-
1
-
-
0035833937
-
Immune reactivity in a mouse model of familial ALS correlates with disease progression
-
CrossRef Medline
-
Alexianu ME, Kozovska M, Appel SH (2001) Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology 57: 1282-1289. CrossRef Medline
-
(2001)
Neurology
, vol.57
, pp. 1282-1289
-
-
Alexianu, M.E.1
Kozovska, M.2
Appel, S.H.3
-
2
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
CrossRef Medline
-
Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S (2004) Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431:805-810. CrossRef Medline
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
3
-
-
77953131418
-
The mitochondrial permeability transition from yeast to mammals
-
CrossRef Medline
-
Azzolin L, von Stockum S, Basso E, Petronilli V, Forte MA, Bernardi P (2010) The mitochondrial permeability transition from yeast to mammals. FEBS Lett 584:2504-2509. CrossRef Medline
-
(2010)
FEBS Lett
, vol.584
, pp. 2504-2509
-
-
Azzolin, L.1
von Stockum, S.2
Basso, E.3
Petronilli, V.4
Forte, M.A.5
Bernardi, P.6
-
4
-
-
15844375853
-
Loss of cyclophilin D reveals a critical role for mitochondrial permeability transition in cell death
-
CrossRef Medline
-
Baines CP, Kaiser RA, Purcell NH, Blair NS, Osinska H, Hambleton MA, Brunskill EW, Sayen MR, Gottlieb RA, Dorn GW, Robbins J, Molkentin JD (2005) Loss of cyclophilin D reveals a critical role for mitochondrial permeability transition in cell death. Nature 434:658-662. CrossRef Medline
-
(2005)
Nature
, vol.434
, pp. 658-662
-
-
Baines, C.P.1
Kaiser, R.A.2
Purcell, N.H.3
Blair, N.S.4
Osinska, H.5
Hambleton, M.A.6
Brunskill, E.W.7
Sayen, M.R.8
Gottlieb, R.A.9
Dorn, G.W.10
Robbins, J.11
Molkentin, J.D.12
-
5
-
-
78751565419
-
Axonal degeneration is mediated by the mitochondrial permeability transition pore
-
CrossRef Medline
-
Barrientos SA, Martinez NW, Yoo S, Jara JS, Zamorano S, Hetz C, Twiss JL, Alvarez J, Court FA (2011) Axonal degeneration is mediated by the mitochondrial permeability transition pore. J Neurosci 31:966-978. CrossRef Medline
-
(2011)
J Neurosci
, vol.31
, pp. 966-978
-
-
Barrientos, S.A.1
Martinez, N.W.2
Yoo, S.3
Jara, J.S.4
Zamorano, S.5
Hetz, C.6
Twiss, J.L.7
Alvarez, J.8
Court, F.A.9
-
6
-
-
79952444039
-
Activation of the mitochondrial permeability transition pore modulates Ca2+ responses to physiological stimuli in adult neurons
-
CrossRef Medline
-
Barsukova A, Komarov A, Hajnóczky G, Bernardi P, Bourdette D, Forte M (2011) Activation of the mitochondrial permeability transition pore modulates Ca2+ responses to physiological stimuli in adult neurons. Eur J Neurosci 33:831-842. CrossRef Medline
-
(2011)
Eur J Neurosci
, vol.33
, pp. 831-842
-
-
Barsukova, A.1
Komarov, A.2
Hajnóczky, G.3
Bernardi, P.4
Bourdette, D.5
Forte, M.6
-
7
-
-
21244446551
-
Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D
-
CrossRef Medline
-
Basso E, Fante L, Fowlkes J, Petronilli V, Forte MA, Bernardi P (2005) Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D. J Biol Chem 280:18558-18561. CrossRef Medline
-
(2005)
J Biol Chem
, vol.280
, pp. 18558-18561
-
-
Basso, E.1
Fante, L.2
Fowlkes, J.3
Petronilli, V.4
Forte, M.A.5
Bernardi, P.6
-
8
-
-
0034763566
-
Parvalbumin overexpression alters immune-mediated increases in intracellular calcium, and delays disease onset in a transgenic model of familial amyotrophic lateral sclerosis
-
Medline
-
Beers DR, Ho BK, Siklós L, Alexianu ME, Mosier DR, Mohamed AH, Otsuka Y, Kozovska ME, McAlhany RE, Smith RG, Appel SH (2001) Parvalbumin overexpression alters immune-mediated increases in intracellular calcium, and delays disease onset in a transgenic model of familial amyotrophic lateral sclerosis. J Neurochem 79:499-509. Medline
-
(2001)
J Neurochem
, vol.79
, pp. 499-509
-
-
Beers, D.R.1
Ho, B.K.2
Siklós, L.3
Alexianu, M.E.4
Mosier, D.R.5
Mohamed, A.H.6
Otsuka, Y.7
Kozovska, M.E.8
McAlhany, R.E.9
Smith, R.G.10
Appel, S.H.11
-
9
-
-
33750478657
-
Wild-type microglia extend survival in PU. 1 knock-out mice with familial amyotrophic lateral sclerosis
-
CrossRef Medline
-
Beers DR, Henkel JS, Xiao Q, Zhao W, Wang J, Yen AA, Siklos L, McKercher SR, Appel SH (2006) Wild-type microglia extend survival in PU. 1 knock-out mice with familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 103:16021-16026. CrossRef Medline
-
(2006)
Proc Natl Acad Sci U S A
, vol.103
, pp. 16021-16026
-
-
Beers, D.R.1
Henkel, J.S.2
Xiao, Q.3
Zhao, W.4
Wang, J.5
Yen, A.A.6
Siklos, L.7
McKercher, S.R.8
Appel, S.H.9
-
10
-
-
33646452761
-
Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models
-
CrossRef Medline
-
Bergemalm D, Jonsson PA, Graffmo KS, Andersen PM, Brännström T, Rehnmark A, Marklund SL (2006) Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models. J Neurosci 26:4147-4154. CrossRef Medline
-
(2006)
J Neurosci
, vol.26
, pp. 4147-4154
-
-
Bergemalm, D.1
Jonsson, P.A.2
Graffmo, K.S.3
Andersen, P.M.4
Brännström, T.5
Rehnmark, A.6
Marklund, S.L.7
-
11
-
-
0032827410
-
Mitochondrial transport of cations: Channels, exchangers, and permeability transition
-
Medline
-
Bernardi P (1999) Mitochondrial transport of cations: channels, exchangers, and permeability transition. Physiol Rev 79:1127-1155. Medline
-
(1999)
Physiol Rev
, vol.79
, pp. 1127-1155
-
-
Bernardi, P.1
-
12
-
-
33646255246
-
The mitochondrial permeability transition from in vitro artifact to disease target
-
CrossRef Medline
-
Bernardi P, Krauskopf A, Basso E, Petronilli V, Blachly-Dyson E, Di Lisa F, Forte MA (2006) The mitochondrial permeability transition from in vitro artifact to disease target. FEBS J 273:2077-2099. CrossRef Medline
-
(2006)
FEBS J
, vol.273
, pp. 2077-2099
-
-
Bernardi, P.1
Krauskopf, A.2
Basso, E.3
Petronilli, V.4
Blachly-Dyson, E.5
Di Lisa, F.6
Forte, M.A.7
-
13
-
-
33749056809
-
ALS: A disease of motor neurons and their non-neuronal neighbors
-
CrossRef Medline
-
Boillée S, Vande Velde C, Cleveland DW (2006a) ALS: a disease of motor neurons and their non-neuronal neighbors. Neuron 52:39-59. CrossRef Medline
-
(2006)
Neuron
, vol.52
, pp. 39-59
-
-
Boillée, S.1
Vande Velde, C.2
Cleveland, D.W.3
-
14
-
-
33744798774
-
Onset and progression in inherited ALS determined by motor neurons and microglia
-
CrossRef Medline
-
Boillée S, Yamanaka K, Lobsiger CS, Copeland NG, Jenkins NA, Kassiotis G, Kollias G, Cleveland DW (2006b) Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312: 1389-1392. CrossRef Medline
-
(2006)
Science
, vol.312
, pp. 1389-1392
-
-
Boillée, S.1
Yamanaka, K.2
Lobsiger, C.S.3
Copeland, N.G.4
Jenkins, N.A.5
Kassiotis, G.6
Kollias, G.7
Cleveland, D.W.8
-
15
-
-
77958519939
-
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
-
CrossRef Medline
-
Bosco DA, Morfini G, Karabacak NM, Song Y, Gros-Louis F, Pasinelli P, Goolsby H, Fontaine BA, Lemay N, McKenna-Yasek D, Frosch MP, Agar JN, Julien JP, Brady ST, Brown RH Jr (2010) Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci 13:1396-1403. CrossRef Medline
-
(2010)
Nat Neurosci
, vol.13
, pp. 1396-1403
-
-
Bosco, D.A.1
Morfini, G.2
Karabacak, N.M.3
Song, Y.4
Gros-Louis, F.5
Pasinelli, P.6
Goolsby, H.7
Fontaine, B.A.8
Lemay, N.9
McKenna-Yasek, D.10
Frosch, M.P.11
Agar, J.N.12
Julien, J.P.13
Brady, S.T.14
Brown Jr., R.H.15
-
16
-
-
0031051485
-
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
-
CrossRef Medline
-
Bruijn LI, Becher MW, Lee MK, Anderson KL, Jenkins NA, Copeland NG, Sisodia SS, Rothstein JD, Borchelt DR, Price DL, Cleveland DW (1997) ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18:327-338. CrossRef Medline
-
(1997)
Neuron
, vol.18
, pp. 327-338
-
-
Bruijn, L.I.1
Becher, M.W.2
Lee, M.K.3
Anderson, K.L.4
Jenkins, N.A.5
Copeland, N.G.6
Sisodia, S.S.7
Rothstein, J.D.8
Borchelt, D.R.9
Price, D.L.10
Cleveland, D.W.11
-
17
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wildtype SOD1
-
CrossRef Medline
-
Bruijn LI, Houseweart MK, Kato S, Anderson KL, Anderson SD, Ohama E, Reaume AG, Scott RW, Cleveland DW (1998) Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wildtype SOD1. Science 281:1851-1854. CrossRef Medline
-
(1998)
Science
, vol.281
, pp. 1851-1854
-
-
Bruijn, L.I.1
Houseweart, M.K.2
Kato, S.3
Anderson, K.L.4
Anderson, S.D.5
Ohama, E.6
Reaume, A.G.7
Scott, R.W.8
Cleveland, D.W.9
-
18
-
-
42949166848
-
Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: Prevention by mitochondrial-targeted antioxidants
-
CrossRef Medline
-
Cassina P, Cassina A, Pehar M, Castellanos R, Gandelman M, de León A, Robinson KM, Mason RP, Beckman JS, Barbeito L, Radi R (2008) Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidants. J Neurosci 28:4115-4122. CrossRef Medline
-
(2008)
J Neurosci
, vol.28
, pp. 4115-4122
-
-
Cassina, P.1
Cassina, A.2
Pehar, M.3
Castellanos, R.4
Gandelman, M.5
de León, A.6
Robinson, K.M.7
Mason, R.P.8
Beckman, J.S.9
Barbeito, L.10
Radi, R.11
-
19
-
-
33645102302
-
Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice
-
CrossRef Medline
-
Damiano M, Starkov AA, Petri S, Kipiani K, Kiaei M, Mattiazzi M, Flint Beal M, Manfredi G (2006) Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice. J Neurochem 96:1349-1361. CrossRef Medline
-
(2006)
J Neurochem
, vol.96
, pp. 1349-1361
-
-
Damiano, M.1
Starkov, A.A.2
Petri, S.3
Kipiani, K.4
Kiaei, M.5
Mattiazzi, M.6
Flint Beal, M.7
Manfredi, G.8
-
20
-
-
0027426169
-
Amyotrophic lateral sclerosis and structural defects in Cu, Zn superoxide dismutase
-
CrossRef Medline
-
Deng HX, Hentati A, Tainer JA, Iqbal Z, Cayabyab A, Hung WY, Getzoff ED, Hu P, Herzfeldt B, Roos RP (1993) Amyotrophic lateral sclerosis and structural defects in Cu, Zn superoxide dismutase. Science 261: 1047-1051. CrossRef Medline
-
(1993)
Science
, vol.261
, pp. 1047-1051
-
-
Deng, H.X.1
Hentati, A.2
Tainer, J.A.3
Iqbal, Z.4
Cayabyab, A.5
Hung, W.Y.6
Getzoff, E.D.7
Hu, P.8
Herzfeldt, B.9
Roos, R.P.10
-
21
-
-
33646466296
-
Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria
-
CrossRef Medline
-
Deng HX, Shi Y, Furukawa Y, Zhai H, Fu R, Liu E, Gorrie GH, Khan MS, Hung WY, Bigio EH, Lukas T, Dal Canto MC, O'Halloran TV, Siddique T (2006) Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A 103:7142-7147. CrossRef Medline
-
(2006)
Proc Natl Acad Sci U S A
, vol.103
, pp. 7142-7147
-
-
Deng, H.X.1
Shi, Y.2
Furukawa, Y.3
Zhai, H.4
Fu, R.5
Liu, E.6
Gorrie, G.H.7
Khan, M.S.8
Hung, W.Y.9
Bigio, E.H.10
Lukas, T.11
Dal Canto, M.C.12
O'Halloran, T.V.13
Siddique, T.14
-
22
-
-
34247473080
-
Noncell autonomous effect of glia on motor neurons in an embryonic stem cellbased ALS model
-
CrossRef Medline
-
Di Giorgio FP, Carrasco MA, Siao MC, Maniatis T, Eggan K (2007) Noncell autonomous effect of glia on motor neurons in an embryonic stem cellbased ALS model. Nat Neurosci 10:608-614. CrossRef Medline
-
(2007)
Nat Neurosci
, vol.10
, pp. 608-614
-
-
Di Giorgio, F.P.1
Carrasco, M.A.2
Siao, M.C.3
Maniatis, T.4
Eggan, K.5
-
23
-
-
53549129483
-
Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease
-
CrossRef Medline
-
Du H, Guo L, Fang F, Chen D, Sosunov AA, McKhann GM, Yan Y, Wang C, Zhang H, Molkentin JD, Gunn-Moore FJ, Vonsattel JP, Arancio O, Chen JX, Yan SD (2008) Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease. Nat Med 14:1097-1105. CrossRef Medline
-
(2008)
Nat Med
, vol.14
, pp. 1097-1105
-
-
Du, H.1
Guo, L.2
Fang, F.3
Chen, D.4
Sosunov, A.A.5
McKhann, G.M.6
Yan, Y.7
Wang, C.8
Zhang, H.9
Molkentin, J.D.10
Gunn-Moore, F.J.11
Vonsattel, J.P.12
Arancio, O.13
Chen, J.X.14
Yan, S.D.15
-
24
-
-
0030777650
-
Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS
-
CrossRef Medline
-
Durham HD, Roy J, Dong L, Figlewicz DA (1997) Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS. J Neuropathol Exp Neurol 56:523-530. CrossRef Medline
-
(1997)
J Neuropathol Exp Neurol
, vol.56
, pp. 523-530
-
-
Durham, H.D.1
Roy, J.2
Dong, L.3
Figlewicz, D.A.4
-
25
-
-
0024994099
-
Ig G reactivity in the spinal cord and motor cortex in amyotrophic lateral sclerosis
-
CrossRef Medline
-
Engelhardt JI, Appel SH (1990) Ig G reactivity in the spinal cord and motor cortex in amyotrophic lateral sclerosis. Arch Neurol 47:1210-1216. CrossRef Medline
-
(1990)
Arch Neurol
, vol.47
, pp. 1210-1216
-
-
Engelhardt, J.I.1
Appel, S.H.2
-
26
-
-
20244390181
-
The Wld S gene modestly prolongs survival in the SOD1G93A fALS mouse
-
CrossRef Medline
-
Fischer LR, Culver DG, Davis AA, Tennant P, Wang M, Coleman M, Asress S, Adalbert R, Alexander GM, Glass JD (2005) The Wld S gene modestly prolongs survival in the SOD1G93A fALS mouse. Neurobiol Dis 19:293-300. CrossRef Medline
-
(2005)
Neurobiol Dis
, vol.19
, pp. 293-300
-
-
Fischer, L.R.1
Culver, D.G.2
Davis, A.A.3
Tennant, P.4
Wang, M.5
Coleman, M.6
Asress, S.7
Adalbert, R.8
Alexander, G.M.9
Glass, J.D.10
-
27
-
-
33748261830
-
Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS
-
CrossRef Medline
-
Gould TW, Buss RR, Vinsant S, Prevette D, Sun W, Knudson CM, Milligan CE, Oppenheim RW (2006) Complete dissociation of motor neuron death from motor dysfunction by Bax deletion in a mouse model of ALS. J Neurosci 26:8774-8786. CrossRef Medline
-
(2006)
J Neurosci
, vol.26
, pp. 8774-8786
-
-
Gould, T.W.1
Buss, R.R.2
Vinsant, S.3
Prevette, D.4
Sun, W.5
Knudson, C.M.6
Milligan, C.E.7
Oppenheim, R.W.8
-
28
-
-
80053652133
-
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
-
CrossRef Medline
-
Grad LI, Guest WC, Yanai A, Pokrishevsky E, O'Neill MA, Gibbs E, Semenchenko V, Yousefi M, Wishart DS, Plotkin SS, Cashman NR (2011) Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A 108:16398-16403. CrossRef Medline
-
(2011)
Proc Natl Acad Sci U S A
, vol.108
, pp. 16398-16403
-
-
Grad, L.I.1
Guest, W.C.2
Yanai, A.3
Pokrishevsky, E.4
O'Neill, M.A.5
Gibbs, E.6
Semenchenko, V.7
Yousefi, M.8
Wishart, D.S.9
Plotkin, S.S.10
Cashman, N.R.11
-
29
-
-
77951924183
-
Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS
-
Medline
-
Gros-Louis F, Soucy G, Larivière R, Julien JP (2010) Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS. J Neurochem 113:1188-1199. Medline
-
(2010)
J Neurochem
, vol.113
, pp. 1188-1199
-
-
Gros-Louis, F.1
Soucy, G.2
Larivière, R.3
Julien, J.P.4
-
30
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation
-
CrossRef Medline
-
Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264:1772-1775. CrossRef Medline
-
(1994)
Science
, vol.264
, pp. 1772-1775
-
-
Gurney, M.E.1
Pu, H.2
Chiu, A.Y.3
Dal Canto, M.C.4
Polchow, C.Y.5
Alexander, D.D.6
Caliendo, J.7
Hentati, A.8
Kwon, Y.W.9
Deng, H.X.10
-
31
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Medline
-
Gutekunst CA, Li SH, Yi H, Mulroy JS, Kuemmerle S, Jones R, Rye D, Ferrante RJ, Hersch SM, Li XJ (1999) Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci 19:2522-2534. Medline
-
(1999)
J Neurosci
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
Mulroy, J.S.4
Kuemmerle, S.5
Jones, R.6
Rye, D.7
Ferrante, R.J.8
Hersch, S.M.9
Li, X.J.10
-
32
-
-
0032125587
-
Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS
-
CrossRef Medline
-
Hall ED, Oostveen JA, Gurney ME (1998) Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS. Glia 23:249-256. CrossRef Medline
-
(1998)
Glia
, vol.23
, pp. 249-256
-
-
Hall, E.D.1
Oostveen, J.A.2
Gurney, M.E.3
-
33
-
-
38849182472
-
SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model
-
Medline
-
Harraz MM, Marden JJ, Zhou W, Zhang Y, Williams A, Sharov VS, Nelson K, Luo M, Paulson H, Schöneich C, Engelhardt JF (2008) SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest 118:659-670. Medline
-
(2008)
J Clin Invest
, vol.118
, pp. 659-670
-
-
Harraz, M.M.1
Marden, J.J.2
Zhou, W.3
Zhang, Y.4
Williams, A.5
Sharov, V.S.6
Nelson, K.7
Luo, M.8
Paulson, H.9
Schöneich, C.10
Engelhardt, J.F.11
-
34
-
-
10744229158
-
Presence of dendritic cells, MCP-1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord tissue
-
CrossRef Medline
-
Henkel JS, Engelhardt JI, Siklós L, Simpson EP, Kim SH, Pan T, Goodman JC, Siddique T, Beers DR, Appel SH (2004) Presence of dendritic cells, MCP-1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord tissue. Ann Neurol 55:221-235. CrossRef Medline
-
(2004)
Ann Neurol
, vol.55
, pp. 221-235
-
-
Henkel, J.S.1
Engelhardt, J.I.2
Siklós, L.3
Simpson, E.P.4
Kim, S.H.5
Pan, T.6
Goodman, J.C.7
Siddique, T.8
Beers, D.R.9
Appel, S.H.10
-
35
-
-
0037022339
-
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
-
CrossRef Medline
-
Howland DS, Liu J, She Y, Goad B, Maragakis NJ, Kim B, Erickson J, Kulik J, DeVito L, Psaltis G, DeGennaro LJ, Cleveland DW, Rothstein JD (2002) Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A 99:1604-1609. CrossRef Medline
-
(2002)
Proc Natl Acad Sci U S A
, vol.99
, pp. 1604-1609
-
-
Howland, D.S.1
Liu, J.2
She, Y.3
Goad, B.4
Maragakis, N.J.5
Kim, B.6
Erickson, J.7
Kulik, J.8
DeVito, L.9
Psaltis, G.10
DeGennaro, L.J.11
Cleveland, D.W.12
Rothstein, J.D.13
-
36
-
-
0018332596
-
The Ca2+-induced membrane transition in mitochondria. I. The protective mechanisms
-
CrossRef Medline
-
Hunter DR, Haworth RA (1979) The Ca2+-induced membrane transition in mitochondria. I. The protective mechanisms. Arch Biochem Biophys 195:453-459. CrossRef Medline
-
(1979)
Arch Biochem Biophys
, vol.195
, pp. 453-459
-
-
Hunter, D.R.1
Haworth, R.A.2
-
37
-
-
74049164709
-
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
-
CrossRef Medline
-
Ilieva H, Polymenidou M, Cleveland DW (2009) Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol 187: 761-772. CrossRef Medline
-
(2009)
J Cell Biol
, vol.187
, pp. 761-772
-
-
Ilieva, H.1
Polymenidou, M.2
Cleveland, D.W.3
-
38
-
-
77955961922
-
Misfolded mutant SOD1 directly inhibits VDAC1conductance in a mouse model of inherited ALS
-
CrossRef Medline
-
Israelson A, Arbel N, Da Cruz S, Ilieva H, Yamanaka K, Shoshan-Barmatz V, Cleveland DW (2010) Misfolded mutant SOD1 directly inhibits VDAC1conductance in a mouse model of inherited ALS. Neuron 67:575-587. CrossRef Medline
-
(2010)
Neuron
, vol.67
, pp. 575-587
-
-
Israelson, A.1
Arbel, N.2
da Cruz, S.3
Ilieva, H.4
Yamanaka, K.5
Shoshan-Barmatz, V.6
Cleveland, D.W.7
-
39
-
-
39849103473
-
Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice
-
CrossRef Medline
-
Jaarsma D, Teuling E, Haasdijk ED, De Zeeuw CI, Hoogenraad CC (2008) Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. J Neurosci 28:2075-2088. CrossRef Medline
-
(2008)
J Neurosci
, vol.28
, pp. 2075-2088
-
-
Jaarsma, D.1
Teuling, E.2
Haasdijk, E.D.3
de Zeeuw, C.I.4
Hoogenraad, C.C.5
-
40
-
-
62149094382
-
Cu/Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of mitochondria and perturbs Ca2+ homeostasis in SOD1G93A mice
-
CrossRef Medline
-
Jaiswal MK, Keller BU (2009) Cu/Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of mitochondria and perturbs Ca2+ homeostasis in SOD1G93A mice. Mol Pharmacol 75:478-489. CrossRef Medline
-
(2009)
Mol Pharmacol
, vol.75
, pp. 478-489
-
-
Jaiswal, M.K.1
Keller, B.U.2
-
41
-
-
0033749379
-
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
-
CrossRef Medline
-
Johnston JA, Dalton MJ, Gurney ME, Kopito RR (2000) Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 97:12571-12576. CrossRef Medline
-
(2000)
Proc Natl Acad Sci U S A
, vol.97
, pp. 12571-12576
-
-
Johnston, J.A.1
Dalton, M.J.2
Gurney, M.E.3
Kopito, R.R.4
-
42
-
-
66049156169
-
Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS
-
CrossRef Medline
-
Karch CM, Prudencio M, Winkler DD, Hart PJ, Borchelt DR (2009) Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS. Proc Natl Acad Sci U S A 106:7774-7779. CrossRef Medline
-
(2009)
Proc Natl Acad Sci U S A
, vol.106
, pp. 7774-7779
-
-
Karch, C.M.1
Prudencio, M.2
Winkler, D.D.3
Hart, P.J.4
Borchelt, D.R.5
-
43
-
-
77956183828
-
Mitochondrial dysfunction and intracellular calcium dysregulation in ALS
-
CrossRef Medline
-
Kawamata H, Manfredi G (2010) Mitochondrial dysfunction and intracellular calcium dysregulation in ALS. Mech Ageing Dev 131:517-526. CrossRef Medline
-
(2010)
Mech Ageing Dev
, vol.131
, pp. 517-526
-
-
Kawamata, H.1
Manfredi, G.2
-
44
-
-
77953028624
-
Amyotrophic lateral sclerosis is a nonamyloid disease in which extensive misfolding of SOD1 is unique to the familial form
-
CrossRef Medline
-
Kerman A, Liu HN, Croul S, Bilbao J, Rogaeva E, Zinman L, Robertson J, Chakrabartty A (2010) Amyotrophic lateral sclerosis is a nonamyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathol 119:335-344. CrossRef Medline
-
(2010)
Acta Neuropathol
, vol.119
, pp. 335-344
-
-
Kerman, A.1
Liu, H.N.2
Croul, S.3
Bilbao, J.4
Rogaeva, E.5
Zinman, L.6
Robertson, J.7
Chakrabartty, A.8
-
45
-
-
33645798615
-
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
-
CrossRef Medline
-
Kikuchi H, Almer G, Yamashita S, Guégan C, Nagai M, Xu Z, Sosunov AA, McKhann GM 2nd, Przedborski S (2006) Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc Natl Acad Sci U S A 103: 6025-6030. CrossRef Medline
-
(2006)
Proc Natl Acad Sci U S A
, vol.103
, pp. 6025-6030
-
-
Kikuchi, H.1
Almer, G.2
Yamashita, S.3
Guégan, C.4
Nagai, M.5
Xu, Z.6
Sosunov, A.A.7
McKhann III, G.M.8
Przedborski, S.9
-
46
-
-
0037007967
-
Alteration in intracellular calcium homeostasis reduces motor neuronal viability expressing mutated Cu/Zn superoxide dismutase through a nitric oxide/guanylyl cyclase cGMP cascade
-
CrossRef Medline
-
Kim HJ, Kim M, Kim SH, Sung JJ, Lee KW (2002) Alteration in intracellular calcium homeostasis reduces motor neuronal viability expressing mutated Cu/Zn superoxide dismutase through a nitric oxide/guanylyl cyclase cGMP cascade. Neuroreport 13:1131-1135. CrossRef Medline
-
(2002)
Neuroreport
, vol.13
, pp. 1131-1135
-
-
Kim, H.J.1
Kim, M.2
Kim, S.H.3
Sung, J.J.4
Lee, K.W.5
-
47
-
-
84866354854
-
The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogenmediated neuroprotection in amyotrophic lateral sclerosis
-
CrossRef Medline
-
Kim HJ, Magran è J, Starkov AA, Manfredi G (2012) The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogenmediated neuroprotection in amyotrophic lateral sclerosis. Brain 135: 2865-2874. CrossRef Medline
-
(2012)
Brain
, vol.135
, pp. 2865-2874
-
-
Kim, H.J.1
Magran è, J.2
Starkov, A.A.3
Manfredi, G.4
-
48
-
-
0036708168
-
Paradigm shifts in Alzheimer's disease and other neurodegenerative disorders: The emerging role of oligomeric assemblies
-
CrossRef Medline
-
Kirkitadze MD, Bitan G, Teplow DB (2002) Paradigm shifts in Alzheimer's disease and other neurodegenerative disorders: the emerging role of oligomeric assemblies. J Neurosci Res 69:567-577. CrossRef Medline
-
(2002)
J Neurosci Res
, vol.69
, pp. 567-577
-
-
Kirkitadze, M.D.1
Bitan, G.2
Teplow, D.B.3
-
49
-
-
0025868659
-
Reactive astrogliosis is widespread in the subcortical white matter of amyotrophic lateral sclerosis brain
-
CrossRef Medline
-
Kushner PD, Stephenson DT, Wright S (1991) Reactive astrogliosis is widespread in the subcortical white matter of amyotrophic lateral sclerosis brain. J Neuropathol Exp Neurol 50:263-277. CrossRef Medline
-
(1991)
J Neuropathol Exp Neurol
, vol.50
, pp. 263-277
-
-
Kushner, P.D.1
Stephenson, D.T.2
Wright, S.3
-
50
-
-
0033485920
-
Astrocytes interact intimately with degenerating motor neurons in mouse amyotrophic lateral sclerosis (ALS)
-
CrossRef Medline
-
Levine JB, Kong J, Nadler M, Xu Z (1999) Astrocytes interact intimately with degenerating motor neurons in mouse amyotrophic lateral sclerosis (ALS). Glia 28:215-224. CrossRef Medline
-
(1999)
Glia
, vol.28
, pp. 215-224
-
-
Levine, J.B.1
Kong, J.2
Nadler, M.3
Xu, Z.4
-
51
-
-
78650503526
-
ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import
-
CrossRef Medline
-
Li Q, Vande Velde C, Israelson A, Xie J, Bailey AO, Dong MQ, Chun SJ, Roy T, Winer L, Yates JR, Capaldi RA, Cleveland DW, Miller TM (2010) ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A 107:21146-21151. CrossRef Medline
-
(2010)
Proc Natl Acad Sci U S A
, vol.107
, pp. 21146-21151
-
-
Li, Q.1
Vande Velde, C.2
Israelson, A.3
Xie, J.4
Bailey, A.O.5
Dong, M.Q.6
Chun, S.J.7
Roy, T.8
Winer, L.9
Yates, J.R.10
Capaldi, R.A.11
Cleveland, D.W.12
Miller, T.M.13
-
52
-
-
84855993170
-
Reduced activity of AMP-activated protein kinase protects against genetic models of motor neuron disease
-
CrossRef Medline
-
Lim MA, Selak MA, Xiang Z, Krainc D, Neve RL, Kraemer BC, Watts JL, Kalb RG (2012) Reduced activity of AMP-activated protein kinase protects against genetic models of motor neuron disease. J Neurosci 32:1123-1141. CrossRef Medline
-
(2012)
J Neurosci
, vol.32
, pp. 1123-1141
-
-
Lim, M.A.1
Selak, M.A.2
Xiang, Z.3
Krainc, D.4
Neve, R.L.5
Kraemer, B.C.6
Watts, J.L.7
Kalb, R.G.8
-
53
-
-
84862854446
-
Targeting of monomer/ misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis
-
CrossRef Medline
-
Liu HN, Tjostheim S, Dasilva K, Taylor D, Zhao B, Rakhit R, Brown M, Chakrabartty A, McLaurin J, Robertson J (2012) Targeting of monomer/ misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis. J Neurosci 32:8791-8799. CrossRef Medline
-
(2012)
J Neurosci
, vol.32
, pp. 8791-8799
-
-
Liu, H.N.1
Tjostheim, S.2
Dasilva, K.3
Taylor, D.4
Zhao, B.5
Rakhit, R.6
Brown, M.7
Chakrabartty, A.8
McLaurin, J.9
Robertson, J.10
-
54
-
-
3242701496
-
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
-
CrossRef Medline
-
Liu J, Lillo C, Jonsson PA, Vande Velde C, Ward CM, Miller TM, Subramaniam JR, Rothstein JD, Marklund S, Andersen PM, Brännström T, Gredal O, Wong PC, Williams DS, Cleveland DW (2004) Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 43:5-17. CrossRef Medline
-
(2004)
Neuron
, vol.43
, pp. 5-17
-
-
Liu, J.1
Lillo, C.2
Jonsson, P.A.3
Vande Velde, C.4
Ward, C.M.5
Miller, T.M.6
Subramaniam, J.R.7
Rothstein, J.D.8
Marklund, S.9
Andersen, P.M.10
Brännström, T.11
Gredal, O.12
Wong, P.C.13
Williams, D.S.14
Cleveland, D.W.15
-
55
-
-
18844444198
-
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis
-
CrossRef Medline
-
Liu J, Shinobu LA, Ward CM, Young D, Cleveland DW (2005) Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem 93:875-882. CrossRef Medline
-
(2005)
J Neurochem
, vol.93
, pp. 875-882
-
-
Liu, J.1
Shinobu, L.A.2
Ward, C.M.3
Young, D.4
Cleveland, D.W.5
-
56
-
-
63149141363
-
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice
-
CrossRef Medline
-
Lobsiger CS, Boillee S, McAlonis-Downes M, Khan AM, Feltri ML, Yamanaka K, Cleveland DW (2009) Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A 106:4465-4470. CrossRef Medline
-
(2009)
Proc Natl Acad Sci U S A
, vol.106
, pp. 4465-4470
-
-
Lobsiger, C.S.1
Boillee, S.2
McAlonis-Downes, M.3
Khan, A.M.4
Feltri, M.L.5
Yamanaka, K.6
Cleveland, D.W.7
-
57
-
-
84855931735
-
Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons
-
CrossRef Medline
-
Magran é J, Sahawneh MA, Przedborski S, Estévez ÁG, Manfredi G (2012) Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci 32:229-242. CrossRef Medline
-
(2012)
J Neurosci
, vol.32
, pp. 229-242
-
-
Magran é, J.1
Sahawneh, M.A.2
Przedborski, S.3
Estévez, A.G.4
Manfredi, G.5
-
58
-
-
67649811029
-
The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice
-
CrossRef Medline
-
Martin LJ, Gertz B, Pan Y, Price AC, Molkentin JD, Chang Q (2009) The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice. Exp Neurol 218:333-346. CrossRef Medline
-
(2009)
Exp Neurol
, vol.218
, pp. 333-346
-
-
Martin, L.J.1
Gertz, B.2
Pan, Y.3
Price, A.C.4
Molkentin, J.D.5
Chang, Q.6
-
59
-
-
30344442440
-
Disease progression of human SOD1 (G93A) transgenic ALS model rats
-
CrossRef Medline
-
Matsumoto A, Okada Y, Nakamichi M, Nakamura M, Toyama Y, Sobue G, Nagai M, Aoki M, Itoyama Y, Okano H (2006) Disease progression of human SOD1 (G93A) transgenic ALS model rats. J Neurosci Res 83:119-133. CrossRef Medline
-
(2006)
J Neurosci Res
, vol.83
, pp. 119-133
-
-
Matsumoto, A.1
Okada, Y.2
Nakamichi, M.3
Nakamura, M.4
Toyama, Y.5
Sobue, G.6
Nagai, M.7
Aoki, M.8
Itoyama, Y.9
Okano, H.10
-
60
-
-
0037119407
-
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice
-
CrossRef Medline
-
Mattiazzi M, D'Aurelio M, Gajewski CD, Martushova K, Kiaei M, Beal MF, Manfredi G (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem 277:29626-29633. CrossRef Medline
-
(2002)
J Biol Chem
, vol.277
, pp. 29626-29633
-
-
Mattiazzi, M.1
D'Aurelio, M.2
Gajewski, C.D.3
Martushova, K.4
Kiaei, M.5
Beal, M.F.6
Manfredi, G.7
-
61
-
-
34247475338
-
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
-
CrossRef Medline
-
Nagai M, Re DB, Nagata T, Chalazonitis A, Jessell TM, Wichterle H, Przedborski S (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci 10:615-622. CrossRef Medline
-
(2007)
Nat Neurosci
, vol.10
, pp. 615-622
-
-
Nagai, M.1
Re, D.B.2
Nagata, T.3
Chalazonitis, A.4
Jessell, T.M.5
Wichterle, H.6
Przedborski, S.7
-
62
-
-
15844407874
-
Cyclophilin D-dependent mitochondrial permeability transition regulates some necrotic but not apoptotic cell death
-
CrossRef Medline
-
Nakagawa T, Shimizu S, Watanabe T, Yamaguchi O, Otsu K, Yamagata H, Inohara H, Kubo T, Tsujimoto Y (2005) Cyclophilin D-dependent mitochondrial permeability transition regulates some necrotic but not apoptotic cell death. Nature 434:652-658. CrossRef Medline
-
(2005)
Nature
, vol.434
, pp. 652-658
-
-
Nakagawa, T.1
Shimizu, S.2
Watanabe, T.3
Yamaguchi, O.4
Otsu, K.5
Yamagata, H.6
Inohara, H.7
Kubo, T.8
Tsujimoto, Y.9
-
63
-
-
68649104009
-
Mitochondrial calcium function and dysfunction in the central nervous system
-
CrossRef Medline
-
Nicholls DG (2009) Mitochondrial calcium function and dysfunction in the central nervous system. Biochim Biophys Acta 1787:1416-1424. CrossRef Medline
-
(2009)
Biochim Biophys Acta
, vol.1787
, pp. 1416-1424
-
-
Nicholls, D.G.1
-
64
-
-
44849124411
-
ALS-linked mutant SOD1 induces ER stress-and ASK1-dependent motor neuron death by targeting Derlin-1
-
CrossRef Medline
-
Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, Noguchi T, Matsuzawa A, Takeda K, Ichijo H (2008) ALS-linked mutant SOD1 induces ER stress-and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev 22:1451-1464. CrossRef Medline
-
(2008)
Genes Dev
, vol.22
, pp. 1451-1464
-
-
Nishitoh, H.1
Kadowaki, H.2
Nagai, A.3
Maruyama, T.4
Yokota, T.5
Fukutomi, H.6
Noguchi, T.7
Matsuzawa, A.8
Takeda, K.9
Ichijo, H.10
-
65
-
-
77955934441
-
ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2
-
CrossRef Medline
-
Pedrini S, Sau D, Guareschi S, Bogush M, Brown RH Jr, Naniche N, Kia A, Trotti D, Pasinelli P (2010) ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2. Hum Mol Genet 19:2974-2986. CrossRef Medline
-
(2010)
Hum Mol Genet
, vol.19
, pp. 2974-2986
-
-
Pedrini, S.1
Sau, D.2
Guareschi, S.3
Bogush, M.4
Brown Jr., R.H.5
Naniche, N.6
Kia, A.7
Trotti, D.8
Pasinelli, P.9
-
66
-
-
0035853721
-
The mitochondrial permeability transition, release of cytochrome c and cell death. Correlation with the duration of pore openings in situ
-
CrossRef Medline
-
Petronilli V, Penzo D, Scorrano L, Bernardi P, Di Lisa F (2001) The mitochondrial permeability transition, release of cytochrome c and cell death. Correlation with the duration of pore openings in situ. J Biol Chem 276: 12030-12034. CrossRef Medline
-
(2001)
J Biol Chem
, vol.276
, pp. 12030-12034
-
-
Petronilli, V.1
Penzo, D.2
Scorrano, L.3
Bernardi, P.4
Di Lisa, F.5
-
67
-
-
84861596118
-
Misfolded SOD1 and ALS: Zeroing in on mitochondria
-
CrossRef Medline
-
Pickles S, Vande Velde C (2012) Misfolded SOD1 and ALS: zeroing in on mitochondria. Amyotroph Lateral Scler 13:333-340. CrossRef Medline
-
(2012)
Amyotroph Lateral Scler
, vol.13
, pp. 333-340
-
-
Pickles, S.1
Vande Velde, C.2
-
68
-
-
0347155578
-
Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis
-
CrossRef Medline
-
Rakhit R, Cunningham P, Furtos-Matei A, Dahan S, Qi XF, Crow JP, Cashman NR, Kondejewski LH, Chakrabartty A (2002) Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis. J BiolChem277:47551-47556. CrossRef Medline
-
(2002)
J Biol Chem
, vol.277
, pp. 47551-47556
-
-
Rakhit, R.1
Cunningham, P.2
Furtos-Matei, A.3
Dahan, S.4
Qi, X.F.5
Crow, J.P.6
Cashman, N.R.7
Kondejewski, L.H.8
Chakrabartty, A.9
-
69
-
-
2442624720
-
Monomeric Cu, Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis
-
CrossRef Medline
-
Rakhit R, Crow JP, Lepock JR, Kondejewski LH, Cashman NR, Chakrabartty A (2004) Monomeric Cu, Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis. J Biol Chem 279:15499-15504. CrossRef Medline
-
(2004)
J Biol Chem
, vol.279
, pp. 15499-15504
-
-
Rakhit, R.1
Crow, J.P.2
Lepock, J.R.3
Kondejewski, L.H.4
Cashman, N.R.5
Chakrabartty, A.6
-
70
-
-
34249980373
-
An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS
-
CrossRef Medline
-
Rakhit R, Robertson J, Vande Velde C, Horne P, Ruth DM, Griffin J, Cleveland DW, Cashman NR, Chakrabartty A (2007) An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med 13:754-759. CrossRef Medline
-
(2007)
Nat Med
, vol.13
, pp. 754-759
-
-
Rakhit, R.1
Robertson, J.2
Vande Velde, C.3
Horne, P.4
Ruth, D.M.5
Griffin, J.6
Cleveland, D.W.7
Cashman, N.R.8
Chakrabartty, A.9
-
71
-
-
0027401203
-
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
-
CrossRef Medline
-
Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng HX (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362:59-62. CrossRef Medline
-
(1993)
Nature
, vol.362
, pp. 59-62
-
-
Rosen, D.R.1
Siddique, T.2
Patterson, D.3
Figlewicz, D.A.4
Sapp, P.5
Hentati, A.6
Donaldson, D.7
Goto, J.8
O'Regan, J.P.9
Deng, H.X.10
-
72
-
-
0025299819
-
Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis
-
CrossRef Medline
-
Rothstein JD, Tsai G, Kuncl RW, Clawson L, Cornblath DR, Drachman DB, Pestronk A, Stauch BL, Coyle JT (1990) Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis. Ann Neurol 28:18-25. CrossRef Medline
-
(1990)
Ann Neurol
, vol.28
, pp. 18-25
-
-
Rothstein, J.D.1
Tsai, G.2
Kuncl, R.W.3
Clawson, L.4
Cornblath, D.R.5
Drachman, D.B.6
Pestronk, A.7
Stauch, B.L.8
Coyle, J.T.9
-
73
-
-
0026597010
-
Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis
-
CrossRef Medline
-
Rothstein JD, Martin LJ, Kuncl RW (1992) Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. NEngl J Med 326:1464-1468. CrossRef Medline
-
(1992)
N Engl J Med
, vol.326
, pp. 1464-1468
-
-
Rothstein, J.D.1
Martin, L.J.2
Kuncl, R.W.3
-
74
-
-
34250612194
-
Sodium valproate exerts neuroprotective effects in vivo through CREB-binding proteindependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model
-
CrossRef Medline
-
Rouaux C, Panteleeva I, Ren é F, Gonzalez de Aguilar JL, Echaniz-Laguna A, Dupuis L, Menger Y, Boutillier AL, Loeffler JP (2007) Sodium valproate exerts neuroprotective effects in vivo through CREB-binding proteindependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model. J Neurosci 27:5535-5545. CrossRef Medline
-
(2007)
J Neurosci
, vol.27
, pp. 5535-5545
-
-
Rouaux, C.1
Panteleeva, I.2
Ren é, F.3
Gonzalez de Aguilar, J.L.4
Echaniz-Laguna, A.5
Dupuis, L.6
Menger, Y.7
Boutillier, A.L.8
Loeffler, J.P.9
-
75
-
-
0032402093
-
Glutamate potentiates the toxicity of mutant Cu/Zn-superoxide dismutase in motor neurons by postsynaptic calcium-dependent mechanisms
-
Medline
-
Roy J, Minotti S, Dong L, Figlewicz DA, Durham HD (1998) Glutamate potentiates the toxicity of mutant Cu/Zn-superoxide dismutase in motor neurons by postsynaptic calcium-dependent mechanisms. J Neurosci 18: 9673-9684. Medline
-
(1998)
J Neurosci
, vol.18
, pp. 9673-9684
-
-
Roy, J.1
Minotti, S.2
Dong, L.3
Figlewicz, D.A.4
Durham, H.D.5
-
76
-
-
67349164383
-
A role for motoneuron subtypeselective ER stress in disease manifestations of FALS mice
-
CrossRef Medline
-
Saxena S, Cabuy E, Caroni P (2009) A role for motoneuron subtypeselective ER stress in disease manifestations of FALS mice. Nat Neurosci 12:627-636. CrossRef Medline
-
(2009)
Nat Neurosci
, vol.12
, pp. 627-636
-
-
Saxena, S.1
Cabuy, E.2
Caroni, P.3
-
77
-
-
24744460273
-
Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia
-
CrossRef Medline
-
Schinzel AC, Takeuchi O, Huang Z, Fisher JK, Zhou Z, Rubens J, Hetz C, Danial NN, Moskowitz MA, Korsmeyer SJ (2005) Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia. Proc Natl Acad Sci U S A 102:12005-12010. CrossRef Medline
-
(2005)
Proc Natl Acad Sci U S A
, vol.102
, pp. 12005-12010
-
-
Schinzel, A.C.1
Takeuchi, O.2
Huang, Z.3
Fisher, J.K.4
Zhou, Z.5
Rubens, J.6
Hetz, C.7
Danial, N.N.8
Moskowitz, M.A.9
Korsmeyer, S.J.10
-
78
-
-
23944490153
-
The oral antidiabetic pioglitazone protects from neurodegeneration and amyotrophic lateral sclerosis-like symptoms in superoxide dismutase-G93A transgenic mice
-
CrossRef Medline
-
Schütz B, Reimann J, Dumitrescu-Ozimek L, Kappes-Horn K, Landreth GE, Schürmann B, Zimmer A, Heneka MT (2005) The oral antidiabetic pioglitazone protects from neurodegeneration and amyotrophic lateral sclerosis-like symptoms in superoxide dismutase-G93A transgenic mice. J Neurosci 25:7805-7812. CrossRef Medline
-
(2005)
J Neurosci
, vol.25
, pp. 7805-7812
-
-
Schütz, B.1
Reimann, J.2
Dumitrescu-Ozimek, L.3
Kappes-Horn, K.4
Landreth, G.E.5
Schürmann, B.6
Zimmer, A.7
Heneka, M.T.8
-
79
-
-
0035918258
-
Mutant Cu/Znsuperoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis
-
CrossRef Medline
-
Shinder GA, Lacourse MC, Minotti S, Durham HD (2001) Mutant Cu/Znsuperoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis. J Biol Chem 276:12791-12796. CrossRef Medline
-
(2001)
J Biol Chem
, vol.276
, pp. 12791-12796
-
-
Shinder, G.A.1
Lacourse, M.C.2
Minotti, S.3
Durham, H.D.4
-
80
-
-
0030026832
-
Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis
-
CrossRef Medline
-
Siklós L, Engelhardt J, Harati Y, Smith RG, Joó F, Appel SH (1996) Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis. Ann Neurol 39:203-216. CrossRef Medline
-
(1996)
Ann Neurol
, vol.39
, pp. 203-216
-
-
Siklós, L.1
Engelhardt, J.2
Harati, Y.3
Smith, R.G.4
Joó, F.5
Appel, S.H.6
-
81
-
-
0031801922
-
Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice
-
CrossRef Medline
-
Siklós L, Engelhardt JI, Alexianu ME, Gurney ME, Siddique T, Appel SH (1998) Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice. J Neuropathol Exp Neurol 57:571-587. CrossRef Medline
-
(1998)
J Neuropathol Exp Neurol
, vol.57
, pp. 571-587
-
-
Siklós, L.1
Engelhardt, J.I.2
Alexianu, M.E.3
Gurney, M.E.4
Siddique, T.5
Appel, S.H.6
-
82
-
-
5444222849
-
CalciumpermeableAMPAreceptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model
-
CrossRef Medline
-
Tateno M, Sadakata H, Tanaka M, Itohara S, Shin RM, Miura M, Masuda M, Aosaki T, Urushitani M, Misawa H, Takahashi R (2004) CalciumpermeableAMPAreceptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model. Hum Mol Genet 13:2183-2196. CrossRef Medline
-
(2004)
Hum Mol Genet
, vol.13
, pp. 2183-2196
-
-
Tateno, M.1
Sadakata, H.2
Tanaka, M.3
Itohara, S.4
Shin, R.M.5
Miura, M.6
Masuda, M.7
Aosaki, T.8
Urushitani, M.9
Misawa, H.10
Takahashi, R.11
-
83
-
-
66749115386
-
Amyloid deposits: Protection against toxic protein species?
-
CrossRef Medline
-
Treusch S, Cyr DM, Lindquist S (2009) Amyloid deposits: protection against toxic protein species? Cell Cycle 8:1668-1674. CrossRef Medline
-
(2009)
Cell Cycle
, vol.8
, pp. 1668-1674
-
-
Treusch, S.1
Cyr, D.M.2
Lindquist, S.3
-
84
-
-
29444443348
-
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
-
CrossRef Medline
-
Urushitani M, Sik A, Sakurai T, Nukina N, Takahashi R, Julien JP (2006) Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci 9:108-118. CrossRef Medline
-
(2006)
Nat Neurosci
, vol.9
, pp. 108-118
-
-
Urushitani, M.1
Sik, A.2
Sakurai, T.3
Nukina, N.4
Takahashi, R.5
Julien, J.P.6
-
85
-
-
0037986558
-
The AMPA receptor antagonist NBQX prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis
-
CrossRef Medline
-
Van Damme P, Leyssen M, Callewaert G, Robberecht W, Van Den Bosch L (2003) The AMPA receptor antagonist NBQX prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis. Neurosci Lett 343:81-84. CrossRef Medline
-
(2003)
Neurosci Lett
, vol.343
, pp. 81-84
-
-
Van Damme, P.1
Leyssen, M.2
Callewaert, G.3
Robberecht, W.4
Van Den Bosch, L.5
-
86
-
-
33751229311
-
The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis
-
CrossRef Medline
-
Van Den Bosch L, Van Damme P, Bogaert E, Robberecht W (2006) The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis. Biochim Biophys Acta 1762:1068-1082. CrossRef Medline
-
(2006)
Biochim Biophys Acta
, vol.1762
, pp. 1068-1082
-
-
Van Den Bosch, L.1
Van Damme, P.2
Bogaert, E.3
Robberecht, W.4
-
87
-
-
29644433445
-
Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease
-
CrossRef Medline
-
Van Raamsdonk JM, Murphy Z, Slow EJ, Leavitt BR, Hayden MR (2005) Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum Mol Genet 14: 3823-3835. CrossRef Medline
-
(2005)
Hum Mol Genet
, vol.14
, pp. 3823-3835
-
-
van Raamsdonk, J.M.1
Murphy, Z.2
Slow, E.J.3
Leavitt, B.R.4
Hayden, M.R.5
-
88
-
-
21644453699
-
The neuroprotective factor Wlds does not attenuate mutant SOD1-mediated motor neuron disease
-
CrossRef Medline
-
Vande Velde C, Garcia ML, Yin X, Trapp BD, Cleveland DW (2004) The neuroprotective factor Wlds does not attenuate mutant SOD1-mediated motor neuron disease. Neuromolecular Med 5:193-203. CrossRef Medline
-
(2004)
Neuromolecular Med
, vol.5
, pp. 193-203
-
-
Vande Velde, C.1
Garcia, M.L.2
Yin, X.3
Trapp, B.D.4
Cleveland, D.W.5
-
89
-
-
41649086378
-
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria
-
CrossRef Medline
-
Vande Velde C, Miller TM, Cashman NR, Cleveland DW (2008) Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A 105:4022-4027. CrossRef Medline
-
(2008)
Proc Natl Acad Sci U S A
, vol.105
, pp. 4022-4027
-
-
Vande Velde, C.1
Miller, T.M.2
Cashman, N.R.3
Cleveland, D.W.4
-
90
-
-
79960112809
-
Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset
-
CrossRef Medline
-
Vande Velde C, McDonald KK, Boukhedimi Y, McAlonis-Downes M, Lobsiger CS, Bel Hadj S, Zandona A, Julien JP, Shah SB, Cleveland DW (2011) Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. Plo S One 6:e22031. CrossRef Medline
-
(2011)
Plo S One
, vol.6
-
-
Vande Velde, C.1
McDonald, K.K.2
Boukhedimi, Y.3
McAlonis-Downes, M.4
Lobsiger, C.S.5
Bel Hadj, S.6
Zandona, A.7
Julien, J.P.8
Shah, S.B.9
Cleveland, D.W.10
-
91
-
-
14944385595
-
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice
-
CrossRef Medline
-
Vijayvergiya C, Beal MF, Buck J, Manfredi G (2005) Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci 25:2463-2470. CrossRef Medline
-
(2005)
J Neurosci
, vol.25
, pp. 2463-2470
-
-
Vijayvergiya, C.1
Beal, M.F.2
Buck, J.3
Manfredi, G.4
-
92
-
-
53249134366
-
Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice
-
CrossRef Medline
-
von Lewinski F, Fuchs J, Vanselow BK, Keller BU (2008) Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice. Neurosci Lett 445:224-228. CrossRef Medline
-
(2008)
Neurosci Lett
, vol.445
, pp. 224-228
-
-
von Lewinski, F.1
Fuchs, J.2
Vanselow, B.K.3
Keller, B.U.4
-
93
-
-
0036076642
-
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site
-
CrossRef Medline
-
Wang J, Xu G, Gonzales V, Coonfield M, Fromholt D, Copeland NG, Jenkins NA, Borchelt DR (2002) Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol Dis 10:128-138. CrossRef Medline
-
(2002)
Neurobiol Dis
, vol.10
, pp. 128-138
-
-
Wang, J.1
Xu, G.2
Gonzales, V.3
Coonfield, M.4
Fromholt, D.5
Copeland, N.G.6
Jenkins, N.A.7
Borchelt, D.R.8
-
94
-
-
0029053881
-
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
-
CrossRef Medline
-
Wong PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG, Jenkins NA, Sisodia SS, Cleveland DW, Price DL (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14: 1105-1116. CrossRef Medline
-
(1995)
Neuron
, vol.14
, pp. 1105-1116
-
-
Wong, P.C.1
Pardo, C.A.2
Borchelt, D.R.3
Lee, M.K.4
Copeland, N.G.5
Jenkins, N.A.6
Sisodia, S.S.7
Cleveland, D.W.8
Price, D.L.9
-
95
-
-
39749188753
-
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
-
CrossRef Medline
-
Yamanaka K, Chun SJ, Boillee S, Fujimori-Tonou N, Yamashita H, Gutmann DH, Takahashi R, Misawa H, Cleveland DW (2008) Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci 11:251-253. CrossRef Medline
-
(2008)
Nat Neurosci
, vol.11
, pp. 251-253
-
-
Yamanaka, K.1
Chun, S.J.2
Boillee, S.3
Fujimori-Tonou, N.4
Yamashita, H.5
Gutmann, D.H.6
Takahashi, R.7
Misawa, H.8
Cleveland, D.W.9
|