(1):17–40. doi: 10.1007/BF00275183. [DOI] [PubMed] [Google Scholar]
  18. Mukoyama M., Kondo K., Hizawa K., Nishitani H. Life spans of Duchenne muscular dystrophy patients in the hospital care program in Japan. J Neurol Sci. 1987 Nov;81(2-3):155–158. doi: 10.1016/0022-510x(87)90092-x. [DOI] [PubMed] [Google Scholar]
  19. Nigro G., Comi L. I., Limongelli F. M., Giugliano M. A., Politano L., Petretta V., Passamano L., Stefanelli S. Prospective study of X-linked progressive muscular dystrophy in Campania. Muscle Nerve. 1983 May;6(4):253–262. doi: 10.1002/mus.880060403. [DOI] [PubMed] [Google Scholar]
  20. Perloff J. K., Roberts W. C., de Leon A. C., Jr, O'Doherty D. The distinctive electrocardiogram of Duchenne's progressive muscular dystrophy. An electrocardiographic-pathologic correlative study. Am J Med. 1967 Feb;42(2):179–188. doi: 10.1016/0002-9343(67)90017-4. [DOI] [PubMed] [Google Scholar]
  21. Perloff J. K., de Leon A. C., Jr, O'Doherty D. The cardiomyopathy of progressive muscular dystrophy. Circulation. 1966 Apr;33(4):625–648. doi: 10.1161/01.cir.33.4.625. [DOI] [PubMed] [Google Scholar]
  22. Sanyal S. K., Johnson W. W., Dische M. R., Pitner S. E., Beard C. Dystrophic degeneration of papillary muscle and ventricular myocardium. A basis for mitral valve prolapse in Duchenne's muscular dystrophy. Circulation. 1980 Aug;62(2):430–438. doi: 10.1161/01.cir.62.2.430. [DOI] [PubMed] [Google Scholar]
  23. Sanyal S. K., Johnson W. W., Thapar M. K., Pitner S. E. An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy. Circulation. 1978 Jun;57(6):1122–1129. doi: 10.1161/01.cir.57.6.1122. [DOI] [PubMed] [Google Scholar]
  24. Tanaka H., Nishi S., Katanasako H. Natural course of cardiomyopathy in Duchenne muscular dystrophy. Jpn Circ J. 1979 Nov;43(11):974–984. doi: 10.1253/jcj.43.974. [DOI] [PubMed] [Google Scholar]
  25. Torres L. F., Duchen L. W. The mutant mdx: inherited myopathy in the mouse. Morphological studies of nerves, muscles and end-plates. Brain. 1987 Apr;110(Pt 2):269–299. doi: 10.1093/brain/110.2.269. [DOI] [PubMed] [Google Scholar]
  26. Valentine B. A., Cooper B. J., de Lahunta A., O'Quinn R., Blue J. T. Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: clinical studies. J Neurol Sci. 1988 Dec;88(1-3):69–81. doi: 10.1016/0022-510x(88)90206-7. [DOI] [PubMed] [Google Scholar]
  27. Wakai S., Minami R., Kameda K., Okabe M., Nagaoka M., Annaka S., Higashidate Y., Tomita H., Tachi N. Electron microscopic study of the biopsied cardiac muscle in Duchenne muscular dystrophy. J Neurol Sci. 1988 Apr;84(2-3):167–175. doi: 10.1016/0022-510x(88)90122-0. [DOI] [PubMed] [Google Scholar]

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