Mitochondrial medicine represents a complex of clinical, biochemical, pathological and genetic information crucial in diagnosis and treatment. An outline of the development of mitochondrial medicine was for the first time published by Luft in 1994 [22]. Several organizations are focused on mitochondrial medicine, from experimental and clinical research (Mitochondrial Research Society – MRS) to patients application (Mitochondrial Medicine Society –MMS), education and family oriented (United Mitochondrial Diseases Foundation – UMDF), and others. Knowledge concerning mitochondrial DNA (mtDNA) changes in several mitochondrial diseases were published recently [10].
This book presents mitochondrial medicine from the viewpoint of several preclinical studies on chronobiology, aging, Alzheimer’s disease, Huntington’s disease, diabetes, supplementary therapy with CoQ10, carnitine, alpha-lipoic acid, n-3, n-6 PUFA, and it provides information on clinical application of mitochondrial medicine in cardiology, diabetology, nephrology, immunology, and andrology.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsPreview
Unable to display preview. Download preview PDF.
References
Aliev G, Smith MA, Torre JC, Perry G (2004) Mitochondria as a primary target for vascular hypoperfusion and oxidative stress in Alzheimer’s disease. Mitochondrion 4:649–663
Andreson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Derouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG (1981) Sequence and organization of the human mitochondrial genome. Nature 290:457–465
Beal MF (2005) Mitochondria take center stage in ageing and neurodegeneration. Ann Neurol 58(4):495–505
Bota DA, Davies KJA (2001) Protein degradation in mitochondria: implications for oxidative stress, ageing and diseases: a novel etiological classification of mitochondrial proteolytic disorders (review). Mitochondrion 1:33–49
Browne SE, Beal MF (2004) The energetics of Huntington’s disease. Neurochem Res 29(3):531–546
Browne SE, Ferrante RJ, Beal MF (1999) Oxidative stress in Huntington’s disease. Brain Pathol 9:147–163
Cooper JM, Schapira AH (2003) Friedrich’s ataxia: disease mechanisms, antioxidant and coenzyme Q10 therapy. Biofactors 18:163–171
Dauer W, Przedborski S (2003) Parkinson’s disease: mechanisms and models. Neuron 39:889–909
DiMauro S., DiMauro PM (1973) Muscle carnitine palmitoyl-trransferase deficiency and myoglobinuria. Science 182:929–931
DiMauro, Hirano M., Schon EA (2006). Mitochondrial Medicine. Informa Healthcare 2006, pp 348
Engel AG, Angelini C (1973) Carnitine deficiency of human skeletal muscle with associated lipid storage myopathy: a new syndrome. Science 179:899–902
Epstein CJ (1995) Down’s syndrome (trisomy 21). In: Scriver CR, Beaudet AL, Sly WS et al. (eds) The Metabolic and Molecular Bases of Inherited Diseases. McGraw-Hill, New York
Fraser PE, Yang DS, Yu G, Lovesque L, Nishimura M, Arawaka S, Serpell LC, Rogaeva E, Hyslop PG (2000) Presenilin structure, function and role in Alzheimer’s disease. Biochim Biophys Acta 1502:1–15
Gardian G, Vecsei L (2004) Huntington’s disease: pathomechanism and therapeutic perspectives. J Neural Transm 111:1485–1494
Haas RH, Nasirian F, Nakano K et al. (1989) Low platelet mitochondrial complex I and complex II/III activity in early untreated Parkinson’s disease. Ann Neurol 37:714–722
Hayakawa M, Torii K, Sugiyama S, Tanaka M, Ozawa T (1991) Age-associated accumulation of 8-hydroxydeoxyquanosine in mitochondrial DNA of human diaphragm. Biochem Biophys Res Commun 179(2):1023–1029
Hayakawa M, Katsumata K, Yoneda M, Tanaka M, Sugyiama S, Ozawa T (1996) Aged-related extensive fragmentation of mitochondrial DNA into minicircles. Biochem Biophys Res Commun 226(2):369–377
Jope R, Blass JP (1975) A comparison of the regulation of pyruvate dehydrogenase in mitochondria from rat brain and liver. Biochem J 150:397–403
Kašparová S, Sumbalová Z, Bystricky P, Kucharská J, Liptaj T, Mlynárik V, Gvozdjáková A (2006) Effect of coenzyme Q10 and vitamin E on brain energy metabolism in the animal model of Huntington’s disease. Neurochem Int 48:93–99
Kim SH, Fountoulakis M, Dierssen M, Lubec G (2001) Decreased protein levels of complex I 30-kDa subunit in fetal Down’s syndrome brains. J Neural Transm Suppl 61:109–116
Kunz WS, Kuznetsov AV, Clark JF, Tracey I, Elger CE (1999) Metabolic consequences of the cytochrome c oxidase deficiency in brain of copper-deficient Mo(vbr) mice. J Neurochem 72:1580–1585
Luft R (1994) The development of mitochondrial medicine. Proc Natl Acad Sci USA 91:9831–9838
Luft R, Landau BR (1995) Mitochondrial medicine. J Intern Med 238:405–421
Luft R, Ikkos D, Palmieri G, Ernster L, Afzelius B (1962) A case of severe hypermetabolism of nonthyroid origin with the defect in the maintenance of mitochondrial respiratory control: a correlated clinical, biochemical and morphological study. J Clin Invest 41:1776–1804
Miquel J, Fleming JE (1984) A two-step hypothesis on the mechanism of in vitro cell ageing: cell differentiation followed by intrinsic mitochondrial mutagenesis. Exp Gerontol 19:31–36
Muchová J, Šustrová M, Garaiová I, Liptáková A, Blažíček P, Kvasnička P, Pueschel S, Duračková Z (2001) Influence of age on activities of antioxidant enzymes and lipid peroxidation products in erythrocytes and neutrophils of Down’s syndrome patients. Free Radic Biol Med 31(4):499–508
Muller-Hocker J (1989) Cytochrome – c –oxidase deficient cardiomyocytes in the human heart–an age-related phenomenon. A histochemical ultracytochemical study. Am J Pathol 134(5):1167–1173
Naviaux RK (2004) Developing a systematic approach to the diagnosis and classification of mitochondrial disease. Mitochondrion 4:351–361
Pereira C, Gazila MM, Oliviera CR (2001) β-amyloid protein impairs mitochondrial function. In: Ebadi M, Nearwah J, Chopra RK (eds) Mitochondrial Ubiquinone, Vol. 2. pp 281–300
Reddy PH (2006) Mitochondrial oxidative damage in ageing and Alzheimer’s disease: Implications for mitochondrially targeted antioxidant therapeutics (review article). J Biomed Biotechnol 31372:1–13
Reddy PH, Beal MF (2005) Are mitochondria critical in the pathogenesis of Alzheimer’s disease? Brain Research. Brain Res Rev 49(3):618–632
Reed JC (2002) Apoptosis-based therapies. Nat Rev/Drug Discov 1:111–121
Remacle J, Lambert D, Raes M, Pigeolet E, Michiels C, Toussaint D (1992) Importance of various antioxidant enzymes for cell stability. Biochem J 286:41–46
Selkoe DJ (2001) Alzheimer’s disease: genes, proteins, and therapy. Physiol Rev 81(2):741–766
Shults CW (2004) Mitochondrial dysfunction and possible treatments in Parkinson’s disease– a review. Mitochondrion 4:641–648
Shults CW, Oakes D, Kieburtz K et al. (2002) Effects of coenzyme Q10 in early Parkinson’s disease: evidence of slowing of the functional decline. Arch Neurol 59:1541–1550
Smith MA, Rottkamp CA, Nunomura A, Raina AK, Perry G (2002) Oxidative stress in Alzheimer’s disease. (Review). Biochim Biophys Acta 1502:139–144
Spiro AJ, Moore CL, Prineas JW, Strasberg PM, Rapin I (1970) A cytochrome-related inherited disorder of the nervous system and muscle. Arch Neurol 23:103–112
Van Gurp M, Festjens N, Van Loo G, Saelens S, Vandenabeele P (2003) Mitochondrial intermembrane proteins in cell death. Biochem Biophys Res Commun 304:487–497
Vielhalber S, Kaufmann J, Kanowski M et al. (2001) Effect of creatine supplementation on metabolite levels in ALS motor cortices. Exp Neurol 172:377–382
Vila M, Przedborski S (2004) Genetic clues to the pathogenesis of Parkinson’s disease. Nat Med 10(Suppl):S58–S62
Wallace DC, Singh G, Lott MT, Hodge JA, Schurr TG, Lezza AM, Elsas LJ, Nicoskelainen EK (1988) Mitochondrial DNA mutation associated with Leber’s hereditary optic neuropathy. Science 24:21427–21430
Zhang C, Linnane AW, Nagley P (1993) Occurrence of a particular base substitution (3243 A to G) in mitochondrial DNA of tissues of ageing humans. Biochem Biophys Res Commun 195(2):1104–1110
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Springer Science + Business Media B.V
About this chapter
Cite this chapter
Gvozdjáková, A. (2008). Mitochondrial Medicine. In: Gvozdjáková, A. (eds) Mitochondrial Medicine. Springer, Dordrecht. https://doi.org/10.1007/978-1-4020-6714-3_5
Download citation
DOI: https://doi.org/10.1007/978-1-4020-6714-3_5
Publisher Name: Springer, Dordrecht
Print ISBN: 978-1-4020-6713-6
Online ISBN: 978-1-4020-6714-3
eBook Packages: MedicineMedicine (R0)