-
2
-
-
0037972522
-
Mitochondrial respiratory-chain diseases
-
S. DiMauro, and E.A. Schon Mitochondrial respiratory-chain diseases N. Engl. J. Med. 348 2003 2656 2668
-
(2003)
N. Engl. J. Med.
, vol.348
, pp. 2656-2668
-
-
Dimauro, S.1
Schon, E.A.2
-
4
-
-
77953507107
-
Mitochondrial DNA mutations in disease and aging
-
D.C. Wallace Mitochondrial DNA mutations in disease and aging Environ. Mol. Mutagen. 51 2010 440 450
-
(2010)
Environ. Mol. Mutagen.
, vol.51
, pp. 440-450
-
-
Wallace, D.C.1
-
5
-
-
0033563018
-
Quantitation and origin of the mitochondrial membrane potential in human cells lacking mitochondrial DNA
-
R.D. Appleby, W.K. Porteous, G. Hughes, A.M. James, D. Shannon, Y.H. Wei, and M.P. Murphy Quantitation and origin of the mitochondrial membrane potential in human cells lacking mitochondrial DNA Eur. J. Biochem. 262 1999 108 116
-
(1999)
Eur. J. Biochem.
, vol.262
, pp. 108-116
-
-
Appleby, R.D.1
Porteous, W.K.2
Hughes, G.3
James, A.M.4
Shannon, D.5
Wei, Y.H.6
Murphy, M.P.7
-
6
-
-
0033556240
-
Decreased ATP synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial tRNA mutations
-
A.M. James, P.W. Sheard, Y.H. Wei, and M.P. Murphy Decreased ATP synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial tRNA mutations Eur. J. Biochem. 259 1999 462 469
-
(1999)
Eur. J. Biochem.
, vol.259
, pp. 462-469
-
-
James, A.M.1
Sheard, P.W.2
Wei, Y.H.3
Murphy, M.P.4
-
7
-
-
0036126826
-
Biochemical-clinical correlation in patients with different loads of the mitochondrial DNA T8993G mutation
-
V. Carelli, A. Baracca, S. Barogi, F. Pallotti, M.L. Valentino, P. Montagna, M. Zeviani, A. Pini, G. Lenaz, A. Baruzzi, and G. Solaini Biochemical-clinical correlation in patients with different loads of the mitochondrial DNA T8993G mutation Arch. Neurol. 59 2002 264 270
-
(2002)
Arch. Neurol.
, vol.59
, pp. 264-270
-
-
Carelli, V.1
Baracca, A.2
Barogi, S.3
Pallotti, F.4
Valentino, M.L.5
Montagna, P.6
Zeviani, M.7
Pini, A.8
Lenaz, G.9
Baruzzi, A.10
Solaini, G.11
-
8
-
-
10644245921
-
Biochemical analysis of respiratory function in cybrid cell lines harbouring mitochondrial DNA mutations
-
F. Pallotti, A. Baracca, E. Hernandez-Rosa, W.F. Walker, G. Solaini, G. Lenaz, G.V. Melzi D'Eril, S. Dimauro, E.A. Schon, and M.M. Davidson Biochemical analysis of respiratory function in cybrid cell lines harbouring mitochondrial DNA mutations Biochem. J. 384 2004 287 293
-
(2004)
Biochem. J.
, vol.384
, pp. 287-293
-
-
Pallotti, F.1
Baracca, A.2
Hernandez-Rosa, E.3
Walker, W.F.4
Solaini, G.5
Lenaz, G.6
Melzi D'Eril, G.V.7
Dimauro, S.8
Schon, E.A.9
Davidson, M.M.10
-
9
-
-
34447106753
-
Biochemical phenotypes associated with the mitochondrial ATP6 gene mutations at nt8993
-
A. Baracca, G. Sgarbi, M. Mattiazzi, G. Casalena, E. Pagnotta, M.L. Valentino, M. Moggio, G. Lenaz, V. Carelli, and G. Solaini Biochemical phenotypes associated with the mitochondrial ATP6 gene mutations at nt8993 Biochim. Biophys. Acta 1767 2007 913 919
-
(2007)
Biochim. Biophys. Acta
, vol.1767
, pp. 913-919
-
-
Baracca, A.1
Sgarbi, G.2
Mattiazzi, M.3
Casalena, G.4
Pagnotta, E.5
Valentino, M.L.6
Moggio, M.7
Lenaz, G.8
Carelli, V.9
Solaini, G.10
-
10
-
-
77949472152
-
Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells
-
M. D'Aurelio, C. Vives-Bauza, M.M. Davidson, and G. Manfredi Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells Hum. Mol. Genet. 19 2010 374 386
-
(2010)
Hum. Mol. Genet.
, vol.19
, pp. 374-386
-
-
D'Aurelio, M.1
Vives-Bauza, C.2
Davidson, M.M.3
Manfredi, G.4
-
11
-
-
50849085156
-
The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex i activity in fibroblasts harboring mutations in these genes
-
C. Bonnet, S. Augustin, S. Ellouze, P. Bénit, A. Bouaita, P. Rustin, J.A. Sahel, and M. Corral-Debrinski The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes Biochim. Biophys. Acta 1783 2008 1707 1717
-
(2008)
Biochim. Biophys. Acta
, vol.1783
, pp. 1707-1717
-
-
Bonnet, C.1
Augustin, S.2
Ellouze, S.3
Bénit, P.4
Bouaita, A.5
Rustin, P.6
Sahel, J.A.7
Corral-Debrinski, M.8
-
12
-
-
73249138396
-
Patients with Leber hereditary optic neuropathy fail to compensate impaired oxidative phosphorylation
-
A. Korsten, I.F. de Coo, L. Spruijt, L.E. de Wit, H.J. Smeets, and W. Sluiter Patients with Leber hereditary optic neuropathy fail to compensate impaired oxidative phosphorylation Biochim. Biophys. Acta 1797 2010 197 203
-
(2010)
Biochim. Biophys. Acta
, vol.1797
, pp. 197-203
-
-
Korsten, A.1
De Coo, I.F.2
Spruijt, L.3
De Wit, L.E.4
Smeets, H.J.5
Sluiter, W.6
-
13
-
-
33645576104
-
Inefficient coupling between proton transport and ATP synthesis may be the pathogenic mechanism for NARP and Leigh syndrome resulting from the T8993G mutation in mtDNA
-
G. Sgarbi, A. Baracca, G. Lenaz, L.M. Valentino, V. Carelli, and G. Solaini Inefficient coupling between proton transport and ATP synthesis may be the pathogenic mechanism for NARP and Leigh syndrome resulting from the T8993G mutation in mtDNA Biochem. J. 395 2006 493 500
-
(2006)
Biochem. J.
, vol.395
, pp. 493-500
-
-
Sgarbi, G.1
Baracca, A.2
Lenaz, G.3
Valentino, L.M.4
Carelli, V.5
Solaini, G.6
-
14
-
-
0028847821
-
Abnormal calcium homeostasis and mitochondrial polarization in a human encephalomyopathy
-
A.M. Moudy, S.D. Handran, M.P. Goldberg, N. Ruffin, I. Karl, P. Kranz-Eble, D.C. DeVivo, and S.M. Rothman Abnormal calcium homeostasis and mitochondrial polarization in a human encephalomyopathy Proc. Natl. Acad. Sci. U. S. A. 92 1995 729 733
-
(1995)
Proc. Natl. Acad. Sci. U. S. A.
, vol.92
, pp. 729-733
-
-
Moudy, A.M.1
Handran, S.D.2
Goldberg, M.P.3
Ruffin, N.4
Karl, I.5
Kranz-Eble, P.6
Devivo, D.C.7
Rothman, S.M.8
-
15
-
-
34547146580
-
2+ homeostasis in respiratory chain-deficient cells but efficient compensation of energetic disadvantage by enhanced anaerobic glycolysis due to low ATP steady state levels
-
2+ homeostasis in respiratory chain-deficient cells but efficient compensation of energetic disadvantage by enhanced anaerobic glycolysis due to low ATP steady state levels Exp. Cell Res. 313 2007 3076 3089
-
(2007)
Exp. Cell Res.
, vol.313
, pp. 3076-3089
-
-
Von Kleist-Retzow, J.C.1
Hornig-Do, H.T.2
Schauen, M.3
Eckertz, S.4
Dinh, T.A.5
Stassen, F.6
Lottmann, N.7
Bust, M.8
Galunska, B.9
Wielckens, K.10
Hein, W.11
Beuth, J.12
Braun, J.M.13
Fischer, J.H.14
Ganitkevich, V.Y.15
Maniura-Weber, K.16
Wiesner, R.J.17
-
16
-
-
0141521643
-
New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters
-
C.D. Gajewski, L. Yang, E.A. Schon, and G. Manfredi New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters Mol. Biol. Cell 14 2003 3628 3635
-
(2003)
Mol. Biol. Cell
, vol.14
, pp. 3628-3635
-
-
Gajewski, C.D.1
Yang, L.2
Schon, E.A.3
Manfredi, G.4
-
17
-
-
33745779229
-
Diabetes-associated mitochondrial DNA mutation A3243G impairs cellular metabolic pathways necessary for beta cell function
-
P.B. de Andrade, B. Rubi, F. Frigerio, J.M. van den Ouweland, J.A. Maassen, and P. Maechler Diabetes-associated mitochondrial DNA mutation A3243G impairs cellular metabolic pathways necessary for beta cell function Diabetologia 49 2006 1816 1826
-
(2006)
Diabetologia
, vol.49
, pp. 1816-1826
-
-
De Andrade, P.B.1
Rubi, B.2
Frigerio, F.3
Van Den Ouweland, J.M.4
Maassen, J.A.5
Maechler, P.6
-
18
-
-
0033990284
-
Increased activities of antioxidant enzymes and decreased ATP concentration in cultured myoblasts with the 3243A → G mutation in mitochondrial DNA
-
H. Rusanen, K. Majamaa, and I.E. Hassinen Increased activities of antioxidant enzymes and decreased ATP concentration in cultured myoblasts with the 3243A → G mutation in mitochondrial DNA Biochim. Biophys. Acta 1500 2000 10 16
-
(2000)
Biochim. Biophys. Acta
, vol.1500
, pp. 10-16
-
-
Rusanen, H.1
Majamaa, K.2
Hassinen, I.E.3
-
19
-
-
37549067007
-
Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption
-
M. McKenzie, D. Liolitsa, N. Akinshina, M. Campanella, S. Sisodiya, I. Hargreaves, N. Nirmalananthan, M.G. Sweeney, P.M. Abou-Sleiman, N.W. Wood, M.G. Hanna, and M.R. Duchen Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption J. Biol. Chem. 282 2007 36845 36852
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 36845-36852
-
-
McKenzie, M.1
Liolitsa, D.2
Akinshina, N.3
Campanella, M.4
Sisodiya, S.5
Hargreaves, I.6
Nirmalananthan, N.7
Sweeney, M.G.8
Abou-Sleiman, P.M.9
Wood, N.W.10
Hanna, M.G.11
Duchen, M.R.12
-
20
-
-
10044230496
-
Influence of a mitochondrial genetic defect on capacitative calcium entry and mitochondrial organization in the osteosarcoma cells
-
J. Szczepanowska, K. Zabłocki, and J. Duszyński Influence of a mitochondrial genetic defect on capacitative calcium entry and mitochondrial organization in the osteosarcoma cells FEBS Lett. 578 2004 316 322
-
(2004)
FEBS Lett.
, vol.578
, pp. 316-322
-
-
Szczepanowska, J.1
Zabłocki, K.2
Duszyński, J.3
-
21
-
-
77950221314
-
Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations
-
A.Y. Abramov, T.K. Smulders-Srinivasan, D.M. Kirby, R. Acin-Perez, J.A. Enriquez, R.N. Lightowlers, M.R. Duchen, and D.M. Turnbull Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations Brain 133 2010 797 807
-
(2010)
Brain
, vol.133
, pp. 797-807
-
-
Abramov, A.Y.1
Smulders-Srinivasan, T.K.2
Kirby, D.M.3
Acin-Perez, R.4
Enriquez, J.A.5
Lightowlers, R.N.6
Duchen, M.R.7
Turnbull, D.M.8
-
22
-
-
0029790507
-
Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations
-
A.M. James, Y.H. Wei, C.Y. Pang, and M.P. Murphy Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations Biochem. J. 318 1996 401 407
-
(1996)
Biochem. J.
, vol.318
, pp. 401-407
-
-
James, A.M.1
Wei, Y.H.2
Pang, C.Y.3
Murphy, M.P.4
-
23
-
-
79955629653
-
Mitochondrial consumption of cytosolic ATP: Not so fast
-
C. Chinopoulos Mitochondrial consumption of cytosolic ATP: not so fast FEBS Lett. 585 2011 1255 1259
-
(2011)
FEBS Lett.
, vol.585
, pp. 1255-1259
-
-
Chinopoulos, C.1
-
24
-
-
0032483497
-
Functional F1-ATPase essential in maintaining growth and membrane potential of human mitochondrial DNA-depleted rho degrees cells
-
K. Buchet, and C. Godinot Functional F1-ATPase essential in maintaining growth and membrane potential of human mitochondrial DNA-depleted rho degrees cells J. Biol. Chem. 273 1998 22983 22989
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 22983-22989
-
-
Buchet, K.1
Godinot, C.2
-
26
-
-
57649199221
-
Cation and anion balance during active accumulation of Ca++ and Mg++ by isolated mitochondria
-
E. Carafoli, C.S. Rossi, and A.L. Lehninger Cation and anion balance during active accumulation of Ca++ and Mg++ by isolated mitochondria J. Biol. Chem. 239 1964 3055 3061
-
(1964)
J. Biol. Chem.
, vol.239
, pp. 3055-3061
-
-
Carafoli, E.1
Rossi, C.S.2
Lehninger, A.L.3
-
27
-
-
80051936634
-
A forty-kiloDalton protein of the inner membrane is the mitochondrial calcium uniporter
-
D. De Stefani, A. Raffaello, E. Teardo, I. Szabo, and R. Rizzuto A forty-kiloDalton protein of the inner membrane is the mitochondrial calcium uniporter Nature 476 2011 336 340
-
(2011)
Nature
, vol.476
, pp. 336-340
-
-
De Stefani, D.1
Raffaello, A.2
Teardo, E.3
Szabo, I.4
Rizzuto, R.5
-
28
-
-
68649090226
-
Regulation of mitochondrial dehydrogenases by calcium ions
-
R.M. Denton Regulation of mitochondrial dehydrogenases by calcium ions Biochim. Biophys. Acta 1787 2009 1309 1316
-
(2009)
Biochim. Biophys. Acta
, vol.1787
, pp. 1309-1316
-
-
Denton, R.M.1
-
29
-
-
0032773120
-
A calcium signaling defect in the pathogenesis of a mitochondrial DNA inherited oxidative phosphorylation deficiency
-
M. Brini, P. Pinton, M.P. King, M. Davidson, E.A. Schon, and R. Rizzuto A calcium signaling defect in the pathogenesis of a mitochondrial DNA inherited oxidative phosphorylation deficiency Nat. Med. 5 1999 951 954
-
(1999)
Nat. Med.
, vol.5
, pp. 951-954
-
-
Brini, M.1
Pinton, P.2
King, M.P.3
Davidson, M.4
Schon, E.A.5
Rizzuto, R.6
-
30
-
-
0034678580
-
Mitochondrial DNA-depleted neuroblastoma (Rho degrees) cells exhibit altered calcium signaling
-
T.B. Sherer, P.A. Trimmer, J.K. Parks, and J.B. Tuttle Mitochondrial DNA-depleted neuroblastoma (Rho degrees) cells exhibit altered calcium signaling Biochim. Biophys. Acta 1496 2000 341 355
-
(2000)
Biochim. Biophys. Acta
, vol.1496
, pp. 341-355
-
-
Sherer, T.B.1
Trimmer, P.A.2
Parks, J.K.3
Tuttle, J.B.4
-
31
-
-
84856555228
-
Effect of selenite on basic mitochondrial function in human osteosarcoma cells with chronic mitochondrial stress
-
M. Wojewoda, J. Duszynski, M. Wieckowski, and J. Szczepanowska Effect of selenite on basic mitochondrial function in human osteosarcoma cells with chronic mitochondrial stress Mitochondrion 12 2012 149 155
-
(2012)
Mitochondrion
, vol.12
, pp. 149-155
-
-
Wojewoda, M.1
Duszynski, J.2
Wieckowski, M.3
Szczepanowska, J.4
-
32
-
-
0042035647
-
Interactions between mitochondrial bioenergetics and cytoplasmic calcium in cultured cerebellar granule cells
-
D.G. Nicholls, S. Vesce, L. Kirk, and S. Chalmers Interactions between mitochondrial bioenergetics and cytoplasmic calcium in cultured cerebellar granule cells Cell Calcium 34 2003 407 424
-
(2003)
Cell Calcium
, vol.34
, pp. 407-424
-
-
Nicholls, D.G.1
Vesce, S.2
Kirk, L.3
Chalmers, S.4
-
33
-
-
77950279988
-
Mitochondrial DNA mutations affect calcium handling in differentiated neurons
-
A.J. Trevelyan, D.M. Kirby, T.K. Smulders-Srinivasan, M. Nooteboom, R. Acin-Perez, J.A. Enriquez, M.A. Whittington, R.N. Lightowlers, and D.M. Turnbull Mitochondrial DNA mutations affect calcium handling in differentiated neurons Brain 133 2010 787 796
-
(2010)
Brain
, vol.133
, pp. 787-796
-
-
Trevelyan, A.J.1
Kirby, D.M.2
Smulders-Srinivasan, T.K.3
Nooteboom, M.4
Acin-Perez, R.5
Enriquez, J.A.6
Whittington, M.A.7
Lightowlers, R.N.8
Turnbull, D.M.9
-
34
-
-
33745618412
-
The regulatory role of mitochondria in capacitative calcium entry
-
J. Duszynski, R. Koziel, W. Brutkowski, J. Szczepanowska, and K. Zablocki The regulatory role of mitochondria in capacitative calcium entry Biochim. Biophys. Acta 1757 2006 380 387
-
(2006)
Biochim. Biophys. Acta
, vol.1757
, pp. 380-387
-
-
Duszynski, J.1
Koziel, R.2
Brutkowski, W.3
Szczepanowska, J.4
Zablocki, K.5
-
35
-
-
0034641757
-
Mitochondrial control of calcium-channel gating: A mechanism for sustained signaling and transcriptional activation in T lymphocytes
-
M. Hoth, D.C. Button, and R.S. Lewis Mitochondrial control of calcium-channel gating: a mechanism for sustained signaling and transcriptional activation in T lymphocytes Proc. Natl. Acad. Sci. U. S. A. 97 2000 10607 10612
-
(2000)
Proc. Natl. Acad. Sci. U. S. A.
, vol.97
, pp. 10607-10612
-
-
Hoth, M.1
Button, D.C.2
Lewis, R.S.3
-
36
-
-
13544251370
-
Extracellular pH modifies mitochondrial control of capacitative calcium entry in Jurkat cells
-
K. Zablocki, J. Szczepanowska, and J. Duszynski Extracellular pH modifies mitochondrial control of capacitative calcium entry in Jurkat cells J. Biol. Chem. 280 2005 3516 3521
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 3516-3521
-
-
Zablocki, K.1
Szczepanowska, J.2
Duszynski, J.3
-
37
-
-
25844520458
-
Mitochondria in homeostasis of reactive oxygen species in cell, tissues, and organism
-
P. Jezek, and L. Hlavata Mitochondria in homeostasis of reactive oxygen species in cell, tissues, and organism Int. J. Biochem. Cell Biol. 37 2005 2478 2503
-
(2005)
Int. J. Biochem. Cell Biol.
, vol.37
, pp. 2478-2503
-
-
Jezek, P.1
Hlavata, L.2
-
38
-
-
4544328198
-
Antioxidant defences and homeostasis of reactive oxygen species in different human mitochondrial DNA-depleted cell lines
-
L. Vergani, M. Floreani, A. Russell, M. Ceccon, E. Napoli, A. Cabrelle, L. Valente, F. Bragantini, B. Leger, and F. Dabbeni-Sala Antioxidant defences and homeostasis of reactive oxygen species in different human mitochondrial DNA-depleted cell lines Eur. J. Biochem. 271 2004 3646 3656
-
(2004)
Eur. J. Biochem.
, vol.271
, pp. 3646-3656
-
-
Vergani, L.1
Floreani, M.2
Russell, A.3
Ceccon, M.4
Napoli, E.5
Cabrelle, A.6
Valente, L.7
Bragantini, F.8
Leger, B.9
Dabbeni-Sala, F.10
-
39
-
-
0023857490
-
Deficiency of subunits of complex i and mitochondrial encephalomyopathy
-
T. Ichiki, M. Tanaka, M. Nishikimi, H. Suzuki, T. Ozawa, M. Kobayashi, and Y. Wada Deficiency of subunits of complex I and mitochondrial encephalomyopathy Ann. Neurol. 23 1988 287 294
-
(1988)
Ann. Neurol.
, vol.23
, pp. 287-294
-
-
Ichiki, T.1
Tanaka, M.2
Nishikimi, M.3
Suzuki, H.4
Ozawa, T.5
Kobayashi, M.6
Wada, Y.7
-
40
-
-
0023117240
-
Two cases of NADH-coenzyme Q reductase deficiency: Relationship to MELAS syndrome
-
M. Kobayashi, H. Morishita, N. Sugiyama, K. Yokochi, M. Nakano, Y. Wada, Y. Hotta, A. Terauchi, and I. Nonaka Two cases of NADH-coenzyme Q reductase deficiency: relationship to MELAS syndrome J. Pediatr. 110 1987 223 227
-
(1987)
J. Pediatr.
, vol.110
, pp. 223-227
-
-
Kobayashi, M.1
Morishita, H.2
Sugiyama, N.3
Yokochi, K.4
Nakano, M.5
Wada, Y.6
Hotta, Y.7
Terauchi, A.8
Nonaka, I.9
-
41
-
-
0026681490
-
MELAS: Clinical features, biochemistry, and molecular genetics
-
E. Ciafaloni, E. Ricci, S. Shanske, C.T. Moraes, G. Silvestri, M. Hirano, S. Simonetti, C. Angelini, M.A. Donati, and C. Garcia MELAS: clinical features, biochemistry, and molecular genetics Ann. Neurol. 31 1992 391 398
-
(1992)
Ann. Neurol.
, vol.31
, pp. 391-398
-
-
Ciafaloni, E.1
Ricci, E.2
Shanske, S.3
Moraes, C.T.4
Silvestri, G.5
Hirano, M.6
Simonetti, S.7
Angelini, C.8
Donati, M.A.9
Garcia, C.10
-
42
-
-
33745137507
-
Involvement of protein kinase C delta in the alteration of mitochondrial mass in human cells under oxidative stress
-
C.F. Lee, Y.C. Chen, C.Y. Liu, and Y.H. Wei Involvement of protein kinase C delta in the alteration of mitochondrial mass in human cells under oxidative stress Free Radic. Biol. Med. 40 2006 2136 2146
-
(2006)
Free Radic. Biol. Med.
, vol.40
, pp. 2136-2146
-
-
Lee, C.F.1
Chen, Y.C.2
Liu, C.Y.3
Wei, Y.H.4
-
43
-
-
10344242925
-
Kinase signaling cascades in the mitochondrion: A matter of life or death
-
C. Horbinski, and C.T. Chu Kinase signaling cascades in the mitochondrion: a matter of life or death Free Radic. Biol. Med. 38 2005 2 11
-
(2005)
Free Radic. Biol. Med.
, vol.38
, pp. 2-11
-
-
Horbinski, C.1
Chu, C.T.2
-
44
-
-
0033767317
-
An out-of-frame cytochrome b gene deletion from a patient with parkinsonism is associated with impaired complex III assembly and an increase in free radical production
-
M. Rana, I. de Coo, F. Diaz, H. Smeets, and C.T. Moraes An out-of-frame cytochrome b gene deletion from a patient with parkinsonism is associated with impaired complex III assembly and an increase in free radical production Ann. Neurol. 48 2000 774 781
-
(2000)
Ann. Neurol.
, vol.48
, pp. 774-781
-
-
Rana, M.1
De Coo, I.2
Diaz, F.3
Smeets, H.4
Moraes, C.T.5
-
45
-
-
77953806512
-
Oxidative stress-dependent p66Shc phosphorylation in skin fibroblasts of children with mitochondrial disorders
-
M. Lebiedzinska, A. Karkucinska-Wieckowska, C. Giorgi, E. Karczmarewicz, E. Pronicka, P. Pinton, J. Duszynski, M. Pronicki, and M.R. Wieckowski Oxidative stress-dependent p66Shc phosphorylation in skin fibroblasts of children with mitochondrial disorders Biochim. Biophys. Acta 1797 2010 952 960
-
(2010)
Biochim. Biophys. Acta
, vol.1797
, pp. 952-960
-
-
Lebiedzinska, M.1
Karkucinska-Wieckowska, A.2
Giorgi, C.3
Karczmarewicz, E.4
Pronicka, E.5
Pinton, P.6
Duszynski, J.7
Pronicki, M.8
Wieckowski, M.R.9
-
46
-
-
28444445608
-
Enhanced ROS production and antioxidant defenses in cybrids harbouring mutations in mtDNA
-
C. Vives-Bauza, R. Gonzalo, G. Manfredi, E. Garcia-Arumi, and A.L. Andreu Enhanced ROS production and antioxidant defenses in cybrids harbouring mutations in mtDNA Neurosci. Lett. 391 2006 136 141
-
(2006)
Neurosci. Lett.
, vol.391
, pp. 136-141
-
-
Vives-Bauza, C.1
Gonzalo, R.2
Manfredi, G.3
Garcia-Arumi, E.4
Andreu, A.L.5
-
47
-
-
77955089549
-
Mitochondrial DNA mutation-elicited oxidative stress, oxidative damage, and altered gene expression in cultured cells of patients with MERRF syndrome
-
S.B. Wu, Y.S. Ma, Y.T. Wu, Y.C. Chen, and Y.H. Wei Mitochondrial DNA mutation-elicited oxidative stress, oxidative damage, and altered gene expression in cultured cells of patients with MERRF syndrome Mol. Neurobiol. 41 2010 256 266
-
(2010)
Mol. Neurobiol.
, vol.41
, pp. 256-266
-
-
Wu, S.B.1
Ma, Y.S.2
Wu, Y.T.3
Chen, Y.C.4
Wei, Y.H.5
-
48
-
-
77953810397
-
Antioxidant defence systems and generation of reactive oxygen species in osteosarcoma cells with defective mitochondria: Effect of selenium
-
M. Wojewoda, J. Duszyński, and J. Szczepanowska Antioxidant defence systems and generation of reactive oxygen species in osteosarcoma cells with defective mitochondria: effect of selenium Biochim. Biophys. Acta 1797 2010 890 896
-
(2010)
Biochim. Biophys. Acta
, vol.1797
, pp. 890-896
-
-
Wojewoda, M.1
Duszyński, J.2
Szczepanowska, J.3
-
49
-
-
1942453308
-
The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants
-
M. Mattiazzi, C. Vijayvergiya, C.D. Gajewski, D.C. DeVivo, G. Lenaz, M. Wiedmann, and G. Manfredi The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants Hum. Mol. Genet. 13 2004 869 879
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 869-879
-
-
Mattiazzi, M.1
Vijayvergiya, C.2
Gajewski, C.D.3
Devivo, D.C.4
Lenaz, G.5
Wiedmann, M.6
Manfredi, G.7
-
50
-
-
4944263224
-
Leber hereditary optic neuropathy mtDNA mutations disrupt glutamate transport in cybrid cell lines
-
S. Beretta, L. Mattavelli, G. Sala, L. Tremolizzo, A.H.V. Schapira, A. Martinuzzi, V. Carelli, and C. Ferrarese Leber hereditary optic neuropathy mtDNA mutations disrupt glutamate transport in cybrid cell lines Brain 127 2004 2183 2192
-
(2004)
Brain
, vol.127
, pp. 2183-2192
-
-
Beretta, S.1
Mattavelli, L.2
Sala, G.3
Tremolizzo, L.4
Schapira, A.H.V.5
Martinuzzi, A.6
Carelli, V.7
Ferrarese, C.8
-
51
-
-
0037084559
-
Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells
-
A. Wong, L. Cavelier, H.E. Collins-Schram, M.L. Selding, M. McGrogan, M.L. Savontaus, and G. Cortopassi Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells Hum. Mol. Genet. 11 2002 431 438
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 431-438
-
-
Wong, A.1
Cavelier, L.2
Collins-Schram, H.E.3
Selding, M.L.4
McGrogan, M.5
Savontaus, M.L.6
Cortopassi, G.7
-
52
-
-
20044381305
-
Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy
-
M. Floreani, E. Napoli, A. Martinuzzi, G. Pantano, V. De Riva, R. Trevisan, E. Bisetto, L. Valente, V. Carelli, and F. Dabbeni-Sala Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy FEBS J. 272 2005 1124 1135
-
(2005)
FEBS J.
, vol.272
, pp. 1124-1135
-
-
Floreani, M.1
Napoli, E.2
Martinuzzi, A.3
Pantano, G.4
De Riva, V.5
Trevisan, R.6
Bisetto, E.7
Valente, L.8
Carelli, V.9
Dabbeni-Sala, F.10
-
53
-
-
2642580016
-
Premature ageing in mice expressing defective mitochondrial DNA polymerase
-
A. Trifunovic, A. Wredenberg, M. Falkenberg, J.N. Spelbrink, A.T. Rovio, C.E. Bruder, M. Bohlooly-Y, S. Gidlöf, A. Oldfors, R. Wibom, J. Törnell, H.T. Jacobs, and N.G. Larsson Premature ageing in mice expressing defective mitochondrial DNA polymerase Nature 429 2004 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
Bruder, C.E.6
Bohlooly-Y, M.7
Gidlöf, S.8
Oldfors, A.9
Wibom, R.10
Törnell, J.11
Jacobs, H.T.12
Larsson, N.G.13
-
54
-
-
33751525408
-
Oxidative stress, mitochondria and mtDNA-mutator mice
-
L.V. Thompson Oxidative stress, mitochondria and mtDNA-mutator mice Exp. Gerontol. 2006 1220 1222
-
(2006)
Exp. Gerontol.
, pp. 1220-1222
-
-
Thompson, L.V.1
-
56
-
-
77951096150
-
Mitochondrial dynamics-fusion, fission, movement, and mitophagy-in neurodegenerative diseases
-
H. Chen, and D.C. Chan Mitochondrial dynamics-fusion, fission, movement, and mitophagy-in neurodegenerative diseases Hum. Mol. Genet. 18 2009 R169 R176
-
(2009)
Hum. Mol. Genet.
, vol.18
-
-
Chen, H.1
Chan, D.C.2
-
57
-
-
77955276765
-
Mitochondrial fusion proteins: Dual regulators of morphology and metabolism
-
A. Zorzano, M. Liesa, D. Sebastián, J. Segalés, and M. Palacín Mitochondrial fusion proteins: dual regulators of morphology and metabolism Semin. Cell Dev. Biol. 26 2010 566 574
-
(2010)
Semin. Cell Dev. Biol.
, vol.26
, pp. 566-574
-
-
Zorzano, A.1
Liesa, M.2
Sebastián, D.3
Segalés, J.4
Palacín, M.5
-
58
-
-
69449091821
-
Basic mechanisms for recognition and transport of synaptic
-
M.A. Schlager, and C.C. Hoogenraad Basic mechanisms for recognition and transport of synaptic Mol. Brain 2 2009 25
-
(2009)
Mol. Brain
, vol.2
, pp. 25
-
-
Schlager, M.A.1
Hoogenraad, C.C.2
-
59
-
-
78649413837
-
Mitochondrial fusion and fission in cell life and death
-
B. Westermann Mitochondrial fusion and fission in cell life and death Nat. Rev. Mol. Cell Biol. 11 2010 872 884
-
(2010)
Nat. Rev. Mol. Cell Biol.
, vol.11
, pp. 872-884
-
-
Westermann, B.1
-
60
-
-
34047173074
-
Mitochondrial bioenergetics and structural network organization
-
G. Benard, N. Bellance, D. James, P. Parrone, H. Fernandez, T. Letellier, and R. Rossignol Mitochondrial bioenergetics and structural network organization J. Cell Sci. 120 2007 838 848
-
(2007)
J. Cell Sci.
, vol.120
, pp. 838-848
-
-
Benard, G.1
Bellance, N.2
James, D.3
Parrone, P.4
Fernandez, H.5
Letellier, T.6
Rossignol, R.7
-
61
-
-
77953812057
-
Multi-site control and regulation of mitochondrial energy production
-
G. Benard, N. Bellance, C. Jose, S. Melser, K. Nouette-Gaulain, and R. Rossignol Multi-site control and regulation of mitochondrial energy production Biochim. Biophys. Acta 1797 2010 698 709
-
(2010)
Biochim. Biophys. Acta
, vol.1797
, pp. 698-709
-
-
Benard, G.1
Bellance, N.2
Jose, C.3
Melser, S.4
Nouette-Gaulain, K.5
Rossignol, R.6
-
62
-
-
33644552417
-
Increased production of reactive oxygen species in hyperglycemic conditions requires dynamic change of mitochondrial morphology
-
T. Yu, J.L. Robotham, and Y. Yoon Increased production of reactive oxygen species in hyperglycemic conditions requires dynamic change of mitochondrial morphology Proc. Natl. Acad. Sci. U. S. A. 21 2006 2653 2658
-
(2006)
Proc. Natl. Acad. Sci. U. S. A.
, vol.21
, pp. 2653-2658
-
-
Yu, T.1
Robotham, J.L.2
Yoon, Y.3
-
63
-
-
0036906665
-
Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins
-
F. Legros, A. Lombès, P. Frachon, and M. Rojo Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins Mol. Biol. Cell 13 2002 4343 4354
-
(2002)
Mol. Biol. Cell
, vol.13
, pp. 4343-4354
-
-
Legros, F.1
Lombès, A.2
Frachon, P.3
Rojo, M.4
-
64
-
-
53049099984
-
Short- and long-term alterations of mitochondrial morphology, dynamics and mtDNA after transient oxidative stress
-
M. Jendrach, S. Mai, S. Pohl, M. Vöth, and J. Bereiter-Hahn Short- and long-term alterations of mitochondrial morphology, dynamics and mtDNA after transient oxidative stress Mitochondrion 8 2008 293 304
-
(2008)
Mitochondrion
, vol.8
, pp. 293-304
-
-
Jendrach, M.1
Mai, S.2
Pohl, S.3
Vöth, M.4
Bereiter-Hahn, J.5
-
65
-
-
25444446126
-
Mitochondrial network complexity and pathological decrease in complex i activity are tightly correlated in isolated human complex i deficiency
-
W.J.H. Koopman, H.-J. Visch, S. Verkaart, L.W.P.J. van den Heuvel, J.A.M. Smeitink, and P.H.G.M. Willems Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency Am. J. Physiol. Cell Physiol. 289 2005 C881 C890
-
(2005)
Am. J. Physiol. Cell Physiol.
, vol.289
-
-
Koopman, W.J.H.1
Visch, H.-J.2
Verkaart, S.3
Van Den Heuvel, L.W.P.J.4
Smeitink, J.A.M.5
Willems, P.H.G.M.6
-
67
-
-
2042459176
-
Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging
-
N.A. Pham, T. Richardson, J. Cameron, B. Chue, and B.H. Robinson Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging Microsc. Microanal. 10 2004 247 260
-
(2004)
Microsc. Microanal.
, vol.10
, pp. 247-260
-
-
Pham, N.A.1
Richardson, T.2
Cameron, J.3
Chue, B.4
Robinson, B.H.5
-
68
-
-
21344447244
-
Human extraocular muscles in mitochondrial diseases: Comparing chronic progressive external ophthalmoplegia with Leber's hereditary optic neuropathy
-
A. Carta, V. Carelli, T. D'Adda, and F.N. Ross-Cisneros Human extraocular muscles in mitochondrial diseases: comparing chronic progressive external ophthalmoplegia with Leber's hereditary optic neuropathy Br. J. Ophthalmol. 89 2005 825 827
-
(2005)
Br. J. Ophthalmol.
, vol.89
, pp. 825-827
-
-
Carta, A.1
Carelli, V.2
D'Adda, T.3
Ross-Cisneros, F.N.4
-
69
-
-
27844586328
-
Infantile mitochondrial leucodystrophy - A case report
-
B. Schmidt-Sidor, K. Szymańska, E. Lewandowska, H. Mierzewska, T. Wierzba-Bobrowicz, E. Pasennik, and T. Stepień Infantile mitochondrial leucodystrophy - a case report Folia Neuropathol. 43 2005 186 190
-
(2005)
Folia Neuropathol.
, vol.43
, pp. 186-190
-
-
Schmidt-Sidor, B.1
Szymańska, K.2
Lewandowska, E.3
Mierzewska, H.4
Wierzba-Bobrowicz, T.5
Pasennik, E.6
Stepień, T.7
-
70
-
-
0029840031
-
Adult-onset MELAS evidence for involvement of neurons as well as cerebral vasculature in stroke like episodes
-
J.M. Gilchrist, M. Sikirica, E. Stopa, and S. Shanske Adult-onset MELAS evidence for involvement of neurons as well as cerebral vasculature in stroke like episodes Stroke 27 1996 1420 1423
-
(1996)
Stroke
, vol.27
, pp. 1420-1423
-
-
Gilchrist, J.M.1
Sikirica, M.2
Stopa, E.3
Shanske, S.4
-
71
-
-
67651152870
-
Mitochondrial mosaics in the liver of 3 infants with mtDNA defects
-
F. Roels, P. Verloo, F. Eyskens, B. François, S. Seneca, B. De Paepe, J.-J. Martin, V. Meersschaut, M. Praet, E. Scalais, M. Espee, J. Smet, G. Van Goethem, and R. Van Coster Mitochondrial mosaics in the liver of 3 infants with mtDNA defects BMC Clin. Pathol. 9 2009 4
-
(2009)
BMC Clin. Pathol.
, vol.9
, pp. 4
-
-
Roels, F.1
Verloo, P.2
Eyskens, F.3
François, B.4
Seneca, S.5
De Paepe, B.6
Martin, J.-J.7
Meersschaut, V.8
Praet, M.9
Scalais, E.10
Espee, M.11
Smet, J.12
Van Goethem, G.13
Van Coster, R.14
-
72
-
-
33845976357
-
Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology
-
S. Duvezin-Caubet, R. Jagasia, J. Wagener, S. Hofmann, A. Trifunovic, A. Hansson, A. Chomyn, M.F. Bauer, G. Attardi, N.-G. Larsson, W. Neupert, W. Neupert, and A.S. Reichert Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology J. Biol. Chem. 281 2006 37972 37979
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 37972-37979
-
-
Duvezin-Caubet, S.1
Jagasia, R.2
Wagener, J.3
Hofmann, S.4
Trifunovic, A.5
Hansson, A.6
Chomyn, A.7
Bauer, M.F.8
Attardi, G.9
Larsson, N.-G.10
Neupert, W.11
Neupert, W.12
Reichert, A.S.13
-
73
-
-
70849122412
-
Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo
-
R.M. Baqri, B.A. Turner, M.B. Rheuben, B.D. Hammond, L.S. Kaguni, and K.E. Miller Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo PLoS One 4 2009 e7874
-
(2009)
PLoS One
, vol.4
, pp. 7874
-
-
Baqri, R.M.1
Turner, B.A.2
Rheuben, M.B.3
Hammond, B.D.4
Kaguni, L.S.5
Miller, K.E.6
-
74
-
-
34249722612
-
Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology
-
M. Rak, E. Tetaud, F. Godard, I. Sagot, B. Salin, S. Duvezin-Caubet, P.P. Slonimski, J. Rytka, and J.-P. di Rago Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology J. Biol. Chem. 282 2007 10853 10864
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 10853-10864
-
-
Rak, M.1
Tetaud, E.2
Godard, F.3
Sagot, I.4
Salin, B.5
Duvezin-Caubet, S.6
Slonimski, P.P.7
Rytka, J.8
Di Rago, J.-P.9
-
75
-
-
0034776305
-
Mitochondrial DNA rearrangements in young onset parkinsonism: Two case reports
-
G. Siciliano, M. Mancuso, R. Ceravolo, V. Lombardi, A. Iudice, and U. Bonuccelli Mitochondrial DNA rearrangements in young onset parkinsonism: two case reports J. Neurol. Neurosurg. Psychiatry 71 2001 685 687
-
(2001)
J. Neurol. Neurosurg. Psychiatry
, vol.71
, pp. 685-687
-
-
Siciliano, G.1
Mancuso, M.2
Ceravolo, R.3
Lombardi, V.4
Iudice, A.5
Bonuccelli, U.6
|