The mitochondria belong to the functionally and metabolically most important subcellular organelles, playing a complex multifactorial role in the cell. They are involved in final oxidation of main energy sources with formation of reduced coenzymes, which deliver their electrons to the electron transport chain of the mitochondrial inner membrane. The process of electron transport is realized by some enzymes and other carriers and is associated with the formation of ATP (oxidative phosphorylation pathway). They are also involved in further processes of the cell– they produce the greatest portion of reactive oxygen species, take part in apoptosis and affect many other important cellular functions.
A defect of mitochondrial structure and function is the cause of many diseases or disturbances in the whole human organism. Various functional failures due to individual mitochondrial defects are known. Many are genetically dependent or acquired in connection with different pathological events. Genetically dependent defects are often demonstrated in several organs or tissues. The most afflicted organs are those with very extensive energy metabolism – brain, skeletal muscle and heart. Therefore, encephalopathies and myopathies are very often associated with cardiomyopathies, especially in genetic-dependent mitochondrial defects. Acquired mitochondrial defects concern ischemia-reperfusion injury and other events associated with ischemia or anoxia. In this review some of these mitochondrial defects, especially those associated with cardiomyopathy are discussed.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Anderson S, Bankier AJ, Barrell BG et al. (1981) Sequence and organization of the human mitochondrial genome. Nature 290:457–465
Bhagavan HN, Chopra RK (2005) Potential role of ubiquinone (coenzyme Q10) in pediatric cardiomyopathy (review). Clin Nutr 24:331–338
Bonnet D, Martin D, De Lonlay P et al. (1999) Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. Circulation 100:2248–2253
Brilla C, Janicki JS, Weber KT (1991) Impaired diastolic function and coronary reserve in genetic hypertension. Role of interstitial fibrosis and medial thickening of intramyocardial coronary arteries. Circ Res 69:107–115
Cai L, Kang YJ (2003) Cell death and diabetic cardiomyopathy. Cardiovasc Toxicol 3:219–228
Cai L, Wang Y, Zhou G et al. (2006) Attenuation by metallothionein of early cardiac cell death via suppression of mitochondrial oxidative stress results in a prevention of diabetic cardiomyopathy. J Am Coll Cardiol 48:1688–1697
Carvajal K, Moreno-Sanchéz R (2003) Heart metabolic disturbances in cardiovascular diseases. Arch Med Res 34:89–99
Casademont J, Miro O (2002) Electron transport chain defects in heart failure. Heart Fail Rev 7:131–139
Das DK, Maulik N (2005) Mitochondrial function in cardiomyocytes: target for cardioprotection. Curr Opin Anaesthesiol 18:77–82
Di Lisa F, Bernardi P (2006) Mitochondria and ischemia-reperfusion injury of the heart: fixing a hole. Cardiovasc Res 70:191–199
DiMauro S, Schon EA (2001) Mitochondrial DNA mutations in human disease. Am J Med Genet 106:18–26
Feigenbaum A, Bai RK, Doherty ES et al. (2006) Novel mitochondrial DNA mutations associated with myopathy, cardiomyopathy, renal failure, and deafness. Am J Med Genet A 140:2216–2222
Fosslien E (2001) Mitochondrial medicine–molecular pathology of defective oxidative phosphorylation. Ann Clin Lab Sci 31:25–67
Fosslien E (2003) Mitochondrial medicine–cardiomyopathy caused by defective oxidative phosphorylation. Ann Clin Lab Sci 33:371–395
Guenthard J, Wyler F, Fowler B et al. (1995) Cardiomyopathy in respiratory chain disorders. Arch Dis Child 72:223–226
Gvozdják J (1973) Non-coronary Cardiomyopathies (in Slovak). Slovak Academy of Sciences Publishing House, Bratislava pp 14–28
Gvozdják J, Gvozdjáková A (1980) The Heart Muscle (in Slovak). Osveta, Martin, Slovakia, pp 331
Holmgren D, Wählander H, Eriksson BO et al. (2003) Cardiomyopathy in children with mitochondrial disease. Clinical course and cardiological findings. Eur Heart J 24:280–288
Laloi-Michelin M, Virally M, Jardel C et al. (2006) Kearns Sayre syndrome: an unusual form of mitochondrial diabetes. Diabetes Metab 32:182–186
Larsson NG, Oldfors A (2001) Mitochondrial myopathies. Acta Physiol Scand 171:383–393
Lesnefsky EJ, Moghaddas S, Tandler B et al. (2001) Mitochondrial dysfunction in cardiac disease: ischemia-reperfusion, aging, and heart failure. J Mol Cell Cardiol 33:1065–1089
Lopes R (2006) Correlation of mitochondrial protein expression in complexes I to V with natural and induced forms of canine idiopathic dilated cardiomyopathy. Am J Vet Res 67:971–977
Lopes R, Solter PF, Sisson DD et al. (2006) Characterization of canine mitochondrial protein expression in natural and induced forms of idiopathic dilated cardiomyopathy. Am J Vet Res 67:963–970
Marin-Garcia J, Goldenthal MJ, Moe GW (2001) Abnormal cardiac and skeletal muscle mitochondrial function in pacing-induced failure. Cardiovasc Res 52:103–110
Marin-Garcia J, Goldenthal MJ (2002) Understanding the impact of mitochondrial defects in cardiovascular disease: a review. J Card Fail 8:347–361
Nojiri H, Shimizu T, Funakoshi M et al. (2006) Oxidative stress causes heart failure with impaired mitochondrial respiration. J Biol Chem 281:33789–33801
Olivetti G, Melissari M, Capasso JM, Anversa P (1991) Cardiomyopathy of the ageing human heart. Myocyte loss and cellular hypertrophy. Circ Res 68:1560–1568
Opie LH (1997) The Heart. Physiology from Cell to Circulation, IIIrd edn. Lippincott-Raven, Philadelphia, New York, pp 45–45
Rendeková V, Pecháň I (1999) Some aspects of myocardial energy metabolism. I. The normoxic myocardium (in Slovak). Cardiology 8:191–195
Shen X, Zheng S, Thongboonkerd V et al. (2004) Cardiac mitochondrial damage and biogenesis in a chronic model of type 1 diabetes. Am J Physiol Endocrinol Metab 287:E896–905
Solaini G, Harris DA (2005) Biochemical dysfunction in heart mitochondria exposed to ischaemia and reperfusion. Biochem J 390:377–394
Stanley CA (1987) New genetic defects in mitochondrial mfatty acid oxidation and carnitine deficiency. Adv Pediatr 34:59–88
Stanley CA, Hale DF (1984) Genetic disorders of mitochondria fatty acid oxidation. Curr Opin Pediatr 6:476–481
Thorburn DR (2004) Mitochondrial disorders: prevalence, myths and advances. J Inherit Metab Dis 27:349–362
Thorburn DR, Sugiana C, Salemi R et al. (2004) Biochemical and molecular diagnosis of mitochondrial respiratory chain disorders. Biochim Biophys Acta 1659:121–128
Tsutsui H (2004) Novel pathophysiological insight and treatment strategies for heart failure–lessons from mice and patients. Circ J 68:1095–1103
Vyatkina G, Bhatia V, Gerstner A et al. (2004) Impaired mitochondrial respiratory chain and bioenergetics during chagasic cardiomyopathy development. Biochim Biophys Acta 1689:162–173
Wallace DC (1999) Mitochondrial diseases in man and mouse. Science 283:1482–1488
Wallace DC (2000) Mitochondrial defects in cardiomyopathy and neuromuscular diseases. Am Heart J 138:S70–S85
Wen JJ, Yachelini PC, Sembaj A et al. (2006) Increased oxidative stress is correlated with mitochondrial dysfunction in chagasic patients. Free Radic Biol Med 41:270–276
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Springer Science + Business Media B.V
About this chapter
Cite this chapter
Pecháň, I. (2008). Mitochondrial Cardiology. In: Gvozdjáková, A. (eds) Mitochondrial Medicine. Springer, Dordrecht. https://doi.org/10.1007/978-1-4020-6714-3_6
Download citation
DOI: https://doi.org/10.1007/978-1-4020-6714-3_6
Publisher Name: Springer, Dordrecht
Print ISBN: 978-1-4020-6713-6
Online ISBN: 978-1-4020-6714-3
eBook Packages: MedicineMedicine (R0)