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Volumn 7, Issue 7, 2012, Pages

Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex iii deficiency due to a bcs1l mutation

Author keywords

[No Author keywords available]

Indexed keywords

ACYLCARNITINE; ADENOSINE PHOSPHATE; AMINO ACID DERIVATIVE; BIOGENIC AMINE; CARBOHYDRATE; FUMARIC ACID; PUTRESCINE; SUCCINIC ACID;

EID: 84864039418     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0041156     Document Type: Article
Times cited : (29)

References (48)
  • 1
    • 79952187160 scopus 로고    scopus 로고
    • Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies
    • Ghezzi D, Arzuffi P, Zordan M, Da Re C, Lamperti C, et al. (2011) Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies. Nat Genet 43: 259-263.
    • (2011) Nat Genet , vol.43 , pp. 259-263
    • Ghezzi, D.1    Arzuffi, P.2    Zordan, M.3    Da Re, C.4    Lamperti, C.5
  • 2
    • 0033214782 scopus 로고    scopus 로고
    • Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex
    • Cruciat CM, Hell K, Folsch H, Neupert W, Stuart RA, (1999) Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex. EMBO J 18: 5226-5233.
    • (1999) EMBO J , vol.18 , pp. 5226-5233
    • Cruciat, C.M.1    Hell, K.2    Folsch, H.3    Neupert, W.4    Stuart, R.A.5
  • 3
    • 0026702012 scopus 로고
    • BCS1, a novel gene required for the expression of functional Rieske iron-sulfur protein in Saccharomyces cerevisiae
    • Nobrega FG, Nobrega MP, Tzagoloff A, (1992) BCS1, a novel gene required for the expression of functional Rieske iron-sulfur protein in Saccharomyces cerevisiae. EMBO J 11: 3821-3829.
    • (1992) EMBO J , vol.11 , pp. 3821-3829
    • Nobrega, F.G.1    Nobrega, M.P.2    Tzagoloff, A.3
  • 4
    • 1642404614 scopus 로고    scopus 로고
    • Further insights into the assembly of the yeast cytochrome bc1 complex based on analysis of single and double deletion mutants lacking supernumerary subunits and cytochrome b
    • Zara V, Palmisano I, Conte L, Trumpower BL, (2004) Further insights into the assembly of the yeast cytochrome bc1 complex based on analysis of single and double deletion mutants lacking supernumerary subunits and cytochrome b. Eur J Biochem 271: 1209-1218.
    • (2004) Eur J Biochem , vol.271 , pp. 1209-1218
    • Zara, V.1    Palmisano, I.2    Conte, L.3    Trumpower, B.L.4
  • 5
    • 56649095917 scopus 로고    scopus 로고
    • Respiratory-chain diseases related to complex III deficiency
    • Benit P, Lebon S, Rustin P, (2009) Respiratory-chain diseases related to complex III deficiency. Biochim Biophys Acta 1793: 181-185.
    • (2009) Biochim Biophys Acta , vol.1793 , pp. 181-185
    • Benit, P.1    Lebon, S.2    Rustin, P.3
  • 6
    • 79959861353 scopus 로고    scopus 로고
    • Mitochondrial disorders caused by mutations in respiratory chain assembly factors
    • Diaz F, Kotarsky H, Fellman V, Moraes CT, (2011) Mitochondrial disorders caused by mutations in respiratory chain assembly factors. Semin Fetal Neonatal Med 16: 197-204.
    • (2011) Semin Fetal Neonatal Med , vol.16 , pp. 197-204
    • Diaz, F.1    Kotarsky, H.2    Fellman, V.3    Moraes, C.T.4
  • 7
    • 33847153121 scopus 로고    scopus 로고
    • Missense mutations in the BCS1L gene as a cause of the Bjornstad syndrome
    • Hinson JT, Fantin VR, Schonberger J, Breivik N, Siem G, et al. (2007) Missense mutations in the BCS1L gene as a cause of the Bjornstad syndrome. N Engl J Med 356: 809-819.
    • (2007) N Engl J Med , vol.356 , pp. 809-819
    • Hinson, J.T.1    Fantin, V.R.2    Schonberger, J.3    Breivik, N.4    Siem, G.5
  • 8
    • 0033619147 scopus 로고    scopus 로고
    • Exercise intolerance due to mutations in the cytochrome b gene of mitochondrial DNA
    • Andreu AL, Hanna MG, Reichmann H, Bruno C, Penn AS, et al. (1999) Exercise intolerance due to mutations in the cytochrome b gene of mitochondrial DNA. N Engl J Med 341: 1037-1044.
    • (1999) N Engl J Med , vol.341 , pp. 1037-1044
    • Andreu, A.L.1    Hanna, M.G.2    Reichmann, H.3    Bruno, C.4    Penn, A.S.5
  • 10
    • 17944381521 scopus 로고    scopus 로고
    • A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
    • de Lonlay P, Valnot I, Barrientos A, Gorbatyuk M, Tzagoloff A, et al. (2001) A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure. Nat Genet 29: 57-60.
    • (2001) Nat Genet , vol.29 , pp. 57-60
    • de Lonlay, P.1    Valnot, I.2    Barrientos, A.3    Gorbatyuk, M.4    Tzagoloff, A.5
  • 11
    • 19044365959 scopus 로고    scopus 로고
    • GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L
    • Visapaa I, Fellman V, Vesa J, Dasvarma A, Hutton JL, et al. (2002) GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Am J Hum Genet 71: 863-876.
    • (2002) Am J Hum Genet , vol.71 , pp. 863-876
    • Visapaa, I.1    Fellman, V.2    Vesa, J.3    Dasvarma, A.4    Hutton, J.L.5
  • 12
    • 0032515555 scopus 로고    scopus 로고
    • Iron-overload disease in infants involving fetal growth retardation, lactic acidosis, liver haemosiderosis, and aminoaciduria
    • Fellman V, Rapola J, Pihko H, Varilo T, Raivio KO, (1998) Iron-overload disease in infants involving fetal growth retardation, lactic acidosis, liver haemosiderosis, and aminoaciduria. Lancet 351: 490-493.
    • (1998) Lancet , vol.351 , pp. 490-493
    • Fellman, V.1    Rapola, J.2    Pihko, H.3    Varilo, T.4    Raivio, K.O.5
  • 13
    • 79251499574 scopus 로고    scopus 로고
    • The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy
    • Leveen P, Kotarsky H, Morgelin M, Karikoski R, Elmer E, et al. (2011) The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy. Hepatology 53: 437-447.
    • (2011) Hepatology , vol.53 , pp. 437-447
    • Leveen, P.1    Kotarsky, H.2    Morgelin, M.3    Karikoski, R.4    Elmer, E.5
  • 14
    • 32844458032 scopus 로고    scopus 로고
    • Mitochondrial dysfunction in NASH: causes, consequences and possible means to prevent it
    • Begriche K, Igoudjil A, Pessayre D, Fromenty B, (2006) Mitochondrial dysfunction in NASH: causes, consequences and possible means to prevent it. Mitochondrion 6: 1-28.
    • (2006) Mitochondrion , vol.6 , pp. 1-28
    • Begriche, K.1    Igoudjil, A.2    Pessayre, D.3    Fromenty, B.4
  • 15
    • 84655169624 scopus 로고    scopus 로고
    • Role of oxidative stress in the pathogenesis of nonalcoholic steatohepatitis
    • Rolo AP, Teodoro JS, Palmeira CM, (2012) Role of oxidative stress in the pathogenesis of nonalcoholic steatohepatitis. Free Radic Biol Med 52: 59-69.
    • (2012) Free Radic Biol Med , vol.52 , pp. 59-69
    • Rolo, A.P.1    Teodoro, J.S.2    Palmeira, C.M.3
  • 16
    • 84856787016 scopus 로고    scopus 로고
    • Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV
    • Diaz F, Enriquez JA, Moraes CT, (2012) Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV. Mol Cell Biol 32: 415-429.
    • (2012) Mol Cell Biol , vol.32 , pp. 415-429
    • Diaz, F.1    Enriquez, J.A.2    Moraes, C.T.3
  • 17
    • 77955059876 scopus 로고    scopus 로고
    • Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency
    • Moran M, Marin-Buera L, Gil-Borlado MC, Rivera H, Blazquez A, et al. (2010) Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency. Hum Mutat 31: 930-941.
    • (2010) Hum Mutat , vol.31 , pp. 930-941
    • Moran, M.1    Marin-Buera, L.2    Gil-Borlado, M.C.3    Rivera, H.4    Blazquez, A.5
  • 19
    • 33645828659 scopus 로고    scopus 로고
    • Lipid metabolism and liver inflammation. II. Fatty liver disease and fatty acid oxidation
    • Reddy JK, Rao MS, (2006) Lipid metabolism and liver inflammation. II. Fatty liver disease and fatty acid oxidation. Am J Physiol Gastrointest Liver Physiol 290: G852-858.
    • (2006) Am J Physiol Gastrointest Liver Physiol , vol.290
    • Reddy, J.K.1    Rao, M.S.2
  • 20
    • 4043077961 scopus 로고    scopus 로고
    • Molecular mediators of hepatic steatosis and liver injury
    • Browning JD, Horton JD, (2004) Molecular mediators of hepatic steatosis and liver injury. J Clin Invest 114: 147-152.
    • (2004) J Clin Invest , vol.114 , pp. 147-152
    • Browning, J.D.1    Horton, J.D.2
  • 21
    • 1542297737 scopus 로고    scopus 로고
    • A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts
    • Hansson A, Hance N, Dufour E, Rantanen A, Hultenby K, et al. (2004) A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts. Proc Natl Acad Sci U S A 101: 3136-3141.
    • (2004) Proc Natl Acad Sci U S A , vol.101 , pp. 3136-3141
    • Hansson, A.1    Hance, N.2    Dufour, E.3    Rantanen, A.4    Hultenby, K.5
  • 23
    • 79959869854 scopus 로고    scopus 로고
    • Mitochondrial hepatopathies in the newborn period
    • Fellman V, Kotarsky H, (2011) Mitochondrial hepatopathies in the newborn period. Semin Fetal Neonatal Med 16: 222-228.
    • (2011) Semin Fetal Neonatal Med , vol.16 , pp. 222-228
    • Fellman, V.1    Kotarsky, H.2
  • 24
    • 34848857902 scopus 로고    scopus 로고
    • Metabolomics (liver and blood profiling) in a mouse model in response to fasting: a study of hepatic steatosis
    • van Ginneken V, Verhey E, Poelmann R, Ramakers R, van Dijk KW, et al. (2007) Metabolomics (liver and blood profiling) in a mouse model in response to fasting: a study of hepatic steatosis. Biochim Biophys Acta 1771: 1263-1270.
    • (2007) Biochim Biophys Acta , vol.1771 , pp. 1263-1270
    • van Ginneken, V.1    Verhey, E.2    Poelmann, R.3    Ramakers, R.4    van Dijk, K.W.5
  • 25
    • 66749192576 scopus 로고    scopus 로고
    • Role of phospholipids in respiratory cytochrome bc(1) complex catalysis and supercomplex formation
    • Wenz T, Hielscher R, Hellwig P, Schagger H, Richers S, et al. (2009) Role of phospholipids in respiratory cytochrome bc(1) complex catalysis and supercomplex formation. Biochim Biophys Acta 1787: 609-616.
    • (2009) Biochim Biophys Acta , vol.1787 , pp. 609-616
    • Wenz, T.1    Hielscher, R.2    Hellwig, P.3    Schagger, H.4    Richers, S.5
  • 26
    • 24944584210 scopus 로고    scopus 로고
    • Mitochondrial dysfunction induces triglyceride accumulation in 3T3-L1 cells: role of fatty acid beta-oxidation and glucose
    • Vankoningsloo S, Piens M, Lecocq C, Gilson A, De Pauw A, et al. (2005) Mitochondrial dysfunction induces triglyceride accumulation in 3T3-L1 cells: role of fatty acid beta-oxidation and glucose. J Lipid Res 46: 1133-1149.
    • (2005) J Lipid Res , vol.46 , pp. 1133-1149
    • Vankoningsloo, S.1    Piens, M.2    Lecocq, C.3    Gilson, A.4    de Pauw, A.5
  • 27
    • 84855987831 scopus 로고    scopus 로고
    • Reductive carboxylation supports growth in tumour cells with defective mitochondria
    • Mullen AR, Wheaton WW, Jin ES, Chen PH, Sullivan LB, et al. (2012) Reductive carboxylation supports growth in tumour cells with defective mitochondria. Nature 481: 385-388.
    • (2012) Nature , vol.481 , pp. 385-388
    • Mullen, A.R.1    Wheaton, W.W.2    Jin, E.S.3    Chen, P.H.4    Sullivan, L.B.5
  • 28
    • 2442649129 scopus 로고    scopus 로고
    • Citric acid cycle intermediates as ligands for orphan G-protein-coupled receptors
    • He W, Miao FJ, Lin DC, Schwandner RT, Wang Z, et al. (2004) Citric acid cycle intermediates as ligands for orphan G-protein-coupled receptors. Nature 429: 188-193.
    • (2004) Nature , vol.429 , pp. 188-193
    • He, W.1    Miao, F.J.2    Lin, D.C.3    Schwandner, R.T.4    Wang, Z.5
  • 29
    • 79951889242 scopus 로고    scopus 로고
    • Spermidine and resveratrol induce autophagy by distinct pathways converging on the acetylproteome
    • Morselli E, Marino G, Bennetzen MV, Eisenberg T, Megalou E, et al. (2011) Spermidine and resveratrol induce autophagy by distinct pathways converging on the acetylproteome. J Cell Biol 192: 615-629.
    • (2011) J Cell Biol , vol.192 , pp. 615-629
    • Morselli, E.1    Marino, G.2    Bennetzen, M.V.3    Eisenberg, T.4    Megalou, E.5
  • 30
    • 84864036447 scopus 로고    scopus 로고
    • Metabolomic analysis of sulfur-containing substances and polyamines in regenerating rat liver
    • Jung YS, Kim SJ, Kwon DY, Kim YC, (2011) Metabolomic analysis of sulfur-containing substances and polyamines in regenerating rat liver. Amino Acids.
    • (2011) Amino Acids
    • Jung, Y.S.1    Kim, S.J.2    Kwon, D.Y.3    Kim, Y.C.4
  • 31
    • 24144444133 scopus 로고    scopus 로고
    • Oxygen sensing requires mitochondrial ROS but not oxidative phosphorylation
    • Brunelle JK, Bell EL, Quesada NM, Vercauteren K, Tiranti V, et al. (2005) Oxygen sensing requires mitochondrial ROS but not oxidative phosphorylation. Cell Metab 1: 409-414.
    • (2005) Cell Metab , vol.1 , pp. 409-414
    • Brunelle, J.K.1    Bell, E.L.2    Quesada, N.M.3    Vercauteren, K.4    Tiranti, V.5
  • 32
    • 77956713136 scopus 로고    scopus 로고
    • Hypoxia-inducible factor-1 activation in nonhypoxic conditions: the essential role of mitochondrial-derived reactive oxygen species
    • Patten DA, Lafleur VN, Robitaille GA, Chan DA, Giaccia AJ, et al. (2010) Hypoxia-inducible factor-1 activation in nonhypoxic conditions: the essential role of mitochondrial-derived reactive oxygen species. Mol Biol Cell 21: 3247-3257.
    • (2010) Mol Biol Cell , vol.21 , pp. 3247-3257
    • Patten, D.A.1    Lafleur, V.N.2    Robitaille, G.A.3    Chan, D.A.4    Giaccia, A.J.5
  • 33
    • 80053904684 scopus 로고    scopus 로고
    • Mitochondrial complex III ROS regulate adipocyte differentiation
    • Tormos KV, Anso E, Hamanaka RB, Eisenbart J, Joseph J, et al. (2011) Mitochondrial complex III ROS regulate adipocyte differentiation. Cell Metab 14: 537-544.
    • (2011) Cell Metab , vol.14 , pp. 537-544
    • Tormos, K.V.1    Anso, E.2    Hamanaka, R.B.3    Eisenbart, J.4    Joseph, J.5
  • 34
    • 63349087445 scopus 로고    scopus 로고
    • Tissue-, substrate-, and site-specific characteristics of mitochondrial reactive oxygen species generation
    • Tahara EB, Navarete FD, Kowaltowski AJ, (2009) Tissue-, substrate-, and site-specific characteristics of mitochondrial reactive oxygen species generation. Free Radic Biol Med 46: 1283-1297.
    • (2009) Free Radic Biol Med , vol.46 , pp. 1283-1297
    • Tahara, E.B.1    Navarete, F.D.2    Kowaltowski, A.J.3
  • 35
    • 35348923542 scopus 로고    scopus 로고
    • Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease
    • Fukui H, Diaz F, Garcia S, Moraes CT, (2007) Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease. Proc Natl Acad Sci U S A 104: 14163-14168.
    • (2007) Proc Natl Acad Sci U S A , vol.104 , pp. 14163-14168
    • Fukui, H.1    Diaz, F.2    Garcia, S.3    Moraes, C.T.4
  • 37
    • 2642580016 scopus 로고    scopus 로고
    • Premature ageing in mice expressing defective mitochondrial DNA polymerase
    • Trifunovic A, Wredenberg A, Falkenberg M, Spelbrink JN, Rovio AT, et al. (2004) Premature ageing in mice expressing defective mitochondrial DNA polymerase. Nature 429: 417-423.
    • (2004) Nature , vol.429 , pp. 417-423
    • Trifunovic, A.1    Wredenberg, A.2    Falkenberg, M.3    Spelbrink, J.N.4    Rovio, A.T.5
  • 38
    • 64549127790 scopus 로고    scopus 로고
    • PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure
    • Canto C, Auwerx J, (2009) PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure. Curr Opin Lipidol 20: 98-105.
    • (2009) Curr Opin Lipidol , vol.20 , pp. 98-105
    • Canto, C.1    Auwerx, J.2
  • 39
    • 79959314684 scopus 로고    scopus 로고
    • In vivo correction of COX deficiency by activation of the AMPK/PGC-1alpha axis
    • Viscomi C, Bottani E, Civiletto G, Cerutti R, Moggio M, et al. (2011) In vivo correction of COX deficiency by activation of the AMPK/PGC-1alpha axis. Cell Metab 14: 80-90.
    • (2011) Cell Metab , vol.14 , pp. 80-90
    • Viscomi, C.1    Bottani, E.2    Civiletto, G.3    Cerutti, R.4    Moggio, M.5
  • 40
    • 77950806433 scopus 로고    scopus 로고
    • SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation
    • Hirschey MD, Shimazu T, Goetzman E, Jing E, Schwer B, et al. (2010) SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation. Nature 464: 121-125.
    • (2010) Nature , vol.464 , pp. 121-125
    • Hirschey, M.D.1    Shimazu, T.2    Goetzman, E.3    Jing, E.4    Schwer, B.5
  • 41
    • 77956227446 scopus 로고    scopus 로고
    • Complexity and pitfalls of mass spectrometry-based targeted metabolomics in brain research
    • Urban M, Enot DP, Dallmann G, Korner L, Forcher V, et al. (2010) Complexity and pitfalls of mass spectrometry-based targeted metabolomics in brain research. Anal Biochem 406: 124-131.
    • (2010) Anal Biochem , vol.406 , pp. 124-131
    • Urban, M.1    Enot, D.P.2    Dallmann, G.3    Korner, L.4    Forcher, V.5
  • 42
    • 77949702635 scopus 로고    scopus 로고
    • Metabolomic analyses of plasma reveals new insights into asphyxia and resuscitation in pigs
    • Solberg R, Enot D, Deigner HP, Koal T, Scholl-Burgi S, et al. (2010) Metabolomic analyses of plasma reveals new insights into asphyxia and resuscitation in pigs. PLoS One 5: e9606.
    • (2010) PLoS One , vol.5
    • Solberg, R.1    Enot, D.2    Deigner, H.P.3    Koal, T.4    Scholl-Burgi, S.5
  • 43
    • 13444304426 scopus 로고    scopus 로고
    • Missing value estimation for DNA microarray gene expression data: local least squares imputation
    • Kim H, Golub GH, Park H, (2005) Missing value estimation for DNA microarray gene expression data: local least squares imputation. Bioinformatics 21: 187-198.
    • (2005) Bioinformatics , vol.21 , pp. 187-198
    • Kim, H.1    Golub, G.H.2    Park, H.3
  • 44
    • 0001677717 scopus 로고
    • Controlling the false discovery rate: a practical and powerful approach to multiple testing
    • Benjamini Y, Hochberg Y, (1995) Controlling the false discovery rate: a practical and powerful approach to multiple testing. J R Stat Soc B pp. 289-300.
    • (1995) J R Stat Soc B , pp. 289-300
    • Benjamini, Y.1    Hochberg, Y.2
  • 45
    • 0002294347 scopus 로고
    • A simple sequentially rejective multiple test procedure
    • Holm S, (1979) A simple sequentially rejective multiple test procedure. Scand J of Statistics 6.
    • (1979) Scand J of Statistics 6
    • Holm, S.1
  • 46
    • 68949148655 scopus 로고    scopus 로고
    • Capacity of oxidative phosphorylation in human skeletal muscle: new perspectives of mitochondrial physiology
    • Gnaiger E, (2009) Capacity of oxidative phosphorylation in human skeletal muscle: new perspectives of mitochondrial physiology. Int J Biochem Cell Biol 41: 1837-1845.
    • (2009) Int J Biochem Cell Biol , vol.41 , pp. 1837-1845
    • Gnaiger, E.1
  • 48
    • 72649104840 scopus 로고    scopus 로고
    • Increased levels of cell-free hemoglobin, oxidation markers, and the antioxidative heme scavenger alpha(1)-microglobulin in preeclampsia
    • Olsson MG, Centlow M, Rutardottir S, Stenfors I, Larsson J, et al. (2010) Increased levels of cell-free hemoglobin, oxidation markers, and the antioxidative heme scavenger alpha(1)-microglobulin in preeclampsia. Free Radic Biol Med 48: 284-291.
    • (2010) Free Radic Biol Med , vol.48 , pp. 284-291
    • Olsson, M.G.1    Centlow, M.2    Rutardottir, S.3    Stenfors, I.4    Larsson, J.5


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