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An Introduction to Mitochondria and the Heart

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Mitochondria and the Heart

Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 256))

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References

  1. Morris RL, Hollenbeck PJ (1995) Axonal transport of mitochondria along microtubules and F-actin in living vertebrate neurons. J Cell Biol 131:1315–26

    Article  PubMed  CAS  Google Scholar 

  2. Rizzuto R, Pinton P, Carrington W, Fay FS, Fogarty KE, Lifshitz LM, Tuft RA, Pozzan T (1998) Close contacts with the endoplasmic reticulum as determinants of mitochondrial Ca2+ responses. Science 280:1763–6

    Article  PubMed  CAS  Google Scholar 

  3. Duchen MR, Surin A, Jacobson J (2003) Imaging mitochondrial function in intact cells. Methods Enzymol 361:353–89

    PubMed  CAS  Google Scholar 

  4. Poot M, Zhang YZ, Kramer JA, Wells KS, Jones LJ, Hanzel DK, Lugade AG, Singer VL, Haugland RP (1996) Analysis of mitochondrial morphology and function with novel fixable fluorescent stains. J Histochem Cytochem 44:1363–72

    PubMed  CAS  Google Scholar 

  5. Reers M, Smiley ST, Mottola-Hartshorn C, Chen A, Lin M, Chen LB (1995) Mitochondrial membrane potential monitored by JC-1 dye. Methods Enzymol 260:406–17

    PubMed  CAS  Google Scholar 

  6. Mathur A, Hong Y, Kemp BK, Barrientos AA, Erusalimsky JD (2000) Evaluation of fluorescent dyes for the detection of mitochondrial membrane potential changes in cultured cardiomyocytes. Cardiovasc Res 46:126–38

    Article  PubMed  CAS  Google Scholar 

  7. Malgaroli A, Milani D, Meldolesi J, Pozzan T (1987) Fura-2 measurement of cytosolic free Ca2+ in monolayers and suspensions of various types of animal cells. J Cell Biol 105: 2145–55

    Article  PubMed  CAS  Google Scholar 

  8. Bowser DN, Minamikawa T, Nagley P, Williams DA (1998) Role of mitochondria in calcium regulation of spontaneously contracting cardiac muscle cells. Biophys J 75:2004–14

    Article  PubMed  CAS  Google Scholar 

  9. King MP, Attardi G (1989) Human cells lacking mtDNA: Repopulation with exogenous mitochondria by complementation. Science 246:500–3

    PubMed  CAS  Google Scholar 

  10. Chomyn A, Meola G, Bresolin N, Lai ST, Scarlato G, Attardi G (1991) In vitro genetic transfer of protein synthesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria. Mol Cell Biol 11:2236–44

    PubMed  CAS  Google Scholar 

  11. Anderson S, Bankier AT, Barrell BG, De Bruijn MHL, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG (1981) Sequence and organization of human mitochondrial genome. Nature 290:457–65

    Article  PubMed  CAS  Google Scholar 

  12. Attardi G, Schatz G (1988) Biogenesis of mitochondria. Annu Rev Cell Biol 4:289–333

    Article  PubMed  CAS  Google Scholar 

  13. Shadel GS, Clayton DA (1997) Mitochondrial DNA maintenance in vertebrates. Annu Rev Biochem 66:409–35

    Article  PubMed  CAS  Google Scholar 

  14. Lopaschuk GD, Collins-Nakai RL, Itoi T (1992) Developmental changes in energy substrate use by the heart. Cardiovasc Res 26:1172–80

    PubMed  CAS  Google Scholar 

  15. Paradies G, Ruggiero FM (1990) Age-related changes in the activity of the pyruvate carrier and in the lipid composition in rat-heart mitochondria. Biochim Biophys Acta 1016:207–21

    Article  PubMed  CAS  Google Scholar 

  16. McMillin JB, Taffet GE, Taegtmeyer H, Hudson EK, Tate CA (1993) Mitochondrial metabolism and substrate competition in the aging Fischer rat heart. Cardiovasc Res 27:2222–8

    Article  PubMed  CAS  Google Scholar 

  17. Raha S, Robinson BH (2000) Mitochondria, oxygen free radicals, disease and ageing. Trends Biochem Sci 25:502–8

    Article  PubMed  CAS  Google Scholar 

  18. Arnes BN, Shigenaga MK, Hagen TM (1995) Mitochondrial decay in aging. Biochim Biophys Acta 1272:165–70

    Google Scholar 

  19. Rustin P, Lebidois J, Chretien D, Bourgeron T, Piechaud JF, Rotig A, Munnich A, Sidi D (1994) Endomyocardial biopsies for early detection of mitochondrial disorders in hypertrophic cardiomyopathies. J Pediatr 124:224–8

    PubMed  CAS  Google Scholar 

  20. Marín-García J, Goldenthal MJ, Pierpont ME, Ananthakrishnan R. (1995) Impaired mitochondrial function in idiopathic dilated cardiomyopathy: Biochemical and molecular analysis. J Card Fail 1:285–91

    Article  PubMed  Google Scholar 

  21. Schon EA, Bonilla E, DiMauro S (1997) Mitochondrial DNA mutations and pathogenesis. J Bioenerg Biomembr 29:131–49

    Article  PubMed  CAS  Google Scholar 

  22. Marín-García J, Goldenthal MJ (2002) Understanding the impact of mitochondrial defects in cardiovascular disease: A review. J Card Fail 8:347–61

    Article  PubMed  CAS  Google Scholar 

  23. Shoffner JM, Wallace DC (1992) Heart disease and mitochondrial DNA mutations. Heart Dis Stroke 1:235–41

    PubMed  CAS  Google Scholar 

  24. McFarland R, Clark KM, Morris AA, Taylor RW, Macphail S, Lightowlers RN, Turnbull DM (2002) Multiple neonatal deaths due to a homoplasmic mitochondrial DNA mutation. Nat Genet 30: 145–6

    Article  PubMed  CAS  Google Scholar 

  25. Pastores GM, Santorelli FM, Shanske S, Gelb BD, Fyfe B, Wolfe D, Willner JP (1994) Leigh syndrome and hypertrophic cardiomyopathy in an infant with a mitochondrial point mutation (T8993G). Am J Med Genet 50:265–71

    Article  PubMed  CAS  Google Scholar 

  26. Graf WD, Marín-García J, Gao HG, Pizzo S, Naviaux RK, Markusic D, Barshop BA, Courchesne E, Haas RH (2000) Autism associated with the mitochondrial DNA G8363A transfer RNA(Lys)mutation. J Child Neurol 15:357–61

    PubMed  CAS  Google Scholar 

  27. Matthews PM, Marchington DR, Squier M, Land J, Brown R, Brown GK (1993) Molecular genetic characterization of an X-linked form of Leigh’s syndrome. Ann Neurol 33:652–5

    Article  PubMed  CAS  Google Scholar 

  28. Bourgeron T, Rustin P, Chretien D, Birch-Machin M, Bourgeois M, Viegas-Pequignot E, Munnich A, Rotig A (1995) Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency. Nat Genet 11:144–9

    Article  PubMed  CAS  Google Scholar 

  29. Filiano JJ, Goldenthal MJ, Mamourian AC, Hall CC, Marín-García J (2002) Mitochondrial DNA depletion in Leigh syndrome. Pediatr Neurol 26:239–42

    Article  PubMed  Google Scholar 

  30. Holt IJ, Harding AE, Morgan-Hughes JA (1988) Deletions of mtDNA in patients with mitochondrial myopathies. Nature 331:717–9

    Article  PubMed  CAS  Google Scholar 

  31. Zeviani M, Moraes CT, DiMauro S, Nakase H, Bonilla E, Schon EA, Rowland LP (1988) Deletions of mitochondrial DNA in Kearns-Sayre syndrome. Neurology 38:1339–4

    PubMed  CAS  Google Scholar 

  32. Bohlega S, Tanji K, Santorelli FM, Hirano M, al-Jishi A, DiMauro S (1996) Multiple mitochondrial DNA deletions associated with autosomal recessive ophthalmoplegia and severe cardiomyopathy severe cardiomyopathy. Neurology 46:1329–34

    PubMed  CAS  Google Scholar 

  33. Suomalainen A, Paetau A, Leinonen H, Majander A, Peltonen L, Somer H. (1992) Inherited idiopathic dilated cardiomyopathy with multiple deletions of mitochondrial DNA. Lancet 340:1319–20

    Article  PubMed  CAS  Google Scholar 

  34. Marín-García J, Goldenthal MJ, Ananthakrishnan R, Pierpont ME, Fricker FJ, Lipshultz S, Perez-Atayde A (1996) Specific mitochondrial DNA deletions in idiopathic dilated cardiomyopathy. Cardiovasc Res 31:306–14

    Article  PubMed  Google Scholar 

  35. Marín-García J, Ananthakrishnan R, Goldenthal MJ, Pierpont ME (2000) Biochemical and molecular basis for mitochondrial cardiomyopathy in neonates and children. J Inherit Metab Dis 23:625–33

    Article  PubMed  Google Scholar 

  36. Poulton J, Sewry C, Potter CG, Bougeron T, Chretien D, Wijberg FA, Morten KJ, Brown G (1995) Variation in mitochondrial DNA levels in muscle from normal controls. Is depletion of mtDNA in patients with mitochondrial myopathy a distinct clinical syndrome. J Inher Metab Dis 18:4–20

    Article  PubMed  CAS  Google Scholar 

  37. Lewis W, Dalakas MC (1995) Mitochondrial toxicity of antiviral drugs. Nature Med 1:417–22

    Article  PubMed  CAS  Google Scholar 

  38. Serrano J, Palmeira CM, Kuehl DW, Wallace KB (1999) Cardioselective and cumulative oxidation of mitochondrial DNA following subchronic doxorubicin administration. Biochim Biophys Acta 1411:201–5

    Article  PubMed  CAS  Google Scholar 

  39. Monros E, Molto MD, Martinez F, Canizares J, Bianca J, Vilchez JJ, Prieto F, de Frutos R, Palau F (1997) Phenotype correlation and intergenerational dynamics of the Friedreich ataxia GAA trinucleotide repeat. Am J Hum Genet 61:101–10

    PubMed  CAS  Google Scholar 

  40. Papadopoulou LC, Sue CM, Davidson MM, Tanji K, Nishino I, Sadlock JE, Krishna S, Walker W, Selby J, Glerum DM, Coster RV, Lyon G, Scalais E, Lebel R, Kaplan P, Shanske S, De Vivo DC, Bonilla E, Hirano M, DiMauro S, Schon EA (1999) Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene. Nat Genet 23:333–7

    Article  PubMed  CAS  Google Scholar 

  41. Kelly DP, Strauss AW (1994) Inherited cardiomyopathies. N Eng J Med 330:913–9

    Article  CAS  Google Scholar 

  42. Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF (1995) Molecular basis of human mitochondriial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood. Proc Natl Acad Sci USA 92:10496–500

    PubMed  CAS  Google Scholar 

  43. Rocchiccioli F, Wanders RJ, Aubourg P, Vianey-Liaud C, Ijlst L, Fabre M, Cartier N, Bougneres PF (1990) Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: A cause of lethal myopathy and cardiomyopathy in early childhood. Pediatr Res 28:657–62

    PubMed  CAS  Google Scholar 

  44. Tein I, Haslam RH, Rhead WJ, Bennett MJ, Becker LE, Vockley J (1999) Short-chain acyl-CoA dehydrogenase deficiency: A cause of ophthalmoplegia and multicore myopathy. Neurology 52:366–72

    PubMed  CAS  Google Scholar 

  45. Brackett JC, Sims HF, Rinaldo P, Shapiro S, Powell CK, Bennett MJ, Strauss AW (1995) Two subunit donor splice site mutations cause human trifunctional protein deficiency. J Clin Invest 95: 2076–82

    PubMed  CAS  Google Scholar 

  46. Stanley CA, Treem WR, Hale DE, Coates PM (1990) A genetic defect in carnitine transport causing primary carnitine deficiency. Prog Clin Biol Res 321:457–64

    PubMed  CAS  Google Scholar 

  47. Bonnet D, Martin D, de Lonlay P, Villain E, Jouvet P, Rabier D, Brivet M, Saudubray JM (1999) Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. Circulation 100:2248–53

    PubMed  CAS  Google Scholar 

  48. Corr PB, Creer MH, Yamada KA, Saffitz JE, Sobel BE (1989) Prophylaxis of early ventricular fibrillation by inhibition of acylcarnitine accumulation. J Clin Invest 83:927–36

    PubMed  CAS  Google Scholar 

  49. Tripp ME (1989) Developmental cardiac metabolism in health and disease. Pediatr Cardiol 10:150–8

    Article  PubMed  CAS  Google Scholar 

  50. D’Adamo P, Fassone L, Gedeon A, Janssen EA, Bione S, Bolhuis PA, Barth PG, Wilson M, Haan E, Orstavik KH, Patton MA, Green AJ, Zammarchi E, Donati MA, Toniolo D (1997) The X-linked gene G4.5 is responsible for different infantile dilated cardiomyopathies. Am J Hum Genet 61:862–7

    PubMed  CAS  Google Scholar 

  51. Bissler JJ, Tsoras M, Goring HH, Hug P, Chuck G, Tombragel E, McGraw C, Schlotman J, Ralston MA, Hug G (2002) Infantile dilated X-linked cardiomyopathy, G4.5 mutations, altered lipids, and ultrastructural malformations of mitochondria in heart, liver, and skeletal muscle. Lab Invest 82:335–44

    PubMed  CAS  Google Scholar 

  52. Vreken P, Valianpour F, Nijtmans LG, Grivell LA, Plecko B, Wanders RJ, Barth PG (2000) Defective remodeling of cardiolipin and phosphatidylglycerol in Barth syndrome. Biochem Biophys Res Commun 279:378–82

    Article  PubMed  CAS  Google Scholar 

  53. Corbucci GG (2000) Adaptive changes in response to acute hypoxia, ischemia and reperfusion in human cardiac cell. Minerva Anestesiol 66:523–30

    PubMed  CAS  Google Scholar 

  54. Ylitalo K, Ala-Rami A, Vuorinen K, Peuhkurinen K, Lepojarvi M, Kaukoranta P, Kiviluoma K, Hassinen I (2001) Reversible ischemic inhibition of F(1)F(0)-ATPase in rat and human myocardium. Biochim Biophys Acta 1504:329–39

    Article  PubMed  CAS  Google Scholar 

  55. Lesnefsky EJ, Moghaddas S, Tandler B, Kerner J, Hoppel CL (2001) Mitochondrial dysfunction in cardiac disease: Ischemia-reperfusion, aging, and heart failure. J Mol Cell Cardiol 33:1065–89

    Article  PubMed  CAS  Google Scholar 

  56. Corral-Debrinski M, Stepien G, Shoffner JM, Lott MT, Kanter K, Wallace DC (1991) Hypoxemia is associated with mitochondrial DNA damage and gene induction. Implications for cardiac disease. JAMA 266:1812–6

    Article  PubMed  CAS  Google Scholar 

  57. Paradies G, Petrosillo G, Pistolese M, Di Venosa N, Serena D, Ruggiero FM (1999) Lipid peroxidation and alterations to oxidative metabolism in mitochondria isolated from rat heart subjected to ischemia and reperfusion. Free Radic Biol Med 27:42–50

    Article  PubMed  CAS  Google Scholar 

  58. Gottleib RA, Burleson KO, Kloner RA, Babior BM, Engler RL (1994) Reperfusion injury induces apoptosis in rabbit cardiomyocites. J Clin Invest 94:1621–28

    Google Scholar 

  59. Murry CE, Jennings RB, Reimer KA (1986) Preconditioning with ischemia: A delay of lethal cell injury in ischemic myocardium. Circulation 74:1124–36

    PubMed  CAS  Google Scholar 

  60. Murphy E (2004) Primary and secondary signaling pathways in early preconditioning that converge on the mitochondria to produce cardioprotection. Circ Res 94:7–16

    Article  PubMed  CAS  Google Scholar 

  61. Halestrap AP, Clarke SJ, Javadov SA (2004) Mitochondrial permeability transition pore opening during myocardial reperfusion: A target for cardioprotection. Cardiovasc Res 61:372–85

    Article  PubMed  CAS  Google Scholar 

  62. O’Rourke B (2004) Evidence for mitochondrial K+ channels and their role in cardioprotection. Circ Res. 94:420–32

    Article  PubMed  CAS  Google Scholar 

  63. Oldenburg O, Cohen M, Yellon D, Downey J (2002) Mitochondrial K(ATP) channels: Role in cardioprotection. Cardiovasc Res 55:429–3764.

    Article  PubMed  CAS  Google Scholar 

  64. Schulz R, Cohen MV, Behrends M, Downey JM, Heusch G (2001) Signal transduction of ischemic preconditioning. Cardiovasc Res 52:181–98

    Article  PubMed  CAS  Google Scholar 

  65. Hausenloy DJ, Maddock HL, Baxter GF, Yellon DM (2002) Inhibiting mitochondrial permeability transition pore opening: A new paradigm for myocardial preconditioning? Cardiovasc Res 55:534–43

    Article  PubMed  CAS  Google Scholar 

  66. Pain T, Yang XM, Critz SD, Yue Y, Nakano A, Liu GS, Heusch G, Cohen MV, Downey JM (2000) Opening of mitochondrial K(ATP) channels triggers the preconditioned state by generating free radicals. Circ Res 87:460–466

    PubMed  CAS  Google Scholar 

  67. Szewczyk A, Wojtczak L (2002) Mitochondria as a pharmacological target. Pharmacol Rev 54:101–27

    Article  PubMed  CAS  Google Scholar 

  68. Garlid KD, Paucek P, Yarov-Yarovoy V, Murray HN, Darbenzio R, D’Alonzo AJ, Lodge NJ, Smith MA, Grover GJ (1997) Cardioprotective effect of diazoxide and its interaction with mitochondrial ATP-sensitive K+ channels: Possible mechanism of cardioprotection. Circ Res 81:1072–82

    PubMed  CAS  Google Scholar 

  69. Ghosh S, Standen NB, Galinanes M (2000) Evidence for mitochondrial K ATP channels as effectors of human myocardial preconditioning. Cardiovasc Res 45:934–40

    Article  PubMed  CAS  Google Scholar 

  70. Regula KM, Ens K, Kirshenbaum LA (2003) Mitochondria-assisted cell suicide: A license to kill. J Mol Cell Cardiol 35:559–67

    Article  PubMed  CAS  Google Scholar 

  71. Danial NN, Korsmeyer SJ (2004) Cell death: Critical control points. Cell 116:205–19

    Article  PubMed  CAS  Google Scholar 

  72. Van Gurp M, Festjens N, van Loo G, Saelens X, Vandenabeele P (2003) Mitochondrial intermembrane proteins in cell death. Biochem Biophys Res Commun 304:487–97

    Article  PubMed  CAS  Google Scholar 

  73. Kroemer G (2003) Mitochondrial control of apoptosis: An introduction. Biochem Biophys Res Commun 304:433–5

    Article  PubMed  CAS  Google Scholar 

  74. Kuwana T, Mackey MR, Perkins G, Ellisman MH, Latterich M, Schneiter R, Green DR, Newmeyer DD (2002) Bid, Bax, and lipids cooperate to form supramolecular openings in the outer mitochondrial membrane. Cell 111:331–42

    Article  PubMed  CAS  Google Scholar 

  75. Marzo I, Brenner C, Zamzami N, Susin SA, Beutner G, Brdiczka D, Remy R, Xie ZH, Reed JC, Kroemer G (1998) The permeability transition pore complex: A target for apoptosis regulation by caspases and Bcl-2 related proteins. J Exp Med 187:1261–7

    Article  PubMed  CAS  Google Scholar 

  76. Lemasters JJ, Nieminen AL, Qian T, Trost LC, Elmore SP, Nishimura Y, Crowe RA, Cascio WE, Bradham CA, Brenner DA, Herman B (1998) The mitochondrial permeability transition in cell death: A common mechanism in necrosis, apoptosis and autophagy. Biochim Biophys Acta 1366:177–96

    Article  PubMed  CAS  Google Scholar 

  77. Marzo I, Brenner C, Zamzami N, Jurgensmeier JM, Susin SA, Vieira HL, Prevost MC, Xie Z, Matsuyama S, Reed JC, Kroemer G (1998) Bax and adenine nucleotide translocator cooperate in the mitochondrial control of apoptosis. Science 281:2027–31

    Article  PubMed  CAS  Google Scholar 

  78. Graham BH, Waymire KG, Cottrell B, Trounce IA, MacGregor GR, Wallace DC (1997) A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator. Nat Genet 16:226–34

    Article  PubMed  CAS  Google Scholar 

  79. Lebovitz RM, Zhang H, Vogel H, Cartwright J Jr, Dionne L, Lu N, Huang S, Matzuk MM (1996) Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice. Proc Natl Acad Sci USA 93:9782–7

    Article  PubMed  CAS  Google Scholar 

  80. Djouadi F, Brandt JM, Weinheimer CJ, Leone TC, González FJ, Kelly DP (1999) The role of the peroxisome proliferator-activated receptor alpha (PPAR alpha) in control of cardiac lipid metabolism. Prostaglandins Leukot Essent Fatty Acids 60:339–43.

    Article  PubMed  CAS  Google Scholar 

  81. Ibdah JA, Paul H, Zhao Y, Binford S, Salleng K, Cline M, Matern D, Bennett MJ, Rinaldo P, Strauss AW (2001) Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death. J Clin Invest 107:1403–9

    PubMed  CAS  Google Scholar 

  82. Wang J, Wilhelmsson H, Graff C, Li H, Oldfors A, Rustin P, Bruning JC, Kahn CR, Clayton DA, Barsh GS, Thoren P, Larsson NG (1999) Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression. Nat Genet 21:133–7

    Article  PubMed  CAS  Google Scholar 

  83. Puccio H, Simon D, Cossee M, Criqui-Filipe P, Tiziano F, Melki J, Hindelang C, Matyas R, Rustin P, Koenig M (2001) Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat Genet 27:181–6

    Article  PubMed  CAS  Google Scholar 

  84. Finck BN, Lehman JJ, Leone TC, Welch MJ, Bennett MJ, Kovacs A, Han X, Gross RW, Kozak R, Lopaschuk GD, Kelly DP (2002) The cardiac phenotype induced by PPARalpha overexpression mimics that caused by diabetes mellitus. J Clin Invest 109:121–30

    Article  PubMed  CAS  Google Scholar 

  85. Lehman JJ, Barger PM, Kovacs A, Saffitz JE, Medeiros DM, Kelly DP (2000) Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis. J Clin Invest 106:847–56

    Article  PubMed  CAS  Google Scholar 

  86. Kubota T, McTiernan CF, Frye CS, Demetris AJ, Feldman AM (1997) Cardiac-specific overexpression of tumor necrosis factor-alpha causes lethal myocarditis in transgenic mice. J Card Fail 3:117–24

    Article  PubMed  CAS  Google Scholar 

  87. Marín-García J, Ananthakrishnan R, Goldenthal MJ, Filiano JJ, Pérez-Atayde A. (1999) Mitochondrial dysfunction in skeletal muscle of children with cardiomyopathy. Pediatrics 103:456–9

    Article  PubMed  Google Scholar 

  88. Pollitt RJ (1995) Disorders of mitochondrial long-chain fatty acid oxidation. J Inherit Metab Dis 18:473–90

    Article  PubMed  CAS  Google Scholar 

  89. Saudubray JM, Martin D, de Lonlay P, Touati G, Poggi-Travert F, Bonnet D, Jouvet P, Boutron M, Slama A, Vianey-Saban C, Bonnefont JP, Rabier D, Kamoun P, Brivet M (1999) Recognition and management of fatty acid oxidation defects: A series of 107 patients. J Inherit Metab Dis 22:488–502

    Article  PubMed  CAS  Google Scholar 

  90. Shoffner JM, Wallace DC (1994) Oxidative phosphorylation diseases and mitochondrial DNA mutations: Diagnosis and treatment. Annu Rev Nutr 14:535–68

    Article  PubMed  CAS  Google Scholar 

  91. Lerman-Sagie T, Rustin P, Lev D, Yanoov M, Leshinsky-Silver E, Sagie A, Ben-Gal T, Munnich A (2001) Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone. J Inherit Metab Dis 24:28–34

    Article  PubMed  CAS  Google Scholar 

  92. Bonnefont JP, Demaugre F, Prip-Buus C, Saudubray JM, Brivet M, Abadi N, Thuillier L (1999) Carnitine palmitoyltransferase deficiencies. Mol Genet Metab 68:424–40

    Article  PubMed  CAS  Google Scholar 

  93. Brown-Harrison MC, Nada MA, Sprecher H, Vianey-Saban C, Farquhar J Jr, Gilladoga AC, Roe CR (1996) Very long chain acyl-CoA dehydrogenase deficiency: Successful treatment of acute cardiomyopathy. Biochem Mol Med 58:59–65

    Article  PubMed  CAS  Google Scholar 

  94. Doetschman T, Shull M, Kier A, Coffin JD (1993) Embryonic stem cell model systems for vascular morphogenesis and cardiac disorders. Hypertension 22:618–29

    PubMed  CAS  Google Scholar 

  95. Muller M, Fleischmann BK, Seibert S, Ji GJ, Endl E, Middeler G, Muller OJ, Schlenke P, Frese S, Wobus AM, Hescheler J, Katus HA, Franz WM (2000) Selection of ventricular-like cardiomyocytes from ES cells in vitro. FASEB J 14:2540–8

    Article  PubMed  CAS  Google Scholar 

  96. Beltrami AP, Barlucchi L, Torella D, Baker M, Limana F, Chimenti S, Kasahara H, Rota M, Musso E, Urbanek K, Leri A, Kajstura J, Nadal-Ginard B, Anversa P (2003) Adult cardiac stem cells are multi-potent and support myocardial regeneration. Cell 114:763–6

    Article  PubMed  CAS  Google Scholar 

  97. Geromel V, Cao A, Briane D, Vassy J, Rotig A, Rustin P, Coudert R, Rigaut JP, Munnich A, Taillandier E (2001) Mitochondria transfection by oligonucleotides containing a signal peptide and vectorized by cationic liposomes. Antisense Nucleic Acid Drug Dev 11:175–80

    Article  PubMed  CAS  Google Scholar 

  98. Taylor RW, Wardell TM, Lightowlers RN, Turnbull DM (2000) Molecular basis for treatment of mitochondrial myopathies. Neurol Sci 21:S909–12

    Article  PubMed  CAS  Google Scholar 

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(2005). An Introduction to Mitochondria and the Heart. In: Marín-García, J. (eds) Mitochondria and the Heart. Developments in Cardiovascular Medicine, vol 256. Springer, Boston, MA. https://doi.org/10.1007/0-387-25575-3_1

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