Neuromuscular Disorders
Volume 20, Issue 1 , Pages 21-28 , January 2010

Attenuation of adverse cardiac effects in prednisolone-treated δ-sarcoglycan-deficient mice by mineralocorticoid-receptor-antagonism

  • Ralf Bauer

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
    • Dept. of Cardiology, Angiology and Pneumology, University Hospital, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany
  • ,
  • Alison Blain

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
  • ,
  • Elizabeth Greally

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
  • ,
  • Hanns Lochmüller

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
  • ,
  • Kate Bushby

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
  • ,
  • Guy A. MacGowan

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
    • Dept. of Cardiology, Freeman Hospital, Newcastle upon Tyne NE7 7DN, UK
  • ,
  • Volker Straub

      Affiliations

    • Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne NE1 3BZ, UK
    • Corresponding Author InformationCorresponding author. Address: Institute of Human Genetics, Newcastle University, International Centre for Life, Central Parkway, Newcastle upon Tyne NE1 3 BZ, United Kingdom. Tel.: +44 19122418655; fax: +44 19124187990.

Received 23 July 2009 ,Revised 29 September 2009 ,Accepted 5 October 2009.

References 

  1. Porter JD, Khanna S, Kaminski HJ, et al. A chronic inflammatory response dominates the skeletal muscle molecular signature in dystrophin-deficient mdx mice. Hum Mol Genet. 2002;11:263–272
  2. Bushby K, Muntoni F, Urtizberea A, Hughes R, Griggs R. Report on the 124th ENMC International Workshop. Treatment of Duchenne muscular dystrophy; defining the gold standards of management in the use of corticosteroids. 2–4 April 2004, Naarden, The Netherlands. Neuromuscul Disord. 2004;14:526–534
  3. Silversides CK, Webb GD, Harris VA, Biggar DW. Effects of deflazacort on left ventricular function in patients with Duchenne muscular dystrophy. Am J Cardiol. 2003;91:769–772
  4. Markham LW, Spicer RL, Khoury PR, Wong BL, Mathews KD, Cripe LH. Steroid therapy and cardiac function in Duchenne muscular dystrophy. Pediatr Cardiol. 2005;26:768–771
  5. Bauer R, MacGowan GA, Blain A, Bushby K, Straub V. Steroid treatment causes deterioration of myocardial function in the δ-sarcoglycan-deficient mouse model for dilated cardiomyopathy. Cardiovasc Res. 2008;79:652–661
  6. Heydemann A, McNally EM. Consequences of disrupting the dystrophin–sarcoglycan complex in cardiac and skeletal myopathy. Trends Cardiovasc Med. 2007;17:55–59
  7. Ozawa E, Mizuno Y, Hagiwara Y, Sasaoka T, Yoshida M. Molecular and cell biology of the sarcoglycan complex. Muscle Nerve. 2005;32:563–576
  8. Lapidos KA, Kakkar R, McNally EM. The dystrophin glycoprotein complex: signaling strength and integrity for the sarcolemma. Circ Res. 2004;94:1023–1031
  9. Danialou G, Comtois AS, Dudley R, et al. Dystrophin-deficient cardiomyocytes are abnormally vulnerable to mechanical stress-induced contractile failure and injury. FASEB J. 2001;15:1655–1657
  10. Wheeler MT, McNally EM. Sarcoglycans in vascular smooth and striated muscle. Trends Cardiovasc Med. 2003;13:238–243
  11. Coral-Vazquez R, Cohn RD, Moore SA, et al. Disruption of the sarcoglycan–sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy. Cell. 1999;98:465–474
  12. Hack AA, Lam MY, Cordier L, et al. Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin–glycoprotein complex. J Cell Sci. 2000;113:2535–2544
  13. Walker BR. Glucocorticoids and cardiovascular disease. Eur J Endocrinol. 2007;157:545–559
  14. Qin W, Rudolph AE, Bond BR, et al. Transgenic model of aldosterone-driven cardiac hypertrophy and heart failure. Circ Res. 2003;93:69–76
  15. Nagata K, Obata K, Xu J, et al. Mineralocorticoid receptor antagonism attenuates cardiac hypertrophy and failure in low-aldosterone hypertensive rats. Hypertension. 2006;47:656–664
  16. Rickard AJ, Funder JW, Fuller PJ, Young MJ. The role of the glucocorticoid receptor in mineralocorticoid/salt-mediated cardiac fibrosis. Endocrinology. 2006;147:5901–5906
  17. Funder JW. RALES, EPHESUS and redox. J Steroid Biochem Mol Biol. 2005;93:121–125
  18. Pitt B, Zannad F, Remme WJ, et al. The effect of spironolactone on morbidity and mortality in patients with severe heart failure. Randomized aldactone evaluation study investigators. N Engl J Med. 1999;341:709–717
  19. Hayashi M, Tsutamoto T, Wada A, et al. Immediate administration of mineralocorticoid receptor antagonist spironolactone prevents post-infarct left ventricular remodeling associated with suppression of a marker of myocardial collagen synthesis in patients with first anterior acute myocardial infarction. Circulation. 2003;107:2559–2565
  20. Katada J, Meguro T, Saito H, et al. Persistent cardiac aldosterone synthesis in angiotensin II type 1A receptor-knockout mice after myocardial infarction. Circulation. 2005;111:2157–2164
  21. MacGowan GA, Rager J, Shroff SG, Mathier MA. In vivo alpha-adrenergic responses and troponin I phosphorylation: anesthesia interactions. J Appl Physiol. 2005;98:1163–1170
  22. Steendijk P, Staal E, Jukema JW, Baan J. Hypertonic saline method accurately determines parallel conductance for dual-field conductance catheter. Am J Physiol Heart Circ Physiol. 2001;281:H755–H763
  23. Suga H, Sagawa K. Instantaneous pressure-volume relationships and their ratio in the excised, supported canine left ventricle. Circ Res. 1974;35:117–126
  24. Glower DD, Spratt JA, Snow ND, et al. Linearity of the Frank–Starling relationship in the intact heart: the concept of preload recruitable stroke work. Circulation. 1985;71:994–1009
  25. Hamer PW, McGeachie JM, Davies MJ, Grounds MD. Evans Blue Dye as an in vivo marker of myofibre damage: optimising parameters for detecting initial myofibre membrane permeability. J Anat. 2002;200:69–79
  26. Pfaffl MW, Horgan GW, Dempfle L. Relative expression software tool (REST) for group-wise comparison and statistical analysis of relative expression results in real-time PCR. Nucleic Acids Res. 2002;30:e36
  27. Connolly AM, Schierbecker J, Renna R, Florence J. High dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy. Neuromuscul Disord. 2002;12:917–925
  28. Barbato JC, Mulrow PJ, Shapiro JI, Franco-Saenz R. Rapid effects of aldosterone and spironolactone in the isolated working rat heart. Hypertension. 2002;40:130–135
  29. Doggrell SA, Brown L. The spironolactone renaissance. Expert Opin Investig Drugs. 2001;10:943–954
  30. Thai HM, Do BQ, Tran TD, Gaballa MA, Goldman S. Aldosterone antagonism improves endothelial-dependent vasorelaxation in heart failure via upregulation of endothelial nitric oxide synthase production. J Card Fail. 2006;12:240–245
  31. Graham HK, Trafford AW. Spatial disruption and enhanced degradation of collagen with the transition from compensated ventricular hypertrophy to symptomatic congestive heart failure. Am J Physiol Heart Circ Physiol. 2007;292:H1364–H1372
  32. Marijianowski MMH, Teelin P, Mann J, Becker AE. Dilated cardiomyopathy is associated with an increase in the type I/type III collagen ration: a quantitative assessment. J Am Coll Cardiol. 1995;25:1263–1272
  33. Kass DA, Hare JM, Georgakopoulos D. Murine cardiac function: a cautionary tail. Circ Res. 1998;82:407–415

PII: S0960-8966(09)00654-3

doi: 10.1016/j.nmd.2009.10.003

Neuromuscular Disorders
Volume 20, Issue 1 , Pages 21-28 , January 2010