-
1
-
-
0029010496
-
Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase
-
Andersen P. M., Nilsson P., Ala-Hurula V., Keranen M. L., Tarvainen I., Haltia T., Nilsson L., Binzer M., Forsgren L. Marklund S. L. (1995) Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase. Nat. Genet. 10, 61 66.
-
(1995)
Nat. Genet.
, vol.10
, pp. 61-66
-
-
Andersen, P.M.1
Nilsson, P.2
Ala-Hurula, V.3
Keranen, M.L.4
Tarvainen, I.5
Haltia, T.6
Nilsson, L.7
Binzer, M.8
Forsgren, L.9
Marklund, S.L.10
-
2
-
-
0038446777
-
Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: A decade of discoveries, defects and disputes
-
Andersen P. M., Sims K. B., Xin W. W. et al. (2003) Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 4, 1 12.
-
(2003)
Amyotroph. Lateral Scler. Other Motor Neuron Disord.
, vol.4
, pp. 1-12
-
-
Andersen, P.M.1
Sims, K.B.2
Xin, W.W.3
-
3
-
-
33749563294
-
Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1
-
Atkin J. D., Farg M. A., Turner B. J. et al. (2006) Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1. J. Biol. Chem. 281, 30152 30165.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 30152-30165
-
-
Atkin, J.D.1
Farg, M.A.2
Turner, B.J.3
-
4
-
-
43649100018
-
Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
-
Atkin J. D., Farg M. A., Walker A. K., McLean C., Tomas D. Horne M. K. (2008) Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol. Dis. 30, 400 407.
-
(2008)
Neurobiol. Dis.
, vol.30
, pp. 400-407
-
-
Atkin, J.D.1
Farg, M.A.2
Walker, A.K.3
McLean, C.4
Tomas, D.5
Horne, M.K.6
-
5
-
-
34547415429
-
Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: A possible general mechanism for familial ALS
-
Banci L., Bertini I., Durazo A., Girotto S., Gralla E. B., Martinelli M., Valentine J. S., Vieru M. Whitelegge J. P. (2007) Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: a possible general mechanism for familial ALS. Proc. Natl. Acad. Sci. USA 104, 11263 11267.
-
(2007)
Proc. Natl. Acad. Sci. USA
, vol.104
, pp. 11263-11267
-
-
Banci, L.1
Bertini, I.2
Durazo, A.3
Girotto, S.4
Gralla, E.B.5
Martinelli, M.6
Valentine, J.S.7
Vieru, M.8
Whitelegge, J.P.9
-
6
-
-
33749070043
-
Insoluble mutant SOD1 is partly oligoubiquitinated in amyotrophic lateral sclerosis mice
-
Basso M., Massignan T., Samengo G., Cheroni C., De B. S., Salmona M., Bendotti C. Bonetto V. (2006) Insoluble mutant SOD1 is partly oligoubiquitinated in amyotrophic lateral sclerosis mice. J. Biol. Chem. 281, 33325 33335.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 33325-33335
-
-
Basso, M.1
Massignan, T.2
Samengo, G.3
Cheroni, C.4
De, B.S.5
Salmona, M.6
Bendotti, C.7
Bonetto, V.8
-
7
-
-
77949505299
-
Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis
-
Basso M., Samengo G., Nardo G. et al. (2009) Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis. PLoS ONE 4, e8130.
-
(2009)
PLoS ONE
, vol.4
, pp. 8130
-
-
Basso, M.1
Samengo, G.2
Nardo, G.3
-
8
-
-
33646452761
-
Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models
-
Bergemalm D., Jonsson P. A., Graffmo K. S., Andersen P. M., Brannstrom T., Rehnmark A. Marklund S. L. (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.
-
(2006)
J. Neurosci.
, vol.26
, pp. 4147-4154
-
-
Bergemalm, D.1
Jonsson, P.A.2
Graffmo, K.S.3
Andersen, P.M.4
Brannstrom, T.5
Rehnmark, A.6
Marklund, S.L.7
-
9
-
-
67650526816
-
Changes in the spinal cord proteome of an amyotrophic lateral sclerosis murine model determined by differential in-gel electrophoresis
-
Bergemalm D., Forsberg K., Jonsson P. A., Graffmo K. S., Brannstrom T., Andersen P. M., Antti H. Marklund S. L. (2009) Changes in the spinal cord proteome of an amyotrophic lateral sclerosis murine model determined by differential in-gel electrophoresis. Mol. Cell Proteomics 8, 1306 1317.
-
(2009)
Mol. Cell Proteomics
, vol.8
, pp. 1306-1317
-
-
Bergemalm, D.1
Forsberg, K.2
Jonsson, P.A.3
Graffmo, K.S.4
Brannstrom, T.5
Andersen, P.M.6
Antti, H.7
Marklund, S.L.8
-
10
-
-
33744798774
-
Onset and progression in inherited ALS determined by motor neurons and microglia
-
Boillee S., Yamanaka K., Lobsiger C. S., Copeland N. G., Jenkins N. A., Kassiotis G., Kollias G. Cleveland D. W. (2006) Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312, 1389 1392.
-
(2006)
Science
, vol.312
, pp. 1389-1392
-
-
Boillee, 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
-
11
-
-
0031051485
-
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
-
Bruijn L. I., Becher M. W., Lee M. K. et al. (1997) ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18, 327 338.
-
(1997)
Neuron
, vol.18
, pp. 327-338
-
-
Bruijn, L.I.1
Becher, M.W.2
Lee, M.K.3
-
12
-
-
57749100302
-
Initiation and elongation in fibrillation of ALS-linked superoxide dismutase
-
Chattopadhyay M., Durazo A., Sohn S. H., Strong C. D., Gralla E. B., Whitelegge J. P. Valentine J. S. (2008) Initiation and elongation in fibrillation of ALS-linked superoxide dismutase. Proc. Natl Acad. Sci. USA 105, 18663 18668.
-
(2008)
Proc. Natl Acad. Sci. USA
, vol.105
, pp. 18663-18668
-
-
Chattopadhyay, M.1
Durazo, A.2
Sohn, S.H.3
Strong, C.D.4
Gralla, E.B.5
Whitelegge, J.P.6
Valentine, J.S.7
-
13
-
-
4344703472
-
Co-chaperone CHIP associates with mutant Cu/Zn-superoxide dismutase proteins linked to familial amyotrophic lateral sclerosis and promotes their degradation by proteasomes
-
Choi J. S., Cho S., Park S. G., Park B. C. Lee D. H. (2004) Co-chaperone CHIP associates with mutant Cu/Zn-superoxide dismutase proteins linked to familial amyotrophic lateral sclerosis and promotes their degradation by proteasomes. Biochem. Biophys. Res. Commun. 321, 574 583.
-
(2004)
Biochem. Biophys. Res. Commun.
, vol.321
, pp. 574-583
-
-
Choi, J.S.1
Cho, S.2
Park, S.G.3
Park, B.C.4
Lee, D.H.5
-
14
-
-
53049109088
-
Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in familial form of ALS
-
Furukawa Y., Kaneko K., Yamanaka K., O'Halloran T. V. Nukina N. (2008) Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in familial form of ALS. J. Biol. Chem. 283, 24167 24176.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 24167-24176
-
-
Furukawa, Y.1
Kaneko, K.2
Yamanaka, K.3
O'Halloran, T.V.4
Nukina, N.5
-
15
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
-
Gurney M. E., Pu H., Chiu A. Y. et al. (1994) Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264, 1772 1775. (Pubitemid 24227760)
-
(1994)
Science
, vol.264
, Issue.5166
, 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
Chen, W.11
Zhai, P.12
Sufit, R.L.13
Siddique, T.14
-
16
-
-
0029037348
-
Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction
-
Haverkamp L. J., Appel V. Appel S. H. (1995) Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 118, 707 719.
-
(1995)
Brain
, vol.118
, pp. 707-719
-
-
Haverkamp, L.J.1
Appel, V.2
Appel, S.H.3
-
17
-
-
70449525135
-
Microglia in ALS: The good, the bad, and the resting
-
Henkel J. S., Beers D. R., Zhao W. Appel S. H. (2009) Microglia in ALS: the good, the bad, and the resting. J. Neuroimmune Pharmacol. 4, 389 398.
-
(2009)
J. Neuroimmune Pharmacol.
, vol.4
, pp. 389-398
-
-
Henkel, J.S.1
Beers, D.R.2
Zhao, W.3
Appel, S.H.4
-
18
-
-
0033749379
-
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
-
Johnston J. A., Dalton M. J., Gurney M. E. Kopito R. R. (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. USA 97, 12571 12576.
-
(2000)
Proc. Natl Acad. Sci. USA
, vol.97
, pp. 12571-12576
-
-
Johnston, J.A.1
Dalton, M.J.2
Gurney, M.E.3
Kopito, R.R.4
-
19
-
-
0347358915
-
Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis
-
Jonsson P. A., Ernhill K., Andersen P. M., Bergemalm D., Brannstrom T., Gredal O., Nilsson P. Marklund S. L. (2004) Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis. Brain 127, 73 88.
-
(2004)
Brain
, vol.127
, pp. 73-88
-
-
Jonsson, P.A.1
Ernhill, K.2
Andersen, P.M.3
Bergemalm, D.4
Brannstrom, T.5
Gredal, O.6
Nilsson, P.7
Marklund, S.L.8
-
20
-
-
31544467869
-
Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models
-
Jonsson P. A., Graffmo K. S., Andersen P. M., Brannstrom T., Lindberg M., Oliveberg M. Marklund S. L. (2006a) Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models. Brain 129, 451 464.
-
(2006)
Brain
, vol.129
, pp. 451-464
-
-
Jonsson, P.A.1
Graffmo, K.S.2
Andersen, P.M.3
Brannstrom, T.4
Lindberg, M.5
Oliveberg, M.6
Marklund, S.L.7
-
21
-
-
33845361242
-
Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1
-
Jonsson P. A., Graffmo K. S., Brannstrom T., Nilsson P., Andersen P. M. Marklund S. L. (2006b) Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1. J. Neuropathol. Exp. Neurol. 65, 1126 1136.
-
(2006)
J. Neuropathol. Exp. Neurol.
, vol.65
, pp. 1126-1136
-
-
Jonsson, P.A.1
Graffmo, K.S.2
Brannstrom, T.3
Nilsson, P.4
Andersen, P.M.5
Marklund, S.L.6
-
22
-
-
44849090909
-
Inclusions of amyotrophic lateral sclerosis-linked superoxide dismutase in ventral horns, liver, and kidney
-
Jonsson P. A., Bergemalm D., Andersen P. M., Gredal O., Brannstrom T. Marklund S. L. (2008) Inclusions of amyotrophic lateral sclerosis-linked superoxide dismutase in ventral horns, liver, and kidney. Ann. Neurol. 63, 671 675.
-
(2008)
Ann. Neurol.
, vol.63
, pp. 671-675
-
-
Jonsson, P.A.1
Bergemalm, D.2
Andersen, P.M.3
Gredal, O.4
Brannstrom, T.5
Marklund, S.L.6
-
23
-
-
46649096661
-
A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis
-
Karch C. M. Borchelt D. R. (2008) A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis. J. Biol. Chem. 283, 13528 13537.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 13528-13537
-
-
Karch, C.M.1
Borchelt, D.R.2
-
24
-
-
66049156169
-
Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS
-
Karch C. M., Prudencio M., Winkler D. D., Hart P. J. Borchelt D. R. (2009) Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS. Proc. Natl Acad. Sci. USA 106, 7774 7779.
-
(2009)
Proc. Natl Acad. Sci. USA
, vol.106
, pp. 7774-7779
-
-
Karch, C.M.1
Prudencio, M.2
Winkler, D.D.3
Hart, P.J.4
Borchelt, D.R.5
-
25
-
-
1942486792
-
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice
-
Kieran D., Kalmar B., Dick J. R., Riddoch-Contreras J., Burnstock G. Greensmith L. (2004) Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat. Med. 10, 402 405.
-
(2004)
Nat. Med.
, vol.10
, pp. 402-405
-
-
Kieran, D.1
Kalmar, B.2
Dick, J.R.3
Riddoch-Contreras, J.4
Burnstock, G.5
Greensmith, L.6
-
26
-
-
33645798615
-
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
-
Kikuchi H., Almer G., Yamashita S., Guegan C., Nagai M., Xu Z., Sosunov A. A., McKhann G. M. 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. USA 103, 6025 6030.
-
(2006)
Proc. Natl Acad. Sci. USA
, vol.103
, pp. 6025-6030
-
-
Kikuchi, H.1
Almer, G.2
Yamashita, S.3
Guegan, C.4
Nagai, M.5
Xu, Z.6
Sosunov, A.A.7
McKhann, G.M.8
Przedborski, S.9
-
27
-
-
3242701496
-
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
-
Liu J., Lillo C., Jonsson P. A. et al. (2004) Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 43, 5 17.
-
(2004)
Neuron
, vol.43
, pp. 5-17
-
-
Liu, J.1
Lillo, C.2
Jonsson, P.A.3
-
28
-
-
34247475338
-
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
-
Nagai M., Re D. B., Nagata T., Chalazonitis A., Jessell T. M., Wichterle H. Przedborski S. (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat. Neurosci. 10, 615 622.
-
(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
-
29
-
-
34948850962
-
Disulfide bond mediates aggregation, toxicity and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1
-
Niwa J. I., Yamada S. I., Ishigaki S., Sone J., Takahashi M., Katsuno M., Tanaka F., Doyu M. Sobue G. (2007) Disulfide bond mediates aggregation, toxicity and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1. J. Biol. Chem. 282, 28087 28095.
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 28087-28095
-
-
Niwa, J.I.1
Yamada, S.I.2
Ishigaki, S.3
Sone, J.4
Takahashi, M.5
Katsuno, M.6
Tanaka, F.7
Doyu, M.8
Sobue, G.9
-
30
-
-
63449092530
-
Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase
-
Oztug Durer Z. A., Cohlberg J. A., Dinh P. et al. (2009) Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase. PLoS ONE 4, e5004.
-
(2009)
PLoS ONE
, vol.4
, pp. 5004
-
-
Oztug Durer, Z.A.1
Cohlberg, J.A.2
Dinh, P.3
-
31
-
-
27744542191
-
No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model
-
Perrin F. E., Boisset G., Docquier M., Schaad O., Descombes P. Kato A. C. (2005) No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model. Hum. Mol. Genet. 14, 3309 3320.
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 3309-3320
-
-
Perrin, F.E.1
Boisset, G.2
Docquier, M.3
Schaad, O.4
Descombes, P.5
Kato, A.C.6
-
32
-
-
0035818579
-
Specificity in intracellular protein aggregation and inclusion body formation
-
Rajan R. S., Illing M. E., Bence N. F. Kopito R. R. (2001) Specificity in intracellular protein aggregation and inclusion body formation. Proc. Natl Acad. Sci. USA 98, 13060 13065.
-
(2001)
Proc. Natl Acad. Sci. USA
, vol.98
, pp. 13060-13065
-
-
Rajan, R.S.1
Illing, M.E.2
Bence, N.F.3
Kopito, R.R.4
-
33
-
-
58149102066
-
The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1
-
Reddehase S., Grumbt B., Neupert W. Hell K. (2009) The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1. J. Mol. Biol. 385, 331 338.
-
(2009)
J. Mol. Biol.
, vol.385
, pp. 331-338
-
-
Reddehase, S.1
Grumbt, B.2
Neupert, W.3
Hell, K.4
-
34
-
-
0027401203
-
Mutations in Cu/Zn superoxide-dismutase gene are associated with familial amyotrophic-lateral-sclerosis
-
Rosen D. R., Siddique T., Patterson D. et al. (1993) Mutations in Cu/Zn superoxide-dismutase gene are associated with familial amyotrophic-lateral- sclerosis. Nature 362, 59 62.
-
(1993)
Nature
, vol.362
, pp. 59-62
-
-
Rosen, D.R.1
Siddique, T.2
Patterson, D.3
-
35
-
-
67349164383
-
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
-
Saxena S., Cabuy E. Caroni P. (2009) A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat. Neurosci. 12, 627 636.
-
(2009)
Nat. Neurosci.
, vol.12
, pp. 627-636
-
-
Saxena, S.1
Cabuy, E.2
Caroni, P.3
-
37
-
-
43749109844
-
Detergent insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1
-
Shaw B. F., Lelie H. L., Durazo A. et al. (2008) Detergent insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1. J. Biol. Chem. 283, 8340 8350.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 8340-8350
-
-
Shaw, B.F.1
Lelie, H.L.2
Durazo, A.3
-
38
-
-
0035918258
-
Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis
-
Shinder G. A., Lacourse M. C., Minotti S. Durham H. D. (2001) Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis. J. Biol. Chem. 276, 12791 12796.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 12791-12796
-
-
Shinder, G.A.1
Lacourse, M.C.2
Minotti, S.3
Durham, H.D.4
-
39
-
-
65249112094
-
Alternative splicing studies of the reactive oxygen species gene network in Populus reveal two isoforms of high-isoelectric-point superoxide dismutase
-
Srivastava V., Srivastava M. K., Chibani K., Nilsson R., Rouhier N., Melzer M. Wingsle G. (2009) Alternative splicing studies of the reactive oxygen species gene network in Populus reveal two isoforms of high-isoelectric-point superoxide dismutase. Plant Physiol. 149, 1848 1859.
-
(2009)
Plant Physiol.
, vol.149
, pp. 1848-1859
-
-
Srivastava, V.1
Srivastava, M.K.2
Chibani, K.3
Nilsson, R.4
Rouhier, N.5
Melzer, M.6
Wingsle, G.7
-
40
-
-
0346034856
-
Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice
-
Tobisawa S., Hozumi Y., Arawaka S., Koyama S., Wada M., Nagai M., Aoki M., Itoyama Y., Goto K. Kato T. (2003) Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice. Biochem. Biophys. Res. Commun. 303, 496 503.
-
(2003)
Biochem. Biophys. Res. Commun.
, vol.303
, pp. 496-503
-
-
Tobisawa, S.1
Hozumi, Y.2
Arawaka, S.3
Koyama, S.4
Wada, M.5
Nagai, M.6
Aoki, M.7
Itoyama, Y.8
Goto, K.9
Kato, T.10
-
41
-
-
12144249923
-
Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis
-
Turner B. J., Atkin J. D., Farg M. A., Zang d. W., Rembach A., Lopes E. C., Patch J. D., Hill A. F. Cheema S. S. (2005) Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis. J. Neurosci. 25, 108 117.
-
(2005)
J. Neurosci.
, vol.25
, pp. 108-117
-
-
Turner, B.J.1
Atkin, J.D.2
Farg, M.A.3
Zang, D.W.4
Rembach, A.5
Lopes, E.C.6
Patch, J.D.7
Hill, A.F.8
Cheema, S.S.9
-
42
-
-
29444443348
-
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
-
Urushitani M., Sik A., Sakurai T., Nukina N., Takahashi R. Julien J. P. (2006) Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat. Neurosci. 9, 108 118.
-
(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
-
43
-
-
41649086378
-
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria
-
Vande Velde C., Miller T. M., Cashman N. R. Cleveland D. W. (2008) Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc. Natl Acad. Sci. USA 105, 4022 4027.
-
(2008)
Proc. Natl Acad. Sci. USA
, vol.105
, pp. 4022-4027
-
-
Vande Velde, C.1
Miller, T.M.2
Cashman, N.R.3
Cleveland, D.W.4
-
44
-
-
14944385595
-
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice
-
Vijayvergiya C., Beal M. F., 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.
-
(2005)
J. Neurosci.
, vol.25
, pp. 2463-2470
-
-
Vijayvergiya, C.1
Beal, M.F.2
Buck, J.3
Manfredi, G.4
-
45
-
-
0036199623
-
High molecular weight complexes of mutant superoxide dismutase 1: Age-dependent and tissue-specific accumulation
-
Wang J., Xu G. Borchelt D. R. (2002a) High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation. Neurobiol. Dis. 9, 139 148.
-
(2002)
Neurobiol. Dis.
, vol.9
, pp. 139-148
-
-
Wang, J.1
Xu, G.2
Borchelt, D.R.3
-
46
-
-
0036076642
-
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site
-
Wang J., Xu G., Gonzales V., Coonfield M., Fromholt D., Copeland N. G., Jenkins N. A. Borchelt D. R. (2002b) Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol. Dis. 10, 128 138.
-
(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
-
47
-
-
0242524455
-
Copper-binding-site-null SOD1 causes ALS in transgenic mice: Aggregates of non-native SOD1 delineate a common feature
-
Wang J., Slunt H., Gonzales V., Fromholt D., Coonfield M., Copeland N. G., Jenkins N. A. Borchelt D. R. (2003) Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Hum. Mol. Genet. 12, 2753 2764.
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 2753-2764
-
-
Wang, J.1
Slunt, H.2
Gonzales, V.3
Fromholt, D.4
Coonfield, M.5
Copeland, N.G.6
Jenkins, N.A.7
Borchelt, D.R.8
-
48
-
-
26444542945
-
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: AlphaB-crystallin modulates aggregation
-
Wang J., Xu G., Li H., Gonzales V., Fromholt D., Karch C., Copeland N. G., Jenkins N. A. Borchelt D. R. (2005) Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation. Hum. Mol. Genet. 14, 2335 2347.
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 2335-2347
-
-
Wang, J.1
Xu, G.2
Li, H.3
Gonzales, V.4
Fromholt, D.5
Karch, C.6
Copeland, N.G.7
Jenkins, N.A.8
Borchelt, D.R.9
-
49
-
-
60849126687
-
Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS
-
Wang J., Farr G. W., Zeiss C. J. et al. (2009) Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS. Proc. Natl Acad. Sci. USA 106, 1392 1397.
-
(2009)
Proc. Natl Acad. Sci. USA
, vol.106
, pp. 1392-1397
-
-
Wang, J.1
Farr, G.W.2
Zeiss, C.J.3
-
50
-
-
0035664213
-
Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues
-
Watanabe M., Dykes-Hoberg M., Culotta V. C., Price D. L., Wong P. C. Rothstein J. D. (2001) Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues. Neurobiol. Dis. 8, 933 941.
-
(2001)
Neurobiol. Dis.
, vol.8
, pp. 933-941
-
-
Watanabe, M.1
Dykes-Hoberg, M.2
Culotta, V.C.3
Price, D.L.4
Wong, P.C.5
Rothstein, J.D.6
-
51
-
-
28844507918
-
Expression of an endoplasmic reticulum-resident chaperone, glucose-regulated stress protein 78, in the spinal cord of a mouse model of amyotrophic lateral sclerosis
-
Wate R., Ito H., Zhang J. H., Ohnishi S., Nakano S. Kusaka H. (2005) Expression of an endoplasmic reticulum-resident chaperone, glucose-regulated stress protein 78, in the spinal cord of a mouse model of amyotrophic lateral sclerosis. Acta Neuropathol. 110, 557 562.
-
(2005)
Acta Neuropathol.
, vol.110
, pp. 557-562
-
-
Wate, R.1
Ito, H.2
Zhang, J.H.3
Ohnishi, S.4
Nakano, S.5
Kusaka, H.6
-
52
-
-
35348874857
-
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
-
Zetterstrom P., Stewart H. G., Bergemalm D., Jonsson P. A., Graffmo K. S., Andersen P. M., Brannstrom T., Oliveberg M. Marklund S. L. (2007) Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models. Proc. Natl Acad. Sci. USA 104, 14157 14162.
-
(2007)
Proc. Natl Acad. Sci. USA
, vol.104
, pp. 14157-14162
-
-
Zetterstrom, P.1
Stewart, H.G.2
Bergemalm, D.3
Jonsson, P.A.4
Graffmo, K.S.5
Andersen, P.M.6
Brannstrom, T.7
Oliveberg, M.8
Marklund, S.L.9
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