Advertisement

Golden Retriever Muscular Dystrophy (GRMD): Developing and Maintaining a Colony and Physiological Functional Measurements

Protocol
First Online:
Part of the Methods in Molecular Biology book series (MIMB, volume 709)

Abstract

Studies of canine models of Duchenne muscular dystrophy (DMD) provide insight regarding disease pathogenesis and treatment efficacy. To take maximal advantage, colonies of affected dogs must be maintained and outcome parameters developed. In this chapter, we review our 25 years of experience with the golden retriever muscular dystrophy (GRMD) model. Key challenges in colony development (breeding, neonatal death, and the risk of inbreeding) and representative functional measurements (tibiotarsal joint angle and torque force; and eccentric contraction decrement) are discussed.

Key words

Duchenne muscular dystrophy Golden retriever muscular dystrophy Neonatal care Inbreeding Joint angle Torque force 
This is a preview of subscription content, log in to check access.

References

  1. 1.
    Bulfield, G., Siller, W.G., Wight, P. A. L., and Moore, K. J. (1984) X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc Natl Acad Sci USA 81, 1189–1192.PubMedCrossRefGoogle Scholar
  2. 2.
    Gillis, J. M. (1999) Understanding dystrophinopathies: an inventory of the structural and functional consequences of the absence of dystrophin in muscles of the mdx mouse. J Muscle Res Cell Motil 20, 605–625.PubMedCrossRefGoogle Scholar
  3. 3.
    Kornegay, J. N., Tuler, S. M., Miller, D. M., and Levesque, D. C. (1988) Muscular dystrophy in a litter of golden retriever dogs. Muscle Nerve 11, 1056–1064.PubMedCrossRefGoogle Scholar
  4. 4.
    Cooper, B. J., Winand, W. N., Stedman, H., Valentine B. A., Hoffman, E. P., Kunkel, L. M., et al. (1988) The homologue of the Duchenne locus is defective in X-linked muscular dystrophy of dogs. Nature 334, 154–156.PubMedCrossRefGoogle Scholar
  5. 5.
    Schatzberg, S. J., Olby, N. J., Breen, M., Anderson, L. V. B., Langford, C. F., Dickens H. F., et al. (1999) Molecular analysis of a spontaneous dystrophin “knockout” dog. Neuromuscul Disord 9, 289–295.PubMedCrossRefGoogle Scholar
  6. 6.
    Jones, B. R., Brennan, S., Mooney, C. T., Callanan, J. J., McAllister, H., Guo, L. T. et al. (2004) Muscular dystrophy with truncated dystrophin in a family of Japanese Spitz dogs. J Neurol Sci 217, 143–149.PubMedCrossRefGoogle Scholar
  7. 7.
    Baltzer, W. I., Calise, D. V., Levine, J. M., Shelton, G. D., Edwards, J. F., and Steiner, J. M. (2007) Dystrophin-deficient muscular dystrophy in a Weimaraner. J Am Anim Hosp Assoc 43, 227–232.PubMedGoogle Scholar
  8. 8.
    Gaschen, F. P., Hoffman, E. P., Gorospe, J. R., Uhl, E. W., Senior, D. F., Cardinet, G. H. 3rd, et al. (1992) Dystrophin deficiency causes lethal muscle hypertrophy in cats. J Neurol Sci 110, 149–159.PubMedCrossRefGoogle Scholar
  9. 9.
    Winand, N. J., Edwards, M., Pradhan, D., Berian, C. A., and Cooper, B. J. (1994) Deletion of the dystrophin muscle promoter in feline muscular dystrophy. Neuromuscul Disord 4, 433–445.PubMedCrossRefGoogle Scholar
  10. 10.
    Sharp, N. J. H., Kornegay, J. N., Van Camp, S. D., Herbstreith, M. H., Secore, S. L., Kettle, S., et al. (1991) An error in dystrophin mRNA processing in golden retriever muscular dystrophy, an animal homologue of Duchenne muscular dystrophy. Genomics 13, 115–121.CrossRefGoogle Scholar
  11. 11.
    Vignos, P. J., Jr., Spencer, G. E., Jr, and Archibald, J. C. (1963) Management of ­muscular dystrophy of childhood. JAMA 184, 89–96.PubMedGoogle Scholar
  12. 12.
    Brooke, M. H., Fenichel, G. M., Griggs, R. C., Mendell, J. R., Moxley, R., Miller, J. P., et al. (1983) Clinical Investigation in Duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history. Muscle Nerve 6, 91–103.PubMedCrossRefGoogle Scholar
  13. 13.
    Siegel, I. M., Miller, J. E., and Ray, R. D. (1968) Subcutaneous lower limb tenotomy in the treatment of pseudohypertrophic muscular dystrophy. J Bone Joint Surg 50A, 1437–1443.Google Scholar
  14. 14.
    Kornegay, J. N., Sharp, N. J. H., Schueler, R. O., and Betts, C. W. (1994) Tarsal joint contracture in dogs with golden retriever muscular dystrophy. Lab Anim Sci 44, 331–333.PubMedGoogle Scholar
  15. 15.
    Kornegay, J. N., Sharp, N. J. H., Bogan, D. J., Van Camp, S. D., Metcalf, J. R., and Schueler, R. O. (1994) Contraction tension and kinetics of the peroneus longus muscle in golden retriever muscular dystrophy. J Neurol Sci 123, 100–107.PubMedCrossRefGoogle Scholar
  16. 16.
    Kornegay, J. N., Bogan, D. J., Bogan, J. R., Childers, M. K., Cundiff, D. D., Petroski, G. F., et al. (1999) Contraction force generated by tarsal joint flexion and extension in dogs with golden retriever muscular dystrophy. J Neurol Sci 166, 115–121.PubMedCrossRefGoogle Scholar
  17. 17.
    Childers, M. K., Okamura, C. S., Bogan, D. J., Bogan, J. R., Petroski, G. F., McDonald K., et al. (2002) Eccentric contraction injury in dystrophic canine muscle. Arch Phys Med Rehabil 83, 1572–1578.PubMedCrossRefGoogle Scholar
  18. 18.
    Liu, J. M. K., Okamura, C. S., Bogan, D. J., Bogan, J. R, Childers, M. K., and Kornegay, J. N. (2004) Effects of prednisone in canine muscular dystrophy. Muscle Nerve 30, 767–773.PubMedCrossRefGoogle Scholar
  19. 19.
    Jaegger, G., Marcellin-Little, D. J., and Levine, D. (2002) Reliability of goniometry in Labrador retrievers. Am J Vet Res 63, 979–986.PubMedCrossRefGoogle Scholar
  20. 20.
    Nicholson, H. L., Osmotherly, P. G., Smith, B.A., and McGowan, C. M. (2007) Determinants of passive hip range of motion in adult Greyhounds. Aust Vet J 85, 217–221.PubMedCrossRefGoogle Scholar
  21. 21.
    Edwards, R. H. T., Jones, D. A., Newham, D. J., and Chapman, S. J. (1984) Role of mechanical damage in the pathogenesis of proximal myopathy in man. Lancet 1, 548–552.PubMedCrossRefGoogle Scholar
  22. 22.
    Dangain, J. and Vrbova, G. (1984) Muscle development in mdx mutant mice. Muscle Nerve 7, 700–704.PubMedCrossRefGoogle Scholar
  23. 23.
    Valentine, B. A. and Cooper, B. J. (1991) Canine X-linked muscular dystrophy: selective involvement of muscles in neonatal dogs. Neuromuscul Disord 1, 31–38.PubMedCrossRefGoogle Scholar
  24. 24.
    Moens, P., Baatsen, P. H. W. W., and Marechal, G. (1993) Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch. J Mus Res Cell Motil 14, 446–451.CrossRefGoogle Scholar
  25. 25.
    Thomassen, R., Sanson, G., Krogenæs, A., Fougnerb, J. A., Andersen Berg, A., and Farstad, W. (2006) Artificial insemination with frozen semen in dogs: a retrospective study of 10 years using a non-surgical approach. Theriogenology 66, 1645–1650.PubMedCrossRefGoogle Scholar
  26. 26.
    Kelley, R. L. (2001) Factors influencing canine reproduction and nutritional management of the pregnant bitch. Canine Reproduction and Nutritional Health – Tufts Animal Expo, pp 9–14.Google Scholar
  27. 27.
    Kornegay, J. N., Cundiff, D. D., Bogan, D. J., Bogan, J. R., and Okamura, C. S. (2003) The cranial sartorius muscle undergoes true hypertrophy in dogs with golden retriever muscular dystrophy. Neuromuscul Disord 13, 493–500.PubMedCrossRefGoogle Scholar
  28. 28.
    Salimena, M. C., Lagrota-Candido, J., and Quírico-Santos, T. (2004) Gender dimorphism influences extracellular matrix expression and regeneration of muscular tissue in mdx dystrophic mice. Histochem Cell Biol 122, 535–544.Google Scholar
  29. 29.
    Valentine, B. A., Cooper, B. J., de Lahunta, A, O’Quinn, R., and Blue, J. T. (1988) Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: clinical studies. J Neurol Sci 88, 69–81.PubMedCrossRefGoogle Scholar
  30. 30.
    Gordon, A. M., Huxley, A. F., and Julian, F. J. (1966) The variation in isometric tension with sarcomere length in vertebrate muscle fibres. J Physiol 184, 170–192.PubMedGoogle Scholar
  31. 31.
    Hoy, M. G., Zajac, F. E., and Gordon, M. E. (1990) A musculoskeletal model of the human lower extremity: the effect of muscle, tendon, and moment arm on the moment-angle relationship of musculotendon actuators at the hip, knee, and ankle. J Biomechanics 23, 157–169.CrossRefGoogle Scholar
  32. 32.
    Lieber, R. L. and Boakes, J. L. (1988) Sarcomere length and joint kinematics during torque production in frog hindlimb. Am J Physiol 254, C759–C768.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory Medicine and The Gene Therapy Center, School of MedicineUniversity of North Carolina-Chapel HillChapel HillUSA
  2. 2.Department of Neurology and Wake Forest Institute for Regenerative Medicine, School of MedicineWake Forest UniversityWinston-SalemUSA
  3. 3.Department of Human Nutrition, Foods, and Exercise, College of Agriculture and Life SciencesVirginia Tech UniversityBlacksburgUSA

Personalised recommendations

Cite protocol

Buy options