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Volumn 11, Issue 5, 2011, Pages 679-685

Why are there no proven therapies for genetic mitochondrial diseases?

Author keywords

Clinical trials; Coenzyme Q10; Dichloroacetate; Mitochondria; Mitochondrial diseases

Indexed keywords

ASCORBIC ACID; DICHLOROACETIC ACID; MENADIONE; PLACEBO; UBIDECARENONE;

EID: 79961209538     PISSN: 15677249     EISSN: 18728278     Source Type: Journal    
DOI: 10.1016/j.mito.2011.05.002     Document Type: Short Survey
Times cited : (22)

References (48)
  • 2
    • 0022545789 scopus 로고
    • Treatment of mitochondrial myopathy due to complex III deficiency with vitamins K3 and C: a 31P-NMR follow-up study
    • Arogov Z., Bank W.J., Maris J., Eleff S., Kennaway N.G., Olson R.E., Chance B. Treatment of mitochondrial myopathy due to complex III deficiency with vitamins K3 and C: a 31P-NMR follow-up study. Ann. Neurol. 1986, 19:598.
    • (1986) Ann. Neurol. , vol.19 , pp. 598
    • Arogov, Z.1    Bank, W.J.2    Maris, J.3    Eleff, S.4    Kennaway, N.G.5    Olson, R.E.6    Chance, B.7
  • 3
    • 13944278132 scopus 로고    scopus 로고
    • Mitochondrial, oxidants and aging
    • Balaban R.S., Nemoto S., Finkel T. Mitochondrial, oxidants and aging. Cell 2005, 120:483-495.
    • (2005) Cell , vol.120 , pp. 483-495
    • Balaban, R.S.1    Nemoto, S.2    Finkel, T.3
  • 5
    • 0038051345 scopus 로고    scopus 로고
    • Mitochondria, oxidative damage, and inflammation in Parkinson's disease
    • Beal M.F. Mitochondria, oxidative damage, and inflammation in Parkinson's disease. Ann NY Acad Sci 2003, 991:120-131.
    • (2003) Ann NY Acad Sci , vol.991 , pp. 120-131
    • Beal, M.F.1
  • 6
    • 33745055953 scopus 로고    scopus 로고
    • Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency
    • Berendzen K., Theriaque D., Shuster J., Stacpoole P.W. Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency. Mitochondrion 2006, 6:126-135.
    • (2006) Mitochondrion , vol.6 , pp. 126-135
    • Berendzen, K.1    Theriaque, D.2    Shuster, J.3    Stacpoole, P.W.4
  • 8
    • 0037180245 scopus 로고    scopus 로고
    • Accumulation of mitochondrial DNA mutations in ageing, cancer, and mitochondrial disease: is there a common mechanism?
    • Chinnery P.F., Samuels D.C., Elson J., Turnbull D.M. Accumulation of mitochondrial DNA mutations in ageing, cancer, and mitochondrial disease: is there a common mechanism?. Lancet 2002, 360(9342):1323-1325.
    • (2002) Lancet , vol.360 , Issue.9342 , pp. 1323-1325
    • Chinnery, P.F.1    Samuels, D.C.2    Elson, J.3    Turnbull, D.M.4
  • 11
    • 1942537035 scopus 로고    scopus 로고
    • Dichloroacetate therapy attenuates the blood lactate response to submaximal exercise in patients with defects in mitochondrial energy metabolism
    • Duncan G.E., Perkins L.A., Theriaque D.W., Neiberger R.E., Stacpoole P.W. Dichloroacetate therapy attenuates the blood lactate response to submaximal exercise in patients with defects in mitochondrial energy metabolism. J. Clin. Endocrinol. Metab. 2004, 89(4):1733-1738.
    • (2004) J. Clin. Endocrinol. Metab. , vol.89 , Issue.4 , pp. 1733-1738
    • Duncan, G.E.1    Perkins, L.A.2    Theriaque, D.W.3    Neiberger, R.E.4    Stacpoole, P.W.5
  • 12
    • 67949102053 scopus 로고    scopus 로고
    • Recent progress in the biology and physiology of sirtuins
    • Finkel T., Deng C.X., Mostoslavsky R. Recent progress in the biology and physiology of sirtuins. Nature 2009, 460(7255):587-591.
    • (2009) Nature , vol.460 , Issue.7255 , pp. 587-591
    • Finkel, T.1    Deng, C.X.2    Mostoslavsky, R.3
  • 16
    • 34248175243 scopus 로고    scopus 로고
    • The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease
    • Haas R.H. The evidence basis for coenzyme Q therapy in oxidative phosphorylation disease. Mitochondrion 2007, 7S:S136-S145.
    • (2007) Mitochondrion , vol.7 S
    • Haas, R.H.1
  • 17
    • 0023883150 scopus 로고
    • Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies
    • Holt I.J., Harding A.E., Morgan Huges J.A. Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies. Nature 1988, 321:717-719.
    • (1988) Nature , vol.321 , pp. 717-719
    • Holt, I.J.1    Harding, A.E.2    Morgan Huges, J.A.3
  • 20
    • 0029035721 scopus 로고
    • Treatment of lactic acidosis: a review
    • Kerr D.S. Treatment of lactic acidosis: a review. Int Pediatr 1995, 10:75-81.
    • (1995) Int Pediatr , vol.10 , pp. 75-81
    • Kerr, D.S.1
  • 22
    • 20144387513 scopus 로고    scopus 로고
    • Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo-controlled, double-blind 31P-MRS crossover study
    • Kornblum C., Schröder R., Müller K., Vorgerd M., Eggers J., Bogdanow M., Papassotiropoulos A., Fabian K., Klockgether T., Zange J. Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo-controlled, double-blind 31P-MRS crossover study. Eur. J. Neurol. 2005, 12(4):300-309.
    • (2005) Eur. J. Neurol. , vol.12 , Issue.4 , pp. 300-309
    • Kornblum, C.1    Schröder, R.2    Müller, K.3    Vorgerd, M.4    Eggers, J.5    Bogdanow, M.6    Papassotiropoulos, A.7    Fabian, K.8    Klockgether, T.9    Zange, J.10
  • 23
    • 79961209470 scopus 로고    scopus 로고
    • Phase 3 trial of coenzyme
    • Kurtz, TL, for the CoQ10 Trial Investigators, United Mitochondrial Disease Foundation, Indianapolis, IN, June 24-28
    • Phase 3 trial of coenzyme. Q10 in genetic mitochondrial diseases 2008, Kurtz, TL, for the CoQ10 Trial Investigators, United Mitochondrial Disease Foundation, Indianapolis, IN, June 24-28.
    • (2008) Q10 in genetic mitochondrial diseases
  • 25
    • 33646684516 scopus 로고    scopus 로고
    • New insights into structure and function of mitochondria and their role in aging and disease
    • Lenaz G., Baracca A., Fato R., Genova M.L., Solaini G. New insights into structure and function of mitochondria and their role in aging and disease. Antioxid. Redox Signal. 2006, 8(3-4):417-437.
    • (2006) Antioxid. Redox Signal. , vol.8 , Issue.3-4 , pp. 417-437
    • Lenaz, G.1    Baracca, A.2    Fato, R.3    Genova, M.L.4    Solaini, G.5
  • 26
    • 0037241026 scopus 로고    scopus 로고
    • The effect of short-term dimethylglycine treatment on oxygen consumption in cytochrome oxidase deficiency: a double-blind randomized crossover clinical trial
    • Liet J.M., Pelletier V., Robinson B.H., Laryea M.D., Wendel U., Morneau S., Morin C., Mitchell G., Lacroix J. The effect of short-term dimethylglycine treatment on oxygen consumption in cytochrome oxidase deficiency: a double-blind randomized crossover clinical trial. J. Pediatr. 2003, 142(1):62-66.
    • (2003) J. Pediatr. , vol.142 , Issue.1 , pp. 62-66
    • Liet, J.M.1    Pelletier, V.2    Robinson, B.H.3    Laryea, M.D.4    Wendel, U.5    Morneau, S.6    Morin, C.7    Mitchell, G.8    Lacroix, J.9
  • 30
    • 0141610413 scopus 로고
    • Muscle coenzyme Q deficiency in familial mitochondrial encephalopathy
    • Ogasahara S., Engel A.G., Frens D., Mack D. Muscle coenzyme Q deficiency in familial mitochondrial encephalopathy. Proc Natl Acad Sci USA 1989, 86:2379-2382.
    • (1989) Proc Natl Acad Sci USA , vol.86 , pp. 2379-2382
    • Ogasahara, S.1    Engel, A.G.2    Frens, D.3    Mack, D.4
  • 31
    • 0029066771 scopus 로고
    • The treatment of mitochondrial myopathies and encephalomyopathies
    • Peterson P.L. The treatment of mitochondrial myopathies and encephalomyopathies. Biochim. Biophys. Acta 1995, 1271:275-280.
    • (1995) Biochim. Biophys. Acta , vol.1271 , pp. 275-280
    • Peterson, P.L.1
  • 35
    • 0027436331 scopus 로고
    • Rules of evidence and clinical recommendations for the management of patients
    • Sackett D.L. Rules of evidence and clinical recommendations for the management of patients. Can. J. Cardiol. 1993, 9:487-489.
    • (1993) Can. J. Cardiol. , vol.9 , pp. 487-489
    • Sackett, D.L.1
  • 36
  • 37
    • 79955826967 scopus 로고    scopus 로고
    • The dichloroacetate dilemma: environmental hazard vs. therapeutic goldmine - both or neither?
    • Stacpoole P.W. The dichloroacetate dilemma: environmental hazard vs. therapeutic goldmine - both or neither?. Environ. Health Perspect. 2011, 119(2):155-158.
    • (2011) Environ. Health Perspect. , vol.119 , Issue.2 , pp. 155-158
    • Stacpoole, P.W.1
  • 42
    • 0024452528 scopus 로고
    • The pharmacology of dichloroacetate
    • Stacpoole P.W. The pharmacology of dichloroacetate. Metabolism 1989, 38:1124-1144.
    • (1989) Metabolism , vol.38 , pp. 1124-1144
    • Stacpoole, P.W.1
  • 44
    • 0030731246 scopus 로고    scopus 로고
    • A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies
    • Tarnopolsky M.A., Roy B.D., MacDonald J.R. A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies. Muscle Nerve 1997, 20(12):1502-1509.
    • (1997) Muscle Nerve , vol.20 , Issue.12 , pp. 1502-1509
    • Tarnopolsky, M.A.1    Roy, B.D.2    MacDonald, J.R.3
  • 45
    • 85009226418 scopus 로고    scopus 로고
    • A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease
    • The Huntington Study Group
    • The Huntington Study Group A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001, 57:397-404.
    • (2001) Neurology , vol.57 , pp. 397-404
  • 46
    • 77956802394 scopus 로고    scopus 로고
    • Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects
    • The Huntington Study Group
    • The Huntington Study Group Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects. Mov. Disord. 2010, 25(12):1924-1928.
    • (2010) Mov. Disord. , vol.25 , Issue.12 , pp. 1924-1928
  • 47
    • 1642287689 scopus 로고    scopus 로고
    • Metabolism and function of coenzyme Q
    • Turunen M., Olsson Dallner G. Metabolism and function of coenzyme Q. Biochim. Biophys. Acta 2004, 1660:171-199.
    • (2004) Biochim. Biophys. Acta , vol.1660 , pp. 171-199
    • Turunen, M.1    Olsson, D.G.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.