-
1
-
-
0344507132
-
Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis
-
W. Bruening, J. Roy, B. Giasson, D.A. Figlewicz, W.E. Mushynski, and H.D. Durham Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis J. Neurochem. 72 1999 693 699
-
(1999)
J. Neurochem.
, vol.72
, pp. 693-699
-
-
Bruening, W.1
Roy, J.2
Giasson, B.3
Figlewicz, D.A.4
Mushynski, W.E.5
Durham, H.D.6
-
2
-
-
0031051485
-
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
-
L.I. Bruijn, M.W. Becher, M.K. Lee, K.L. Anderson, N.A. Jenkins, N.G. Copeland, S.S. Sisodia, J.D. Rothstein, D.R. Borchelt, D.L. Price, and D.W. Cleveland ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions Neuron 18 1997 327 338
-
(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
-
3
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
-
L.I. Bruijn, M.K. Houseweart, S. Kato, K.L. Anderson, S.D. Anderson, E. Ohama, A.G. Reaume, R.W. Scott, and D.W. Cleveland Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1 Science 281 1998 1851 1854
-
(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
-
4
-
-
0029739282
-
A simple assay to determine the functionality of Cre or FLP recombination targets in genomic manipulation constructs
-
F. Buchholz, P.O. Angrand, and A.F. Stewart A simple assay to determine the functionality of Cre or FLP recombination targets in genomic manipulation constructs Nucleic Acids Res. 24 1996 3118 3119
-
(1996)
Nucleic Acids Res.
, vol.24
, pp. 3118-3119
-
-
Buchholz, F.1
Angrand, P.O.2
Stewart, A.F.3
-
5
-
-
0141642203
-
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice
-
A.M. Clement, M.D. Nguyen, E.A. Roberts, M.L. Garcia, S. Boillee, M. Rule, A.P. McMahon, W. Doucette, D. Siwek, R.J. Ferrante, R.H. Brown Jr., J.P. Julien, L.S. Goldstein, and D.W. Cleveland Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice Science 302 2003 113 117
-
(2003)
Science
, vol.302
, pp. 113-117
-
-
Clement, A.M.1
Nguyen, M.D.2
Roberts, E.A.3
Garcia, M.L.4
Boillee, S.5
Rule, M.6
McMahon, A.P.7
Doucette, W.8
Siwek, D.9
Ferrante, R.J.10
Brown Jr., R.H.11
Julien, J.P.12
Goldstein, L.S.13
Cleveland, D.W.14
-
6
-
-
0035516124
-
From Charcot to Lou Gehrig: Deciphering selective motor neuron death in ALS
-
D.W. Cleveland, and J.D. Rothstein From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS Nat. Rev., Neurosci. 2 2001 806 819
-
(2001)
Nat. Rev., Neurosci.
, vol.2
, pp. 806-819
-
-
Cleveland, D.W.1
Rothstein, J.D.2
-
7
-
-
0042736853
-
ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization
-
M. DiDonato, L. Craig, M.E. Huff, M.M. Thayer, R.M. Cardoso, C.J. Kassmann, T.P. Lo, C.K. Bruns, E.T. Powers, J.W. Kelly, E.D. Getzoff, and J.A. Tainer ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization J. Mol. Biol. 332 2003 601 615
-
(2003)
J. Mol. Biol.
, vol.332
, pp. 601-615
-
-
Didonato, M.1
Craig, L.2
Huff, M.E.3
Thayer, M.M.4
Cardoso, R.M.5
Kassmann, C.J.6
Lo, T.P.7
Bruns, C.K.8
Powers, E.T.9
Kelly, J.W.10
Getzoff, E.D.11
Tainer, J.A.12
-
8
-
-
0347357617
-
Protein folding and misfolding
-
C.M. Dobson Protein folding and misfolding Nature 426 2003 884 890
-
(2003)
Nature
, vol.426
, pp. 884-890
-
-
Dobson, C.M.1
-
9
-
-
0038442784
-
Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS
-
J.S. Elam, A.B. Taylor, R. Strange, S. Antonyuk, P.A. Doucette, J.A. Rodriguez, S.S. Hasnain, L.J. Hayward, J.S. Valentine, T.O. Yeates, and P.J. Hart Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS Nat. Struct. Biol. 10 2003 461 467
-
(2003)
Nat. Struct. Biol.
, vol.10
, pp. 461-467
-
-
Elam, J.S.1
Taylor, A.B.2
Strange, R.3
Antonyuk, S.4
Doucette, P.A.5
Rodriguez, J.A.6
Hasnain, S.S.7
Hayward, L.J.8
Valentine, J.S.9
Yeates, T.O.10
Hart, P.J.11
-
10
-
-
0038275772
-
Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
-
E. Flechsig, I. Hegyi, R. Leimeroth, A. Zuniga, D. Rossi, A. Cozzio, P. Schwarz, T. Rulicke, J. Gotz, A. Aguzzi, and C. Weissmann Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia EMBO J. 22 2003 3095 3101
-
(2003)
EMBO J.
, vol.22
, pp. 3095-3101
-
-
Flechsig, E.1
Hegyi, I.2
Leimeroth, R.3
Zuniga, A.4
Rossi, D.5
Cozzio, A.6
Schwarz, P.7
Rulicke, T.8
Gotz, J.9
Aguzzi, A.10
Weissmann, C.11
-
11
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
-
M.E. Gurney, H. Pu, A.Y. Chiu, M.C. Dal Canto, C.Y. Polchow, D.D. Alexander, J. Caliendo, A. Hentati, Y.W. Kwon, and H.X. Deng Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation Science 264 1994 1772 1775 (see comments, published erratum appears in Science 1995 Jul. 14;269(5221):149)
-
(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
-
12
-
-
0026335854
-
Thermostabilization of recombinant human and bovine CuZn superoxide dismutases by replacement of free cysteines
-
R.A. Hallewell, K.C. Imlay, P. Lee, N.M. Fong, C. Gallegos, E.D. Getzoff, J.A. Tainer, D.E. Cabelli, P. Tekamp-Olson, and G.T. Mullenbach Thermostabilization of recombinant human and bovine CuZn superoxide dismutases by replacement of free cysteines Biochem. Biophys. Res. Commun. 181 1991 474 480
-
(1991)
Biochem. Biophys. Res. Commun.
, vol.181
, pp. 474-480
-
-
Hallewell, R.A.1
Imlay, K.C.2
Lee, P.3
Fong, N.M.4
Gallegos, C.5
Getzoff, E.D.6
Tainer, J.A.7
Cabelli, D.E.8
Tekamp-Olson, P.9
Mullenbach, G.T.10
-
13
-
-
0037013264
-
Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis
-
L.J. Hayward, J.A. Rodriguez, J.W. Kim, A. Tiwari, J.J. Goto, D.E. Cabelli, J.S. Valentine, and R.H. Brown Jr. Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis J. Biol. Chem. 277 2002 15923 15931 (electronic publication 12002 Feb. 15919)
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 15923-15931
-
-
Hayward, L.J.1
Rodriguez, J.A.2
Kim, J.W.3
Tiwari, A.4
Goto, J.J.5
Cabelli, D.E.6
Valentine, J.S.7
Brown Jr., R.H.8
-
14
-
-
0033749379
-
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
-
J.A. Johnston, M.J. Dalton, M.E. Gurney, and R.R. Kopito 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 2000 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
-
15
-
-
0038521282
-
Polyglutamines placed into context
-
A.R. La Spada, and J.P. Taylor Polyglutamines placed into context Neuron 38 2003 681 684
-
(2003)
Neuron
, vol.38
, pp. 681-684
-
-
La Spada, A.R.1
Taylor, J.P.2
-
16
-
-
0036724314
-
No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis
-
J.P. Lee, C. Gerin, V. Bindokas, R.J. Miller, G.D. Ghadge, and R.P. Roos No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis J. Neurochem. 82 2002 1229 1238
-
(2002)
J. Neurochem.
, vol.82
, pp. 1229-1238
-
-
Lee, J.P.1
Gerin, C.2
Bindokas, V.3
Miller, R.J.4
Ghadge, G.D.5
Roos, R.P.6
-
17
-
-
0037059063
-
De novo designed peptide-based amyloid fibrils
-
M. Lopez De La Paz, K. Goldie, J. Zurdo, E. Lacroix, C.M. Dobson, A. Hoenger, and L. Serrano De novo designed peptide-based amyloid fibrils Proc. Natl. Acad. Sci. U. S. A. 99 2002 16052 16057 (electronic publication 12002 Nov. 16027)
-
(2002)
Proc. Natl. Acad. Sci. U. S. A.
, vol.99
, pp. 16052-16057
-
-
Lopez De La Paz, M.1
Goldie, K.2
Zurdo, J.3
Lacroix, E.4
Dobson, C.M.5
Hoenger, A.6
Serrano, L.7
-
18
-
-
0032981398
-
Efficient targeting of gene expression in chick embryos by microelectroporation
-
T. Momose, A. Tonegawa, J. Takeuchi, H. Ogawa, K. Umesono, and K. Yasuda Efficient targeting of gene expression in chick embryos by microelectroporation Dev. Growth Differ. 41 1999 335 344
-
(1999)
Dev. Growth Differ.
, vol.41
, pp. 335-344
-
-
Momose, T.1
Tonegawa, A.2
Takeuchi, J.3
Ogawa, H.4
Umesono, K.5
Yasuda, K.6
-
19
-
-
0031566158
-
Comparison of three nonviral transfection methods for foreign gene expression in early chicken embryos in ovo
-
T. Muramatsu, Y. Mizutani, Y. Ohmori, and J. Okumura Comparison of three nonviral transfection methods for foreign gene expression in early chicken embryos in ovo Biochem. Biophys. Res. Commun. 230 1997 376 380
-
(1997)
Biochem. Biophys. Res. Commun.
, vol.230
, pp. 376-380
-
-
Muramatsu, T.1
Mizutani, Y.2
Ohmori, Y.3
Okumura, J.4
-
20
-
-
0028067985
-
A two basepair deletion in the SOD 1 gene causes familial amyotrophic lateral sclerosis
-
A. Pramatarova, J. Goto, E. Nanba, K. Nakashima, K. Takahashi, A. Takagi, I. Kanazawa, D.A. Figlewicz, and G.A. Rouleau A two basepair deletion in the SOD 1 gene causes familial amyotrophic lateral sclerosis Hum. Mol. Genet. 3 1994 2061 2062
-
(1994)
Hum. Mol. Genet.
, vol.3
, pp. 2061-2062
-
-
Pramatarova, A.1
Goto, J.2
Nanba, E.3
Nakashima, K.4
Takahashi, K.5
Takagi, A.6
Kanazawa, I.7
Figlewicz, D.A.8
Rouleau, G.A.9
-
21
-
-
2142761528
-
An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis
-
S.S. Ray, R.J. Nowak, K. Strokovich, R.H. Brown Jr., T. Walz, and P.T. Lansbury Jr. An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis Biochemistry 43 2004 4899 4905
-
(2004)
Biochemistry
, vol.43
, pp. 4899-4905
-
-
Ray, S.S.1
Nowak, R.J.2
Strokovich, K.3
Brown Jr., R.H.4
Walz, T.5
Lansbury Jr., P.T.6
-
22
-
-
15844393658
-
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
-
A.G. Reaume, J.L. Elliott, E.K. Hoffman, N.W. Kowall, R.J. Ferrante, D.F. Siwek, H.M. Wilcox, D.G. Flood, M.F. Beal, R.H. Brown Jr., R.W. Scott, and W.D. Snider Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury Nat. Genet. 13 1996 43 47
-
(1996)
Nat. Genet.
, vol.13
, pp. 43-47
-
-
Reaume, A.G.1
Elliott, J.L.2
Hoffman, E.K.3
Kowall, N.W.4
Ferrante, R.J.5
Siwek, D.F.6
Wilcox, H.M.7
Flood, D.G.8
Beal, M.F.9
Brown Jr., R.H.10
Scott, R.W.11
Snider, W.D.12
-
23
-
-
0037022563
-
Natural beta-sheet proteins use negative design to avoid edge-to-edge aggregation
-
J.S. Richardson, and D.C. Richardson Natural beta-sheet proteins use negative design to avoid edge-to-edge aggregation Proc. Natl. Acad. Sci. U. S. A. 99 2002 2754 2759
-
(2002)
Proc. Natl. Acad. Sci. U. S. A.
, vol.99
, pp. 2754-2759
-
-
Richardson, J.S.1
Richardson, D.C.2
-
24
-
-
0347987853
-
Folding proteins in fatal ways
-
D.J. Selkoe Folding proteins in fatal ways Nature 426 2003 900 904
-
(2003)
Nature
, vol.426
, pp. 900-904
-
-
Selkoe, D.J.1
-
25
-
-
0035918258
-
Mutant Cu/Zn-Superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis
-
G. Shinder, M.-C. Lacourse, S. Minotti, and H.D. Durham 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 2001 12791 12796
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 12791-12796
-
-
Shinder, G.1
Lacourse, M.-C.2
Minotti, S.3
Durham, H.D.4
-
26
-
-
0344944630
-
Protein aggregation and aggregate toxicity: New insights into protein folding, misfolding diseases and biological evolution
-
M. Stefani, and C.M. Dobson Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution J. Mol. Med. 81 2003 678 699 (electronic publication 2003 Aug. 2027)
-
(2003)
J. Mol. Med.
, vol.81
, pp. 678-699
-
-
Stefani, M.1
Dobson, C.M.2
-
27
-
-
0035099885
-
A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice
-
S. Supattapone, E. Bouzamondo, H.L. Ball, H. Wille, H.O. Nguyen, F.E. Cohen, S.J. DeArmond, S.B. Prusiner, and M. Scott A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice Mol. Cell. Biol. 21 2001 2608 2616
-
(2001)
Mol. Cell. Biol.
, vol.21
, pp. 2608-2616
-
-
Supattapone, S.1
Bouzamondo, E.2
Ball, H.L.3
Wille, H.4
Nguyen, H.O.5
Cohen, F.E.6
Dearmond, S.J.7
Prusiner, S.B.8
Scott, M.9
-
28
-
-
0035887995
-
Long-lasting aberrant tubulovesicular membrane inclusions accumulate in developing motoneurons after a sublethal excitotoxic insult: A possible model for neuronal pathology in neurodegenerative disease
-
O. Tarabal, J. Caldero, J. Llado, R.W. Oppenheim, and J.E. Esquerda Long-lasting aberrant tubulovesicular membrane inclusions accumulate in developing motoneurons after a sublethal excitotoxic insult: a possible model for neuronal pathology in neurodegenerative disease J. Neurosci. 21 2001 8072 8081
-
(2001)
J. Neurosci.
, vol.21
, pp. 8072-8081
-
-
Tarabal, O.1
Caldero, J.2
Llado, J.3
Oppenheim, R.W.4
Esquerda, J.E.5
-
29
-
-
0037044732
-
Charge attraction and beta propensity are necessary for amyloid fibril formation from tetrapeptides
-
L. Tjernberg, W. Hosia, N. Bark, J. Thyberg, and J. Johansson Charge attraction and beta propensity are necessary for amyloid fibril formation from tetrapeptides J. Biol. Chem. 277 2002 43243 43246 (electronic publication 42002 Sep. 43244)
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 43243-43246
-
-
Tjernberg, L.1
Hosia, W.2
Bark, N.3
Thyberg, J.4
Johansson, J.5
-
30
-
-
0036199623
-
High molecular weight complexes of mutant superoxide dismutase 1: Age-dependent and tissue-specific accumulation
-
J. Wang, G. Xu, and D.R. Borchelt High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation Neurobiol. Dis. 9 2002 139 148
-
(2002)
Neurobiol. Dis.
, vol.9
, pp. 139-148
-
-
Wang, J.1
Xu, G.2
Borchelt, D.R.3
-
31
-
-
0242524455
-
Copper-binding-site-null SOD1 causes ALS in transgenic mice: Aggregates of non-native SOD1 delineate a common feature
-
J. Wang, H. Slunt, V. Gonzales, D. Fromholt, M. Coonfield, N.G. Copeland, N.A. Jenkins, and D.R. Borchelt Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature Hum. Mol. Genet. 12 2003 2753 2764 (electronic publication 2003 Sep. 2759)
-
(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
-
32
-
-
0030990991
-
Disruption of overlapping transcripts in the ROSA beta geo 26 gene trap strain leads to widespread expression of beta-galactosidase in mouse embryos and hematopoietic cells
-
B.P. Zambrowicz, A. Imamoto, S. Fiering, L.A. Herzenberg, W.G. Kerr, and P. Soriano Disruption of overlapping transcripts in the ROSA beta geo 26 gene trap strain leads to widespread expression of beta-galactosidase in mouse embryos and hematopoietic cells Proc. Natl. Acad. Sci. U. S. A. 94 1997 3789 3794
-
(1997)
Proc. Natl. Acad. Sci. U. S. A.
, vol.94
, pp. 3789-3794
-
-
Zambrowicz, B.P.1
Imamoto, A.2
Fiering, S.3
Herzenberg, L.A.4
Kerr, W.G.5
Soriano, P.6
-
33
-
-
0031128772
-
Exon 5 encoded domain is not required for the toxic function of mutant SOD1 but essential for the dismutase activity: Identification and characterization of two new SOD1 mutations associated with familial amyotrophic lateral sclerosis
-
J.S. Zu, H.X. Deng, T.P. Lo, H. Mitsumoto, M.S. Ahmed, W.Y. Hung, Z.J. Cai, J.A. Tainer, and T. Siddique Exon 5 encoded domain is not required for the toxic function of mutant SOD1 but essential for the dismutase activity: identification and characterization of two new SOD1 mutations associated with familial amyotrophic lateral sclerosis Neurogenetics 1 1997 65 71
-
(1997)
Neurogenetics
, vol.1
, pp. 65-71
-
-
Zu, J.S.1
Deng, H.X.2
Lo, T.P.3
Mitsumoto, H.4
Ahmed, M.S.5
Hung, W.Y.6
Cai, Z.J.7
Tainer, J.A.8
Siddique, T.9
|