Trends in Cardiovascular Medicine
Volume 17, Issue 2 , Pages 55-59 , February 2007

Consequences of Disrupting the Dystrophin-Sarcoglycan Complex in Cardiac and Skeletal Myopathy

  • Ahlke Heydemann

      Affiliations

    • Department of Medicine, Section of Cardiology, The University of Chicago, Chicago, IL 60637, USA
    • ,
  • Elizabeth M. McNally

      Affiliations

    • Department of Medicine, Section of Cardiology, The University of Chicago, Chicago, IL 60637, USA
    • Department of Human Genetics, The University of Chicago, Chicago, IL 60637, USA
    • Corresponding Author InformationAddress correspondence to: Dr. Elizabeth M. McNally, 5841 S. Maryland, MC6088, Chicago, IL 60637, USA. Tel.: (+1) 773 702 2672; fax: (+1) 773 702 2681.

References 

  1. Armstrong SC, Latham CA, Shivell CL, Ganote CE. Ischemic loss of sarcolemmal dystrophin and spectrin: correlation with myocardial injury. J Mol Cell Cardiol. 2001;33:1165–1179
  2. Badorff C, Knowlton KU. Dystrophin disruption in enterovirus-induced myocarditis and dilated cardiomyopathy: from bench to bedside. Med Microbiol Immunol (Berl). 2004;193:121–126
  3. Barton ER. Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle. Am J Physiol Cell Physiol. 2006;290:C411–C419
  4. Basset O, Boittin FX, Cognard C, et al. Bcl-2 overexpression prevents calcium overload and subsequent apoptosis in dystrophic myotubes. Biochem J. 2006;395:267–276
  5. Betto R, Senter L, Ceoldo S, et al. Ecto-ATPase activity of alpha-sarcoglycan (adhalin). J Biol Chem. 1999;274:7907–7912
  6. Danko I, Chapman V, Wolff JA. The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr Res. 1992;32:128–131
  7. Dell'Agnola C, Wang Z, Storb R, et al. Hematopoietic stem cell transplantation does not restore dystrophin expression in Duchenne muscular dystrophy dogs. Blood. 2004;104:4311–4318
  8. Doherty KR, Cave A, Davis DB, et al. Normal myoblast fusion requires myoferlin. Development. 2005;132:5565–5575
  9. Duboc D, Meune C, Lerebours G, et al. Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy. J Am Coll Cardiol. 2005;45:855–857
  10. Gee SH, Madhavan R, Levinson SR, et al. Interaction of muscle and brain sodium channels with multiple members of the syntrophin family of dystrophin-associated proteins. J Neurosci. 1998;18:128–137
  11. Griffin MA, Feng H, Tewari M, et al. Gamma-sarcoglycan deficiency increases cell contractility, apoptosis and MAPK pathway activation but does not affect adhesion. J Cell Sci. 2005;118:1405–1416
  12. Hack AA, Cordier L, Shoturma DI, et al. Muscle degeneration without mechanical injury in sarcoglycan deficiency. Proc Natl Acad Sci U S A. 1999;96:10723–10728
  13. Hack AA, Ly CT, Jiang F, et al. Gamma-sarcoglycan deficiency leads to muscle membrane defects and apoptosis independent of dystrophin. J Cell Biol. 1998;142:1279–1287
  14. Hainsey TA, Senapati S, Kuhn DE, Rafael JA. Cardiomyopathic features associated with muscular dystrophy are independent of dystrophin absence in cardiovasculature. Neuromuscul Disord. 2003;13:294–302
  15. Heydemann A, Huber JM, Kakkar R, et al. Functional nitric oxide synthase mislocalization in cardiomyopathy. J Mol Cell Cardiol. 2004;36:213–223
  16. Heydemann A, Demonbreun A, Hadhazy M, et al. Nuclear sequestration of δ-sarcoglycan disrupts the nuclear localization of lamin A/C and emerin in cardiomyocytes. Hum Mol Genet. 2006;(in press)
  17. Hoffman EP, Morgan JE, Watkins SC, Partridge TA. Somatic reversion/suppression of the mouse mdx phenotype in vivo. J Neurol Sci. 1990;99:9–25
  18. Holt KH, Campbell KP. Assembly of the sarcoglycan complex. Insights for muscular dystrophy. J Biol Chem. 1998;273:34667–34670
  19. Ichihara S, Yamada Y, Ichihara G, et al. Attenuation of oxidative stress and cardiac dysfunction by bisoprolol in an animal model of dilated cardiomyopathy. Biochem Biophys Res Commun. 2006;350:105–113
  20. Jefferies JL, Eidem BW, Belmont JW, et al. Genetic predictors and remodeling of dilated cardiomyopathy in muscular dystrophy. Circulation. 2005;112:2799–2804
  21. Kyoi S, Otani H, Matsuhisa S, et al. Role of oxidative/nitrosative stress in the tolerance to ischemia/reperfusion injury in cardiomyopathic hamster heart. Antioxid Redox Signal. 2006;8:1351–1361
  22. Lapidos KA, Chen YE, Earley JU, et al. Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle. J Clin Invest. 2004;114:1577–1585
  23. Lee GH, Badorff C, Knowlton KU. Dissociation of sarcoglycans and the dystrophin carboxyl terminus from the sarcolemma in enteroviral cardiomyopathy. Circ Res. 2000;87:489–495
  24. Matsumura K, Arai K, Zhong D, et al. Disruption of dystroglycan axis by beta-dystroglycan processing in cardiomyopathic hamster muscle. Neuromuscul Disord. 2003;13:796–803
  25. Matsumura K, Zhong D, Saito F, et al. Proteolysis of beta-dystroglycan in muscular diseases. Neuromuscul Disord. 2005;15:336–341
  26. Oak SA, Russo K, Petrucci TC, Jarrett HW. Mouse alpha1-syntrophin binding to Grb2: further evidence of a role for syntrophin in cell signaling. Biochemistry. 2001;40:11270–11278
  27. Rodriguez M, Cai WJ, Kostin S, et al. Ischemia depletes dystrophin and inhibits protein synthesis in the canine heart: mechanisms of myocardial ischemic injury. J Mol Cell Cardiol. 2005;38:723–733
  28. Rosenzweig A. Cardiac cell therapy—mixed results from mixed cells. N Engl J Med. 2006;355:1274–1277
  29. Rybakova IN, Patel JR, Ervasti JM. The dystrophin complex forms a mechanically strong link between the sarcolemma and costameric actin. J Cell Biol. 2000;150:1209–1214
  30. Sakamoto A, Ono K, Abe M, et al. Both hypertrophic and dilated cardiomyopathies are caused by mutation of the same gene, delta-sarcoglycan, in hamster: an animal model of disrupted dystrophin-associated glycoprotein complex. Proc Natl Acad Sci U S A. 1997;94:13873–13878
  31. Sandona D, Gastaldello S, Martinello T, Betto R. Characterization of the ATP-hydrolysing activity of alpha-sarcoglycan. Biochem J. 2004;381:105–112
  32. Shelton GD, Engvall E. Canine and feline models of human inherited muscle diseases. Neuromuscul Disord. 2005;15:127–138
  33. Tews DS. Apoptosis and muscle fibre loss in neuromuscular disorders. Neuromuscul Disord. 2002;12:613–622
  34. Tews DS. Tumour necrosis factor-mediated cell death pathways do not contribute to muscle fibre death in dystrophinopathies. Acta Neuropathol (Berl). 2005;109:217–225
  35. Thompson TG, Chan YM, Hack AA, et al. Filamin 2 (FLN2): a muscle-specific sarcoglycan interacting protein. J Cell Biol. 2000;148:115–126
  36. Vatta M, Stetson SJ, Perez-Verdia A, et al. Molecular remodelling of dystrophin in patients with end-stage cardiomyopathies and reversal in patients on assistance-device therapy. Lancet. 2002;359:936–941
  37. Wheeler MT, Allikian MJ, Heydemann A, et al. Smooth muscle cell-extrinsic vascular spasm arises from cardiomyocyte degeneration in sarcoglycan-deficient cardiomyopathy. J Clin Invest. 2004;113:668–675
  38. Yamada H, Saito F, Fukuta-Ohi H, et al. Processing of beta-dystroglycan by matrix metalloproteinase disrupts the link between the extracellular matrix and cell membrane via the dystroglycan complex. Hum Mol Genet. 2001;10:1563–1569
  39. Yang B, Jung D, Motto D, et al. SH3 domain-mediated interaction of dystroglycan and Grb2. J Biol Chem. 1995;270:11711–11714
  40. Yasuda S, Townsend D, Michele DE, et al. Dystrophic heart failure blocked by membrane sealant poloxamer. Nature. 2005;436:1025–1029
  41. Yoshida T, Pan Y, Hanada H, et al. Bidirectional signaling between sarcoglycans and the integrin adhesion system in cultured L6 myocytes. J Biol Chem. 1998;273:1583–1590

PII: S1050-1738(07)00002-3

doi: 10.1016/j.tcm.2006.12.002

Trends in Cardiovascular Medicine
Volume 17, Issue 2 , Pages 55-59 , February 2007

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