-
1
-
-
0036828798
-
"True" sporadic ALS associated with a novel SOD-1 mutation
-
DOI 10.1002/ana.10369
-
Alexander M. D., Traynor B. J., Miller N., Corr B., Frost E., McQuaid S., Brett F. M., Green A., and, Hardiman O., (2002) "True" sporadic ALS associated with a novel SOD-1 mutation. Ann. Neurol. 52, 680-683. (Pubitemid 35246878)
-
(2002)
Annals of Neurology
, vol.52
, Issue.5
, pp. 680-683
-
-
Alexander, M.D.1
Traynor, B.J.2
Miller, N.3
Corr, B.4
Frost, E.5
McQuaid, S.6
Brett, F.M.7
Green, A.8
Hardiman, O.9
-
2
-
-
57649210302
-
The disulfide relay of the intermembrane space of mitochondria: An oxygen-sensing system?
-
Bihlmaier K., Mesecke N., Kloeppel C., and, Herrmann J. M., (2008) The disulfide relay of the intermembrane space of mitochondria: an oxygen-sensing system? Ann. N Y Acad. Sci. 1147, 293-302.
-
(2008)
Ann. N y Acad. Sci.
, vol.1147
, pp. 293-302
-
-
Bihlmaier, K.1
Mesecke, N.2
Kloeppel, C.3
Herrmann, J.M.4
-
3
-
-
0030499678
-
A vector for expressing foreign genes in the brains and hearts of transgenic mice
-
DOI 10.1016/S1050-3862(96)00167-2
-
Borchelt D. R., Davis J., Fischer M., et al. (1996) A vector for expressing foreign genes in the brains and hearts of transgenic mice. Genet. Anal. 13, 159-163. (Pubitemid 27063321)
-
(1996)
Genetic Analysis - Biomolecular Engineering
, vol.13
, Issue.6
, pp. 159-163
-
-
Borchelt, D.R.1
Davis, J.2
Fischer, M.3
Lee, M.K.4
Slunt, H.H.5
Ratovitsky, T.6
Regard, J.7
Copeland, N.G.8
Jenkins, N.A.9
Sisodia, S.S.10
Price, D.L.11
-
4
-
-
1942437507
-
Mechanisms for activating Cu- and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone
-
DOI 10.1073/pnas.0308298101
-
Carroll M. C., Girouard J. B., Ulloa J. L., Subramaniam J. R., Wong P. C., Valentine J. S., and, Culotta V. C., (2004) Mechanisms for activating Cu- and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone. Proc. Natl. Acad. Sci. U S A 101, 5964-5969. (Pubitemid 38520511)
-
(2004)
Proceedings of the National Academy of Sciences of the United States of America
, vol.101
, Issue.16
, pp. 5964-5969
-
-
Carroll, M.C.1
Girouard, J.B.2
Ulloa, J.L.3
Subramaniam, J.R.4
Wong, P.C.5
Valentine, J.S.6
Culotta, V.C.7
-
5
-
-
0032508609
-
The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase
-
DOI 10.1074/jbc.273.37.23625
-
Casareno R. L., Waggoner D., and, Gitlin J. D., (1998) The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase. J. Biol. Chem. 273, 23625-23628. (Pubitemid 28435686)
-
(1998)
Journal of Biological Chemistry
, vol.273
, Issue.37
, pp. 23625-23628
-
-
Casareno, R.L.B.1
Waggoner, D.2
Gitlin, J.D.3
-
6
-
-
0030802648
-
The copper chaperone for superoxide dismutase
-
DOI 10.1074/jbc.272.38.23469
-
Culotta V. C., Klomp L. W., Strain J., Casareno R. L., Krems B., and, Gitlin J. D., (1997) The copper chaperone for superoxide dismutase. J. Biol. Chem. 272, 23469-23472. (Pubitemid 27410910)
-
(1997)
Journal of Biological Chemistry
, vol.272
, Issue.38
, pp. 23469-23472
-
-
Culotta, V.C.1
Klomp, L.W.J.2
Strain, J.3
Casareno, R.L.B.4
Krems, B.5
Gitlin, J.D.6
-
7
-
-
0027946294
-
Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis
-
Dal Canto M. C., and, Gurney M. E., (1994) Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis. Am. J. Pathol. 145, 1271-1279. (Pubitemid 24378480)
-
(1994)
American Journal of Pathology
, vol.145
, Issue.6
, pp. 1271-1279
-
-
Dal Canto, M.C.1
Gurney, M.E.2
-
8
-
-
33646466296
-
Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria
-
Deng H. X., Shi Y., Furukawa Y., et al. (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.
-
(2006)
Proc. Natl. Acad. Sci. U S A
, vol.103
, pp. 7142-7147
-
-
Deng, H.X.1
Shi, Y.2
Furukawa, Y.3
-
9
-
-
33748795566
-
Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials
-
DOI 10.1073/pnas.0605814103
-
Ferri A., Cozzolino M., Crosio C., Nencini M., Casciati A., Gralla E. B., Rotilio G., Valentine J. S., and, Carri M. T., (2006) Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials. Proc. Natl. Acad. Sci. U S A 103, 13860-13865. (Pubitemid 44413998)
-
(2006)
Proceedings of the National Academy of Sciences of the United States of America
, vol.103
, Issue.37
, pp. 13860-13865
-
-
Ferri, A.1
Cozzolino, M.2
Crosio, C.3
Nencini, M.4
Casciati, A.5
Gralla, E.B.6
Rotilio, G.7
Valentine, J.S.8
Carri, M.T.9
-
10
-
-
33745821631
-
Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyptrophic lateral sclerosis
-
DOI 10.1089/ars.2006.8.847
-
Furukawa Y., and, O'Halloran T. V., (2006) Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis. Antioxid. Redox Signal. 8, 847-867. (Pubitemid 44036502)
-
(2006)
Antioxidants and Redox Signaling
, vol.8
, Issue.5-6
, pp. 847-867
-
-
Furukawa, Y.1
O'Halloran, T.V.2
-
11
-
-
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
-
12
-
-
41449099521
-
The Erv1-Mia40 disulfide relay system in the intermembrane space of mitochondria
-
Hell K., (2008) The Erv1-Mia40 disulfide relay system in the intermembrane space of mitochondria. Biochim. Biophys. Acta 1783, 601-609.
-
(2008)
Biochim. Biophys. Acta
, vol.1783
, pp. 601-609
-
-
Hell, K.1
-
13
-
-
0037088793
-
Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS
-
Higgins C. M., Jung C., Ding H., and, Xu Z., (2002) Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS. J. Neurosci. 22, RC215.
-
(2002)
J. Neurosci.
, vol.22
-
-
Higgins, C.M.1
Jung, C.2
Ding, H.3
Xu, Z.4
-
14
-
-
0343537868
-
Three novel mutations and two variants in the gene for Cu/Zn superoxide dismutase in familial amyotrophic lateral sclerosis
-
DOI 10.1016/0960-8966(96)00353-7, PII S0960896696003537
-
Hosler B. A., Nicholson G. A., Sapp P. C., et al. (1996) Three novel mutations and two variants in the gene for Cu/Zn superoxide dismutase in familial amyotrophic lateral sclerosis. Neuromusc. Disord. 6, 361-366. (Pubitemid 26388003)
-
(1996)
Neuromuscular Disorders
, vol.6
, Issue.5
, pp. 361-366
-
-
Hosler, B.A.1
Nicholson, G.A.2
Sapp, P.C.3
Chin, W.4
Orrell, R.W.5
De Belleroche, J.S.6
Esteban, J.7
Hayward, L.J.8
McKenna-Yasek, D.9
Yeung, L.10
Cherryson, A.K.11
Dench, J.E.12
Wilton, S.D.13
Laing, N.G.14
Horvitz, H.R.15
Brown Jr., R.H.16
-
15
-
-
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., and, 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. U S A 97, 12571-12576.
-
(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
-
16
-
-
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., and, Borchelt D. R., (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.
-
(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
-
17
-
-
54449092952
-
Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria
-
Kawamata H., and, Manfredi G., (2008) Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria. Hum. Mol. Genet. 17, 3303-3317.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 3303-3317
-
-
Kawamata, H.1
Manfredi, G.2
-
18
-
-
33745956238
-
Novel mutations that enhance or repress the aggregation potential of SOD1
-
DOI 10.1007/s11010-005-9112-4
-
Krishnan U., Son M., Rajendran B., and, Elliott J. L., (2006) Novel mutations that enhance or repress the aggregation potential of SOD1. Mol. Cell. Biochem. 287, 201-211. (Pubitemid 44057408)
-
(2006)
Molecular and Cellular Biochemistry
, vol.287
, Issue.1-2
, pp. 201-211
-
-
Krishnan, U.1
Son, M.2
Rajendran, B.3
Elliott, J.L.4
-
19
-
-
3242701496
-
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
-
DOI 10.1016/j.neuron.2004.06.016, PII S0896627304003630
-
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. (Pubitemid 38952815)
-
(2004)
Neuron
, vol.43
, Issue.1
, pp. 5-17
-
-
Liu, J.1
Lillo, C.2
Jonsson, P.A.3
Velde, C.V.4
Ward, C.M.5
Miller, T.M.6
Subramaniam, J.R.7
Rothstein, J.D.8
Marklund, S.9
Andersen, P.M.10
Brannstrom, T.11
Gredal, O.12
Wong, P.C.13
Williams, D.S.14
Cleveland, D.W.15
-
20
-
-
21244445718
-
A disulfide relay system in the intermembrane space of mitochondria that mediates protein import
-
DOI 10.1016/j.cell.2005.04.011, PII S0092867405003570
-
Mesecke N., Terziyska N., Kozany C., Baumann F., Neupert W., Hell K., and, Herrmann J. M., (2005) A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell 121, 1059-1069. (Pubitemid 40884396)
-
(2005)
Cell
, vol.121
, Issue.7
, pp. 1059-1069
-
-
Mesecke, N.1
Terziyska, N.2
Kozany, C.3
Baumann, F.4
Neupert, W.5
Hell, K.6
Herrmann, J.M.7
-
21
-
-
77954757268
-
Exposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants
-
Munch C., and, Bertolotti A., (2010) Exposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants. J. Mol. Biol. 399, 512-525.
-
(2010)
J. Mol. Biol.
, vol.399
, pp. 512-525
-
-
Munch, C.1
Bertolotti, A.2
-
22
-
-
70349104733
-
The ALS-associated mutation G93A in human copper-zinc superoxide dismutase selectively destabilizes the remote metal binding region
-
Museth A. K., Brorsson A. C., Lundqvist M., Tibell L. A., and, Jonsson B. H., (2009) The ALS-associated mutation G93A in human copper-zinc superoxide dismutase selectively destabilizes the remote metal binding region. Biochemistry 48, 8817-8829.
-
(2009)
Biochemistry
, vol.48
, pp. 8817-8829
-
-
Museth, A.K.1
Brorsson, A.C.2
Lundqvist, M.3
Tibell, L.A.4
Jonsson, B.H.5
-
23
-
-
44849139852
-
Biological effects of CCS in the absence of SOD1 enzyme activation: Implications for disease in a mouse model for ALS
-
DOI 10.1093/hmg/ddn063
-
Proescher J. B., Son M., Elliott J. L., and, Culotta V. C., (2008) Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. Hum. Mol. Genet. 17, 1728-1737. (Pubitemid 351791499)
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.12
, pp. 1728-1737
-
-
Proescher, J.B.1
Son, M.2
Elliott, J.L.3
Culotta, V.C.4
-
24
-
-
27744601821
-
Non-neuronal induction of immunoproteasome subunits in an ALS model: Possible mediation by cytokines
-
DOI 10.1016/j.expneurol.2005.08.027, PII S0014488605003195
-
Puttaparthi K., and, Elliott J. L., (2005) Non-neuronal induction of immunoproteasome subunits in an ALS model: possible mediation by cytokines. Exp. Neurol. 196, 441-451. (Pubitemid 41607833)
-
(2005)
Experimental Neurology
, vol.196
, Issue.2
, pp. 441-451
-
-
Puttaparthi, K.1
Elliott, J.L.2
-
25
-
-
0037109771
-
Disease progression in a transgenic model of familial amyotrophic lateral sclerosis is dependent on both neuronal and non-neuronal zinc binding proteins
-
Puttaparthi K., Gitomer W. L., Krishnan U., Son M., Rajendran B., and, Elliott J. L., (2002) Disease progression in a transgenic model of familial amyotrophic lateral sclerosis is dependent on both neuronal and non-neuronal zinc binding proteins. J. Neurosci. 22, 8790-8796. (Pubitemid 35379048)
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.20
, pp. 8790-8796
-
-
Puttaparthi, K.1
Gitomer, W.L.2
Krishnan, U.3
Son, M.4
Rajendran, B.5
Elliott, J.L.6
-
26
-
-
0344393089
-
Aggregate formation in the spinal cord of mutant SOD1 transgenic mice is reversible and mediated by proteasomes
-
DOI 10.1046/j.1471-4159.2003.02028.x
-
Puttaparthi K., Wojcik C., Rajendran B., DeMartino G. N., and, Elliott J. L., (2003) Aggregate formation in the spinal cord of mutant SOD1 transgenic mice is reversible and mediated by proteasomes. J. Neurochem. 87, 851-860. (Pubitemid 37444561)
-
(2003)
Journal of Neurochemistry
, vol.87
, Issue.4
, pp. 851-860
-
-
Puttaparthi, K.1
Wojcik, C.2
Rajendran, B.3
DeMartino, G.N.4
Elliott, J.L.5
-
27
-
-
0032815965
-
Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds
-
DOI 10.1093/hmg/8.8.1451
-
Ratovitski T., Corson L. B., Strain J., Wong P., Cleveland D. W., Culotta V. C., and, Borchelt D. R., (1999) Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds. Hum. Mol. Genet. 8, 1451-1460. (Pubitemid 29374078)
-
(1999)
Human Molecular Genetics
, vol.8
, Issue.8
, pp. 1451-1460
-
-
Ratovitski, T.1
Corson, L.B.2
Strain, J.3
Wong, P.4
Cleveland, D.W.5
Culotta, V.C.6
Borchelt, D.R.7
-
28
-
-
58149102066
-
The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1
-
Reddehase S., Grumbt B., Neupert W., and, 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
-
29
-
-
34848900432
-
Structural Characterization of Zinc-deficient Human Superoxide Dismutase and Implications for ALS
-
DOI 10.1016/j.jmb.2007.07.043, PII S0022283607009825
-
Roberts B. R., Tainer J. A., Getzoff E. D., Malencik D. A., Anderson S. R., Bomben V. C., Meyers K. R., Karplus P. A., and, Beckman J. S., (2007) Structural characterization of zinc-deficient human superoxide dismutase and implications for ALS. J. Mol. Biol. 373, 877-890. (Pubitemid 47505245)
-
(2007)
Journal of Molecular Biology
, vol.373
, Issue.4
, pp. 877-890
-
-
Roberts, B.R.1
Tainer, J.A.2
Getzoff, E.D.3
Malencik, D.A.4
Anderson, S.R.5
Bomben, V.C.6
Meyers, K.R.7
Karplus, P.A.8
Beckman, J.S.9
-
30
-
-
77958557012
-
Cu, Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability
-
Sahawneh M. A., Ricart K. C., Roberts B. R., et al. (2010) Cu, Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability. J. Biol. Chem. 285, 33885-33897.
-
(2010)
J. Biol. Chem.
, vol.285
, pp. 33885-33897
-
-
Sahawneh, M.A.1
Ricart, K.C.2
Roberts, B.R.3
-
31
-
-
0034721928
-
Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1
-
Schmidt P. J., Kunst C., and, Culotta V. C., (2000) Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1. J. Biol. Chem. 275, 33771-33776.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 33771-33776
-
-
Schmidt, P.J.1
Kunst, C.2
Culotta, V.C.3
-
32
-
-
70349759885
-
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis
-
Seetharaman S. V., Prudencio M., Karch C., Holloway S. P., Borchelt D. R., and, Hart P. J., (2009) Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis. Exp. Biol. Med. (Maywood) 234, 1140-1154.
-
(2009)
Exp. Biol. Med. (Maywood)
, vol.234
, pp. 1140-1154
-
-
Seetharaman, S.V.1
Prudencio, M.2
Karch, C.3
Holloway, S.P.4
Borchelt, D.R.5
Hart, P.J.6
-
33
-
-
77954753858
-
Disrupted zinc-binding sites in structures of pathogenic SOD1 variants D124V and H80R
-
Seetharaman S. V., Winkler D. D., Taylor A. B., et al. (2010) Disrupted zinc-binding sites in structures of pathogenic SOD1 variants D124V and H80R. Biochemistry 49, 5714-5725.
-
(2010)
Biochemistry
, vol.49
, pp. 5714-5725
-
-
Seetharaman, S.V.1
Winkler, D.D.2
Taylor, A.B.3
-
34
-
-
34347237645
-
Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology
-
DOI 10.1073/pnas.0610923104
-
Son M., Puttaparthi K., Kawamata H., Rajendran B., Boyer P. J., Manfredi G., and, Elliott J. L., (2007) Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proc. Natl. Acad. Sci. U S A 104, 6072-6077. (Pubitemid 47175660)
-
(2007)
Proceedings of the National Academy of Sciences of the United States of America
, vol.104
, Issue.14
, pp. 6072-6077
-
-
Son, M.1
Puttaparthi, K.2
Kawamata, H.3
Rajendran, B.4
Boyer, P.J.5
Manfredi, G.6
Elliott, J.L.7
-
35
-
-
45549093247
-
Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein
-
Son M., Leary S. C., Romain N., Pierrel F., Winge D. R., Haller R. G., and, Elliott J. L., (2008) Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein. J. Biol. Chem. 283, 12267-12275.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 12267-12275
-
-
Son, M.1
Leary, S.C.2
Romain, N.3
Pierrel, F.4
Winge, D.R.5
Haller, R.G.6
Elliott, J.L.7
-
36
-
-
62149091069
-
Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo
-
Son M., Fu Q., Puttaparthi K., Matthews C. M., and, Elliott J. L., (2009) Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo. Neurobiol. Dis. 34, 155-162.
-
(2009)
Neurobiol. Dis.
, vol.34
, pp. 155-162
-
-
Son, M.1
Fu, Q.2
Puttaparthi, K.3
Matthews, C.M.4
Elliott, J.L.5
-
37
-
-
23844471242
-
Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis
-
DOI 10.1074/jbc.M504039200
-
Tiwari A., Xu Z., and, Hayward L. J., (2005) Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis. J. Biol. Chem. 280, 29771-29779. (Pubitemid 41177054)
-
(2005)
Journal of Biological Chemistry
, vol.280
, Issue.33
, pp. 29771-29779
-
-
Tiwari, A.1
Xu, Z.2
Hayward, L.J.3
-
38
-
-
70350435485
-
Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis
-
Tiwari A., Liba A., Sohn S. H., Seetharaman S. V., Bilsel O., Matthews C. R., Hart P. J., Valentine J. S., and, Hayward L. J., (2009) Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis. J. Biol. Chem. 284, 27746-27758.
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 27746-27758
-
-
Tiwari, A.1
Liba, A.2
Sohn, S.H.3
Seetharaman, S.V.4
Bilsel, O.5
Matthews, C.R.6
Hart, P.J.7
Valentine, J.S.8
Hayward, L.J.9
-
39
-
-
66749175710
-
A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis
-
Trumbull K. A., and, Beckman J. S., (2009) A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis. Antioxid. Redox Signal. 11, 1627-1639.
-
(2009)
Antioxid. Redox Signal.
, vol.11
, pp. 1627-1639
-
-
Trumbull, K.A.1
Beckman, J.S.2
-
40
-
-
14944385595
-
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice
-
DOI 10.1523/JNEUROSCI.4385-04.2005
-
Vijayvergiya C., Beal M. F., Buck J., and, Manfredi G., (2005) Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J. Neurosci. 25, 2463-2470. (Pubitemid 40365156)
-
(2005)
Journal of Neuroscience
, vol.25
, Issue.10
, pp. 2463-2470
-
-
Vijayvergiya, C.1
Beal, M.F.2
Buck, J.3
Manfredi, G.4
-
41
-
-
0242524455
-
Copper-binding-site-null SOD1 causes ALS in transgenic mice: Aggregates of non-native SOD1 delineate a common feature
-
DOI 10.1093/hmg/ddg312
-
Wang J., Slunt H., Gonzales V., Fromholt D., Coonfield M., Copeland N. G., Jenkins N. A., and, 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. (Pubitemid 37407112)
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.21
, 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
-
42
-
-
26244451092
-
Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA
-
DOI 10.1016/j.nbd.2005.06.005, PII S0969996105001646
-
Wang J., Xu G., Slunt H. H., Gonzales V., Coonfield M., Fromholt D., Copeland N. G., Jenkins N. A., and, Borchelt D. R., (2005) Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA. Neurobiol. Dis. 20, 943-952. (Pubitemid 41619369)
-
(2005)
Neurobiology of Disease
, vol.20
, Issue.3
, pp. 943-952
-
-
Wang, J.1
Xu, G.2
Slunt, H.H.3
Gonzales, V.4
Coonfield, M.5
Fromholt, D.6
Copeland, N.G.7
Jenkins, N.A.8
Borchelt, D.R.9
-
43
-
-
0029053881
-
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
-
Wong P. C., Pardo C. A., Borchelt D. R., Lee M. K., Copeland N. G., Jenkins N. A., Sisodia S. S., Cleveland D. W., and, Price D. L., (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.
-
(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
-
44
-
-
35348874857
-
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
-
DOI 10.1073/pnas.0700477104
-
Zetterstrom P., Stewart H. G., Bergemalm D., Jonsson P. A., Graffmo K. S., Andersen P. M., Brannstrom T., Oliveberg M., and, 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. U S A 104, 14157-14162. (Pubitemid 350003351)
-
(2007)
Proceedings of the National Academy of Sciences of the United States of America
, vol.104
, Issue.35
, 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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