-
1
-
-
84875920169
-
Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model
-
Aidt F.H., Nielsen S.M., Kanters J., Pesta D., Nielsen T.T., Norremolle A., Hasholt L., Christiansen M., Hagen C.M. Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model. PLoS Curr. 2013, 5.
-
(2013)
PLoS Curr.
, vol.5
-
-
Aidt, F.H.1
Nielsen, S.M.2
Kanters, J.3
Pesta, D.4
Nielsen, T.T.5
Norremolle, A.6
Hasholt, L.7
Christiansen, M.8
Hagen, C.M.9
-
2
-
-
38049053467
-
A stress sensitive ER membrane-association domain in Huntingtin protein defines a potential role for Huntingtin in the regulation of autophagy
-
Atwal R.S., Truant R. A stress sensitive ER membrane-association domain in Huntingtin protein defines a potential role for Huntingtin in the regulation of autophagy. Autophagy 2008, 4:91-93.
-
(2008)
Autophagy
, vol.4
, pp. 91-93
-
-
Atwal, R.S.1
Truant, R.2
-
3
-
-
0027433553
-
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid
-
Beal M.F., Brouillet E., Jenkins B.G., Ferrante R.J., Kowall N.W., Miller J.M., Storey E., Srivastava R., Rosen B.R., Hyman B.T. Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid. J. Neurosci. Off. J. Soc. Neurosci. 1993, 13:4181-4192.
-
(1993)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.13
, pp. 4181-4192
-
-
Beal, M.F.1
Brouillet, E.2
Jenkins, B.G.3
Ferrante, R.J.4
Kowall, N.W.5
Miller, J.M.6
Storey, E.7
Srivastava, R.8
Rosen, B.R.9
Hyman, B.T.10
-
4
-
-
33745392939
-
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin
-
Benchoua A., Trioulier Y., Zala D., Gaillard M.C., Lefort N., Dufour N., Saudou F., Elalouf J.M., Hirsch E., Hantraye P., Déglon N., Brouillet E. Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol. Biol. Cell 2006, 17(4):1652-1663.
-
(2006)
Mol. Biol. Cell
, vol.17
, Issue.4
, pp. 1652-1663
-
-
Benchoua, A.1
Trioulier, Y.2
Zala, D.3
Gaillard, M.C.4
Lefort, N.5
Dufour, N.6
Saudou, F.7
Elalouf, J.M.8
Hirsch, E.9
Hantraye, P.10
Déglon, N.11
Brouillet, E.12
-
5
-
-
0033681149
-
Chronic systemic pesticide exposure reproduces features of Parkinson's disease
-
Betarbet R., Sherer T.B., MacKenzie G., Garcia-Osuna M., Panov A.V., Greenamyre J.T. Chronic systemic pesticide exposure reproduces features of Parkinson's disease. Nat. Neurosci. 2000, 3:1301-1306.
-
(2000)
Nat. Neurosci.
, vol.3
, pp. 1301-1306
-
-
Betarbet, R.1
Sherer, T.B.2
MacKenzie, G.3
Garcia-Osuna, M.4
Panov, A.V.5
Greenamyre, J.T.6
-
8
-
-
0027448161
-
Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid
-
Brouillet E., Jenkins B.G., Hyman B.T., Ferrante R.J., Kowall N.W., Srivastava R., Roy D.S., Rosen B.R., Beal M.F. Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid. J. Neurochem. 1993, 60:356-359.
-
(1993)
J. Neurochem.
, vol.60
, pp. 356-359
-
-
Brouillet, E.1
Jenkins, B.G.2
Hyman, B.T.3
Ferrante, R.J.4
Kowall, N.W.5
Srivastava, R.6
Roy, D.S.7
Rosen, B.R.8
Beal, M.F.9
-
9
-
-
57649187103
-
Mitochondria and Huntington's disease pathogenesis: insight from genetic and chemical models
-
Browne S.E. Mitochondria and Huntington's disease pathogenesis: insight from genetic and chemical models. Ann. N. Y. Acad. Sci. 2008, 1147:358-382.
-
(2008)
Ann. N. Y. Acad. Sci.
, vol.1147
, pp. 358-382
-
-
Browne, S.E.1
-
10
-
-
0030919567
-
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia
-
Browne S.E., Bowling A.C., MacGarvey U., Baik M.J., Berger S.C., Muqit M.M., Bird E.D., Beal M.F. Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann. Neurol. 1997, 41:646-653.
-
(1997)
Ann. Neurol.
, vol.41
, pp. 646-653
-
-
Browne, S.E.1
Bowling, A.C.2
MacGarvey, U.3
Baik, M.J.4
Berger, S.C.5
Muqit, M.M.6
Bird, E.D.7
Beal, M.F.8
-
11
-
-
0037741021
-
Increased susceptibility of striatal mitochondria to calcium-induced permeability transition
-
Brustovetsky N., Brustovetsky T., Purl K.J., Capano M., Crompton M., Dubinsky J.M. Increased susceptibility of striatal mitochondria to calcium-induced permeability transition. J. Neurosci. Off. J. Soc. Neurosci. 2003, 23:4858-4867.
-
(2003)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.23
, pp. 4858-4867
-
-
Brustovetsky, N.1
Brustovetsky, T.2
Purl, K.J.3
Capano, M.4
Crompton, M.5
Dubinsky, J.M.6
-
12
-
-
67650061723
-
Impaired PGC-1alpha function in muscle in Huntington's disease
-
Chaturvedi R.K., Adhihetty P., Shukla S., Hennessy T., Calingasan N., Yang L., Starkov A., Kiaei M., Cannella M., Sassone J., Ciammola A., Squitieri F., Beal M.F. Impaired PGC-1alpha function in muscle in Huntington's disease. Hum. Mol. Genet. 2009, 18:3048-3065.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 3048-3065
-
-
Chaturvedi, R.K.1
Adhihetty, P.2
Shukla, S.3
Hennessy, T.4
Calingasan, N.5
Yang, L.6
Starkov, A.7
Kiaei, M.8
Cannella, M.9
Sassone, J.10
Ciammola, A.11
Squitieri, F.12
Beal, M.F.13
-
13
-
-
3543141113
-
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
-
Choo Y.S., Johnson G.V., MacDonald M., Detloff P.J., Lesort M. Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum. Mol. Genet. 2004, 13:1407-1420.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 1407-1420
-
-
Choo, Y.S.1
Johnson, G.V.2
MacDonald, M.3
Detloff, P.J.4
Lesort, M.5
-
15
-
-
33749042331
-
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
-
Cui L., Jeong H., Borovecki F., Parkhurst C.N., Tanese N., Krainc D. Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006, 127:59-69.
-
(2006)
Cell
, vol.127
, pp. 59-69
-
-
Cui, L.1
Jeong, H.2
Borovecki, F.3
Parkhurst, C.N.4
Tanese, N.5
Krainc, D.6
-
16
-
-
84888792149
-
A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin
-
Damiano M., Diguet E., Malgorn C., D'Aurelio M., Galvan L., Petit F., Benhaim L., Guillermier M., Houitte D., Dufour N., Hantraye P., Canals J.M., Alberch J., Delzescaux T., Deglon N., Beal M.F., Brouillet E. A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum. Mol. Genet. 2013, 22:3869-3882.
-
(2013)
Hum. Mol. Genet.
, vol.22
, pp. 3869-3882
-
-
Damiano, M.1
Diguet, E.2
Malgorn, C.3
D'Aurelio, M.4
Galvan, L.5
Petit, F.6
Benhaim, L.7
Guillermier, M.8
Houitte, D.9
Dufour, N.10
Hantraye, P.11
Canals, J.M.12
Alberch, J.13
Delzescaux, T.14
Deglon, N.15
Beal, M.F.16
Brouillet, E.17
-
17
-
-
0029982347
-
Mitochondrial DNA polymerase gamma is expressed and translated in the absence of mitochondrial DNA maintenance and replication
-
Davis A.F., Ropp P.A., Clayton D.A., Copeland W.C. Mitochondrial DNA polymerase gamma is expressed and translated in the absence of mitochondrial DNA maintenance and replication. Nucleic Acids Res. 1996, 24:2753-2759.
-
(1996)
Nucleic Acids Res.
, vol.24
, pp. 2753-2759
-
-
Davis, A.F.1
Ropp, P.A.2
Clayton, D.A.3
Copeland, W.C.4
-
18
-
-
2442431673
-
Mitochondrial transcription factor A regulates mtDNA copy number in mammals
-
Ekstrand M.I., Falkenberg M., Rantanen A., Park C.B., Gaspari M., Hultenby K., Rustin P., Gustafsson C.M., Larsson N.G. Mitochondrial transcription factor A regulates mtDNA copy number in mammals. Hum. Mol. Genet. 2004, 13:935-944.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 935-944
-
-
Ekstrand, M.I.1
Falkenberg, M.2
Rantanen, A.3
Park, C.B.4
Gaspari, M.5
Hultenby, K.6
Rustin, P.7
Gustafsson, C.M.8
Larsson, N.G.9
-
20
-
-
34347335707
-
P-body formation is a consequence, not the cause, of RNA-mediated gene silencing
-
Eulalio A., Behm-Ansmant I., Schweizer D., Izaurralde E. P-body formation is a consequence, not the cause, of RNA-mediated gene silencing. Mol. Cell. Biol. 2007, 27:3970-3981.
-
(2007)
Mol. Cell. Biol.
, vol.27
, pp. 3970-3981
-
-
Eulalio, A.1
Behm-Ansmant, I.2
Schweizer, D.3
Izaurralde, E.4
-
21
-
-
0028075773
-
Aconitase is a sensitive and critical target of oxygen poisoning in cultured mammalian cells and in rat lungs
-
(1994 Dec)
-
Gardner P.R., Nguyen D.D., White C.W. Aconitase is a sensitive and critical target of oxygen poisoning in cultured mammalian cells and in rat lungs. Proc. Natl. Acad. Sci. U. S. A. 1994, 6:12248-12252. (1994 Dec).
-
(1994)
Proc. Natl. Acad. Sci. U. S. A.
, vol.6
, pp. 12248-12252
-
-
Gardner, P.R.1
Nguyen, D.D.2
White, C.W.3
-
22
-
-
57649158657
-
Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats
-
Gellerich F.N., Gizatullina Z., Nguyen H.P., Trumbeckaite S., Vielhaber S., Seppet E., Zierz S., Landwehrmeyer B., Riess O., von Horsten S., Striggow F. Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats. J. Biol. Chem. 2008, 283:30715-30724.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 30715-30724
-
-
Gellerich, F.N.1
Gizatullina, Z.2
Nguyen, H.P.3
Trumbeckaite, S.4
Vielhaber, S.5
Seppet, E.6
Zierz, S.7
Landwehrmeyer, B.8
Riess, O.9
von Horsten, S.10
Striggow, F.11
-
23
-
-
0022345539
-
Brain enzyme and clinical alterations induced in rats and mice by nitroaliphatic toxicants
-
Gould D.H., Wilson M.P., Hamar D.W. Brain enzyme and clinical alterations induced in rats and mice by nitroaliphatic toxicants. Toxicol. Lett. 1985, 27:83-89.
-
(1985)
Toxicol. Lett.
, vol.27
, pp. 83-89
-
-
Gould, D.H.1
Wilson, M.P.2
Hamar, D.W.3
-
24
-
-
0029875381
-
Mitochondrial defect in Huntington's disease caudate nucleus
-
Gu M., Gash M.T., Mann V.M., Javoy-Agid F., Cooper J.M., Schapira A.H. Mitochondrial defect in Huntington's disease caudate nucleus. Ann. Neurol. 1996, 39:385-389.
-
(1996)
Ann. Neurol.
, vol.39
, pp. 385-389
-
-
Gu, M.1
Gash, M.T.2
Mann, V.M.3
Javoy-Agid, F.4
Cooper, J.M.5
Schapira, A.H.6
-
25
-
-
0034994207
-
Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production
-
Guidetti P., Charles V., Chen E.Y., Reddy P.H., Kordower J.H., Whetsell W.O., Schwarcz R., Tagle D.A. Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production. Exp. Neurol. 2001, 169:340-350.
-
(2001)
Exp. Neurol.
, vol.169
, pp. 340-350
-
-
Guidetti, P.1
Charles, V.2
Chen, E.Y.3
Reddy, P.H.4
Kordower, J.H.5
Whetsell, W.O.6
Schwarcz, R.7
Tagle, D.A.8
-
26
-
-
0030997707
-
Quantifiable bradykinesia, gait abnormalities and Huntington's disease-like striatal lesions in rats chronically treated with 3-nitropropionic acid
-
Guyot M.C., Hantraye P., Dolan R., Palfi S., Maziere M., Brouillet E. Quantifiable bradykinesia, gait abnormalities and Huntington's disease-like striatal lesions in rats chronically treated with 3-nitropropionic acid. Neuroscience 1997, 79:45-56.
-
(1997)
Neuroscience
, vol.79
, pp. 45-56
-
-
Guyot, M.C.1
Hantraye, P.2
Dolan, R.3
Palfi, S.4
Maziere, M.5
Brouillet, E.6
-
27
-
-
45449087921
-
QBase relative quantification framework and software for management and automated analysis of real-time quantitative PCR data
-
Hellemans J., Mortier G., De Paepe A., Speleman F., Vandesompele J. qBase relative quantification framework and software for management and automated analysis of real-time quantitative PCR data. Genome Biol. 2007, 8:R19.
-
(2007)
Genome Biol.
, vol.8
, pp. R19
-
-
Hellemans, J.1
Mortier, G.2
De Paepe, A.3
Speleman, F.4
Vandesompele, J.5
-
28
-
-
0033378683
-
Metabolic and glutamatergic disturbances in the Huntington's disease transgenic mouse
-
Higgins D.S., Hoyt K.R., Baic C., Vensel J., Sulka M. Metabolic and glutamatergic disturbances in the Huntington's disease transgenic mouse. Ann. N. Y. Acad. Sci. 1999, 893:298-300.
-
(1999)
Ann. N. Y. Acad. Sci.
, vol.893
, pp. 298-300
-
-
Higgins, D.S.1
Hoyt, K.R.2
Baic, C.3
Vensel, J.4
Sulka, M.5
-
29
-
-
84867422702
-
Structural basis for malfunction in complex II
-
Iverson T.M., Maklashina E., Cecchini G. Structural basis for malfunction in complex II. J. Biol. Chem. 2012, 287:35430-35438.
-
(2012)
J. Biol. Chem.
, vol.287
, pp. 35430-35438
-
-
Iverson, T.M.1
Maklashina, E.2
Cecchini, G.3
-
30
-
-
0031596998
-
1H NMR spectroscopy studies of Huntington's disease: correlations with CAG repeat numbers
-
Jenkins B.G., Rosas H.D., Chen Y.C., Makabe T., Myers R., MacDonald M., Rosen B.R., Beal M.F., Koroshetz W.J. 1H NMR spectroscopy studies of Huntington's disease: correlations with CAG repeat numbers. Neurology 1998, 50:1357-1365.
-
(1998)
Neurology
, vol.50
, pp. 1357-1365
-
-
Jenkins, B.G.1
Rosas, H.D.2
Chen, Y.C.3
Makabe, T.4
Myers, R.5
MacDonald, M.6
Rosen, B.R.7
Beal, M.F.8
Koroshetz, W.J.9
-
31
-
-
0034307476
-
Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy
-
Kegel K.B., Kim M., Sapp E., McIntyre C., Castano J.G., Aronin N., DiFiglia M. Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. J. Neurosci. Off. J. Soc. Neurosci. 2000, 20:7268-7278.
-
(2000)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.20
, pp. 7268-7278
-
-
Kegel, K.B.1
Kim, M.2
Sapp, E.3
McIntyre, C.4
Castano, J.G.5
Aronin, N.6
DiFiglia, M.7
-
32
-
-
18544368523
-
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
-
Kegel K.B., Meloni A.R., Yi Y., Kim Y.J., Doyle E., Cuiffo B.G., Sapp E., Wang Y., Qin Z.H., Chen J.D., Nevins J.R., Aronin N., DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J. Biol. Chem. 2002, 277:7466-7476.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 7466-7476
-
-
Kegel, K.B.1
Meloni, A.R.2
Yi, Y.3
Kim, Y.J.4
Doyle, E.5
Cuiffo, B.G.6
Sapp, E.7
Wang, Y.8
Qin, Z.H.9
Chen, J.D.10
Nevins, J.R.11
Aronin, N.12
DiFiglia, M.13
-
33
-
-
77957742105
-
Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease
-
Kim J., Moody J.P., Edgerly C.K., Bordiuk O.L., Cormier K., Smith K., Beal M.F., Ferrante R.J. Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease. Hum. Mol. Genet. 2010, 19:3919-3935.
-
(2010)
Hum. Mol. Genet.
, vol.19
, pp. 3919-3935
-
-
Kim, J.1
Moody, J.P.2
Edgerly, C.K.3
Bordiuk, O.L.4
Cormier, K.5
Smith, K.6
Beal, M.F.7
Ferrante, R.J.8
-
34
-
-
0014197852
-
Studies on cytochrome oxidase. 3. Ligand reactions and Schiff base formation of heme a
-
(1967 Mar 10)
-
King T.E., Yong F.C., Takemori S. Studies on cytochrome oxidase. 3. Ligand reactions and Schiff base formation of heme a. J. Biol. Chem. 1967, 10:819-829. (1967 Mar 10).
-
(1967)
J. Biol. Chem.
, vol.10
, pp. 819-829
-
-
King, T.E.1
Yong, F.C.2
Takemori, S.3
-
35
-
-
71049146803
-
Levels of plasma circulating cell free nuclear and mitochondrial DNA as potential biomarkers for breast tumors
-
Kohler C., Radpour R., Barekati Z., Asadollahi R., Bitzer J., Wight E., Burki N., Diesch C., Holzgreve W., Zhong X.Y. Levels of plasma circulating cell free nuclear and mitochondrial DNA as potential biomarkers for breast tumors. Mol. Cancer 2009, 8:105.
-
(2009)
Mol. Cancer
, vol.8
, pp. 105
-
-
Kohler, C.1
Radpour, R.2
Barekati, Z.3
Asadollahi, R.4
Bitzer, J.5
Wight, E.6
Burki, N.7
Diesch, C.8
Holzgreve, W.9
Zhong, X.Y.10
-
36
-
-
0031044805
-
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
-
Koroshetz W.J., Jenkins B.G., Rosen B.R., Beal M.F. Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann. Neurol. 1997, 41:160-165.
-
(1997)
Ann. Neurol.
, vol.41
, pp. 160-165
-
-
Koroshetz, W.J.1
Jenkins, B.G.2
Rosen, B.R.3
Beal, M.F.4
-
37
-
-
0028223609
-
Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion
-
Larsson N.G., Oldfors A., Holme E., Clayton D.A. Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion. Biochem. Biophys. Res. Commun. 1994, 200:1374-1381.
-
(1994)
Biochem. Biophys. Res. Commun.
, vol.200
, pp. 1374-1381
-
-
Larsson, N.G.1
Oldfors, A.2
Holme, E.3
Clayton, D.A.4
-
38
-
-
16044373842
-
Exon 1 of the HD Gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini L. Exon 1 of the HD Gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87:493-506.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
-
39
-
-
0025078573
-
Mitochondrial function and parental sex effect in Huntington's disease
-
Mann V.M., Cooper J.M., Javoy-Agid F., Agid Y., Jenner P., Schapira A.H. Mitochondrial function and parental sex effect in Huntington's disease. Lancet 1990, 336:749.
-
(1990)
Lancet
, vol.336
, pp. 749
-
-
Mann, V.M.1
Cooper, J.M.2
Javoy-Agid, F.3
Agid, Y.4
Jenner, P.5
Schapira, A.H.6
-
40
-
-
3042695377
-
Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells
-
Matsushima Y., Garesse R., Kaguni L.S. Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells. J. Biol. Chem. 2004, 279:26900-26905.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 26900-26905
-
-
Matsushima, Y.1
Garesse, R.2
Kaguni, L.S.3
-
41
-
-
24744444740
-
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin
-
Milakovic T., Johnson G.V. Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J. Biol. Chem. 2005, 280:30773-30782.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 30773-30782
-
-
Milakovic, T.1
Johnson, G.V.2
-
42
-
-
0023115210
-
Congenital deficiency of two polypeptide subunits of the iron-protein fragment of mitochondrial complex I
-
Moreadith R.W.C.M., Ragan C.I., Batshaw M.L., Lehninger A.L. Congenital deficiency of two polypeptide subunits of the iron-protein fragment of mitochondrial complex I. J. Clin. Invest. 1987, 79:463-467.
-
(1987)
J. Clin. Invest.
, vol.79
, pp. 463-467
-
-
Moreadith, R.W.C.M.1
Ragan, C.I.2
Batshaw, M.L.3
Lehninger, A.L.4
-
43
-
-
77953302472
-
Nature and cause of mitochondrial dysfunction in Huntington's disease: focusing on huntingtin and the striatum
-
Oliveira J.M. Nature and cause of mitochondrial dysfunction in Huntington's disease: focusing on huntingtin and the striatum. J. Neurochem. 2010, 114:1-12.
-
(2010)
J. Neurochem.
, vol.114
, pp. 1-12
-
-
Oliveira, J.M.1
-
44
-
-
40849147435
-
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking
-
Orr A.L., Li S., Wang C.E., Li H., Wang J., Rong J., Xu X., Mastroberardino P.G., Greenamyre J.T., Li X.J. N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J. Neurosci. Off. J. Soc. Neurosci. 2008, 28:2783-2792.
-
(2008)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.28
, pp. 2783-2792
-
-
Orr, A.L.1
Li, S.2
Wang, C.E.3
Li, H.4
Wang, J.5
Rong, J.6
Xu, X.7
Mastroberardino, P.G.8
Greenamyre, J.T.9
Li, X.J.10
-
45
-
-
0030001887
-
Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease
-
Palfi S., Ferrante R.J., Brouillet E., Beal M.F., Dolan R., Guyot M.C., Peschanski M., Hantraye P. Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease. J. Neurosci. Off. J. Soc. Neurosci. 1996, 16:3019-3025.
-
(1996)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.16
, pp. 3019-3025
-
-
Palfi, S.1
Ferrante, R.J.2
Brouillet, E.3
Beal, M.F.4
Dolan, R.5
Guyot, M.C.6
Peschanski, M.7
Hantraye, P.8
-
46
-
-
0036327065
-
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines
-
Panov A.V., Gutekunst C.A., Leavitt B.R., Hayden M.R., Burke J.R., Strittmatter W.J., Greenamyre J.T. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 2002, 5:731-736.
-
(2002)
Nat. Neurosci.
, vol.5
, pp. 731-736
-
-
Panov, A.V.1
Gutekunst, C.A.2
Leavitt, B.R.3
Hayden, M.R.4
Burke, J.R.5
Strittmatter, W.J.6
Greenamyre, J.T.7
-
47
-
-
61849093278
-
Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models
-
Phan J., Hickey M.A., Zhang P., Chesselet M.F., Reue K. Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models. Hum. Mol. Genet. 2009, 18:1006-1016.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 1006-1016
-
-
Phan, J.1
Hickey, M.A.2
Zhang, P.3
Chesselet, M.F.4
Reue, K.5
-
48
-
-
82555174637
-
Striatal dysfunctions associated with mitochondrial DNA damage in dopaminergic neurons in a mouse model of Parkinson's disease
-
Pickrell A.M., Pinto M., Hida A., Moraes C.T. Striatal dysfunctions associated with mitochondrial DNA damage in dopaminergic neurons in a mouse model of Parkinson's disease. J. Neurosci. Off. J. Soc. Neurosci. 2011, 31:17649-17658.
-
(2011)
J. Neurosci. Off. J. Soc. Neurosci.
, vol.31
, pp. 17649-17658
-
-
Pickrell, A.M.1
Pinto, M.2
Hida, A.3
Moraes, C.T.4
-
49
-
-
0028029271
-
Deficiency of the human mitochondrial transcription factor h-mtTFA in infantile mitochondrial myopathy is associated with mtDNA depletion
-
Poulton J., Morten K., Freeman-Emmerson C., Potter C., Sewry C., Dubowitz V., Kidd H., Stephenson J., Whitehouse W., Hansen F.J., et al. Deficiency of the human mitochondrial transcription factor h-mtTFA in infantile mitochondrial myopathy is associated with mtDNA depletion. Hum. Mol. Genet. 1994, 3:1763-1769.
-
(1994)
Hum. Mol. Genet.
, vol.3
, pp. 1763-1769
-
-
Poulton, J.1
Morten, K.2
Freeman-Emmerson, C.3
Potter, C.4
Sewry, C.5
Dubowitz, V.6
Kidd, H.7
Stephenson, J.8
Whitehouse, W.9
Hansen, F.J.10
-
50
-
-
14844328611
-
Tissue-specific regulation of metabolic pathways through the transcriptional coactivator PGC1-alpha
-
Puigserver P. Tissue-specific regulation of metabolic pathways through the transcriptional coactivator PGC1-alpha. Int. J. Obes. (Lond) 2005, 29(Suppl. 1):S5-S9.
-
(2005)
Int. J. Obes. (Lond)
, vol.29
, pp. S5-S9
-
-
Puigserver, P.1
-
51
-
-
0032549811
-
A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis
-
Puigserver P., Wu Z., Park C.W., Graves R., Wright M., Spiegelman B.M. A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis. Cell 1998, 92:829-839.
-
(1998)
Cell
, vol.92
, pp. 829-839
-
-
Puigserver, P.1
Wu, Z.2
Park, C.W.3
Graves, R.4
Wright, M.5
Spiegelman, B.M.6
-
52
-
-
70349422148
-
Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease
-
Quintanilla R.A., Johnson G.V. Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease. Brain Res. Bull. 2009, 80:242-247.
-
(2009)
Brain Res. Bull.
, vol.80
, pp. 242-247
-
-
Quintanilla, R.A.1
Johnson, G.V.2
-
53
-
-
67349247037
-
Mitochondrial structural and functional dynamics in Huntington's disease
-
Reddy P.H., Mao P., Manczak M. Mitochondrial structural and functional dynamics in Huntington's disease. Brain Res. Rev. 2009, 61:33-48.
-
(2009)
Brain Res. Rev.
, vol.61
, pp. 33-48
-
-
Reddy, P.H.1
Mao, P.2
Manczak, M.3
-
54
-
-
78650031174
-
Huntington's disease: from molecular pathogenesis to clinical treatment
-
Ross C.A., Tabrizi S.J. Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol. 2011, 10:83-98.
-
(2011)
Lancet Neurol.
, vol.10
, pp. 83-98
-
-
Ross, C.A.1
Tabrizi, S.J.2
-
55
-
-
35848953648
-
Dyneins, autophagy, aggregation and neurodegeneration
-
Rubinsztein D.C., Ravikumar B., Acevedo-Arozena A., Imarisio S., O'Kane C.J., Brown S.D. Dyneins, autophagy, aggregation and neurodegeneration. Autophagy 2005, 1:177-178.
-
(2005)
Autophagy
, vol.1
, pp. 177-178
-
-
Rubinsztein, D.C.1
Ravikumar, B.2
Acevedo-Arozena, A.3
Imarisio, S.4
O'Kane, C.J.5
Brown, S.D.6
-
56
-
-
23044486663
-
Acute intranigral infusion of rotenone in rats causes progressive biochemical lesions in the striatum similar to Parkinson's disease
-
Saravanan K.S., Sindhu K.M., Mohanakumar K.P. Acute intranigral infusion of rotenone in rats causes progressive biochemical lesions in the striatum similar to Parkinson's disease. Brain Res. 2005, 1049:147-155.
-
(2005)
Brain Res.
, vol.1049
, pp. 147-155
-
-
Saravanan, K.S.1
Sindhu, K.M.2
Mohanakumar, K.P.3
-
58
-
-
0036566855
-
Modulation of mitochondrial transcription in response to mtDNA depletion and repletion in HeLa cells
-
Seidel-Rogol B.L., Shadel G.S. Modulation of mitochondrial transcription in response to mtDNA depletion and repletion in HeLa cells. Nucleic Acids Res. 2002, 30:1929-1934.
-
(2002)
Nucleic Acids Res.
, vol.30
, pp. 1929-1934
-
-
Seidel-Rogol, B.L.1
Shadel, G.S.2
-
59
-
-
33847302564
-
Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space
-
Strehlow A.N., Li J.Z., Myers R.M. Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space. Hum. Mol. Genet. 2007, 16:391-409.
-
(2007)
Hum. Mol. Genet.
, vol.16
, pp. 391-409
-
-
Strehlow, A.N.1
Li, J.Z.2
Myers, R.M.3
-
60
-
-
33751348377
-
The human mitochondrial genome: mechanisms of expression and maintenance
-
Chapter 3, CRC Press/Taylor & Francis Group, Boca Raton, ISBN: 0824754425. C.D. Berdanier (Ed.)
-
Taanman J.-W., Williams S.L. The human mitochondrial genome: mechanisms of expression and maintenance. Mitochondria in health and disease. Oxidative stress and disease series. Series edited by Packer,L., Cadenas.E. 2005, 95-246. Chapter 3, CRC Press/Taylor & Francis Group, Boca Raton, ISBN: 0824754425. C.D. Berdanier (Ed.).
-
(2005)
Mitochondria in health and disease. Oxidative stress and disease series. Series edited by Packer,L., Cadenas.E.
, pp. 95-246
-
-
Taanman, J.-W.1
Williams, S.L.2
-
61
-
-
0032900574
-
Biochemical abnormalities and excitotoxicity in Huntington's disease brain
-
Tabrizi S.J., Cleeter M.W., Xuereb J., Taanman J.W., Cooper J.M., Schapira A.H. Biochemical abnormalities and excitotoxicity in Huntington's disease brain. Ann. Neurol. 1999, 45:25-32.
-
(1999)
Ann. Neurol.
, vol.45
, pp. 25-32
-
-
Tabrizi, S.J.1
Cleeter, M.W.2
Xuereb, J.3
Taanman, J.W.4
Cooper, J.M.5
Schapira, A.H.6
-
62
-
-
0035984909
-
Regulation of mitochondrial D-loops by transcription factor A and single-stranded DNA-binding protein
-
Takamatsu C., Umeda S., Ohsato T., Ohno T., Abe Y., Fukuoh A., Shinagawa H., Hamasaki N., Kang D. Regulation of mitochondrial D-loops by transcription factor A and single-stranded DNA-binding protein. EMBO Rep. 2002, 3:451-456.
-
(2002)
EMBO Rep.
, vol.3
, pp. 451-456
-
-
Takamatsu, C.1
Umeda, S.2
Ohsato, T.3
Ohno, T.4
Abe, Y.5
Fukuoh, A.6
Shinagawa, H.7
Hamasaki, N.8
Kang, D.9
-
63
-
-
77954206550
-
The role of mitochondrial DNA copy number in mammalian fertility
-
Wai T., Ao A., Zhang X., Cyr D., Dufort D., Shoubridge E.A. The role of mitochondrial DNA copy number in mammalian fertility. Biol. Reprod. 2010, 83:52-62.
-
(2010)
Biol. Reprod.
, vol.83
, pp. 52-62
-
-
Wai, T.1
Ao, A.2
Zhang, X.3
Cyr, D.4
Dufort, D.5
Shoubridge, E.A.6
-
64
-
-
33750437278
-
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration
-
Weydt P., Pineda V.V., Torrence A.E., Libby R.T., Satterfield T.F., Lazarowski E.R., Gilbert M.L., Morton G.J., Bammler T.K., Strand A.D., Cui L., Beyer R.P., Easley C.N., Smith A.C., Krainc D., Luquet S., Sweet I.R., Schwartz M.W., La Spada A.R. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab. 2006, 4:349-362.
-
(2006)
Cell Metab.
, vol.4
, pp. 349-362
-
-
Weydt, P.1
Pineda, V.V.2
Torrence, A.E.3
Libby, R.T.4
Satterfield, T.F.5
Lazarowski, E.R.6
Gilbert, M.L.7
Morton, G.J.8
Bammler, T.K.9
Strand, A.D.10
Cui, L.11
Beyer, R.P.12
Easley, C.N.13
Smith, A.C.14
Krainc, D.15
Luquet, S.16
Sweet, I.R.17
Schwartz, M.W.18
La Spada, A.R.19
-
65
-
-
2042432480
-
Cytochrome oxidase from beef heart mitochondria
-
Wharton D.C., Tzagoloff A. Cytochrome oxidase from beef heart mitochondria. Methods Enzymol. 1967, 10:245-250.
-
(1967)
Methods Enzymol.
, vol.10
, pp. 245-250
-
-
Wharton, D.C.1
Tzagoloff, A.2
-
66
-
-
66149110042
-
A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for Huntington disease
-
Zabel C., Mao L., Woodman B., Rohe M., Wacker M.A., Kläre Y., Koppelstätter A., Nebrich G., Klein O., Grams S., Strand A., Luthi-Carter R., Hartl D., Klose J., Bates G.P. A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for Huntington disease. Mol. Cell. Proteomics 2009, 8(4):720-734.
-
(2009)
Mol. Cell. Proteomics
, vol.8
, Issue.4
, pp. 720-734
-
-
Zabel, C.1
Mao, L.2
Woodman, B.3
Rohe, M.4
Wacker, M.A.5
Kläre, Y.6
Koppelstätter, A.7
Nebrich, G.8
Klein, O.9
Grams, S.10
Strand, A.11
Luthi-Carter, R.12
Hartl, D.13
Klose, J.14
Bates, G.P.15
|