-
1
-
-
0037068826
-
Fas(t) balls and Lou Gehrig disease. A clue to selective vulnerability of motor neurons?
-
Xiong, Z. Q., and McNamara, J. O. (2002) Fas(t) balls and Lou Gehrig disease. A clue to selective vulnerability of motor neurons? Neuron 35, 1011-1013.
-
(2002)
Neuron
, vol.35
, pp. 1011-1013
-
-
Xiong, Z.Q.1
McNamara, J.O.2
-
2
-
-
0035516124
-
From Charcot to Lou Gehrig: Deciphering selective motor neuron death in ALS
-
Cleveland, D. W., and Rothstein, J. D. (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS, Nat. Rev. Neurosci. 2, 806-819.
-
(2001)
Nat. Rev. Neurosci.
, vol.2
, pp. 806-819
-
-
Cleveland, D.W.1
Rothstein, J.D.2
-
3
-
-
0029584941
-
Superoxide dismutase in familial amyotrophic lateral sclerosis: Models for gain of function
-
Brown, R. H., Jr. (1995) Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function, Curr. Opin. Neurobiol. 5, 841-846.
-
(1995)
Curr. Opin. Neurobiol.
, vol.5
, pp. 841-846
-
-
Brown Jr., R.H.1
-
4
-
-
0028105029
-
A transgenic-mouse model of amyotrophic lateral sclerosis
-
Brown, R. H. (1994) A transgenic-mouse model of amyotrophic lateral sclerosis, N. Engl. J. Med. 331, 1091-1092.
-
(1994)
N. Engl. J. Med.
, vol.331
, pp. 1091-1092
-
-
Brown, R.H.1
-
5
-
-
0028811152
-
Toxic mutants in Charcot's sclerosis
-
Cleveland, D. W., Laing, N., Hurse, P. V., and Brown, R. H., Jr. (1995) Toxic mutants in Charcot's sclerosis, Nature 378, 342-343.
-
(1995)
Nature
, vol.378
, pp. 342-343
-
-
Cleveland, D.W.1
Laing, N.2
Hurse, P.V.3
Brown Jr., R.H.4
-
6
-
-
0034961104
-
Amyotrophic lateral sclerosis: Pathogenesis
-
Brown, R. H., Jr., and Robberecht, W. (2001) Amyotrophic lateral sclerosis: pathogenesis, Semin. Neurol. 21, 131-139.
-
(2001)
Semin. Neurol.
, vol.21
, pp. 131-139
-
-
Brown Jr., R.H.1
Robberecht, W.2
-
7
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
-
Bruijn, L. I., Houseweart, M. K., Kato, S., Anderson, K. L., Anderson, S. D., Ohama, E., Reaume, A. G., Scott, R. W., and Cleveland, D. W. (1998) Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1, Science 281, 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
-
8
-
-
0027359334
-
Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis
-
Bowling, A. C., Schulz, J. B., Brown, R. H., Jr., and Beal, M. F. (1993) Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis, J. Neurochem. 61, 2322-2325.
-
(1993)
J. Neurochem.
, vol.61
, pp. 2322-2325
-
-
Bowling, A.C.1
Schulz, J.B.2
Brown Jr., R.H.3
Beal, M.F.4
-
9
-
-
0027965073
-
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity
-
Borchelt, D. R., Lee, M. K., Slunt, H. S., Guarnieri, M., Xu, Z. S., Wong, P. C., Brown, R. H., Jr., Price, D. L., Sisodia, S. S., and Cleveland, D. W. (1994) Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity, Proc. Natl. Acad. Sci. U.S.A. 91, 8292-8296.
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 8292-8296
-
-
Borchelt, D.R.1
Lee, M.K.2
Slunt, H.S.3
Guarnieri, M.4
Xu, Z.S.5
Wong, P.C.6
Brown Jr., R.H.7
Price, D.L.8
Sisodia, S.S.9
Cleveland, D.W.10
-
10
-
-
0028205372
-
Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons
-
Rothstein, J. D., Bristol, L. A., Hosler, B., Brown, R. H., Jr., and Kuncl, R. W. (1994) Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons, Proc. Natl. Acad. Sci. U.S.A. 91, 4155-4159.
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 4155-4159
-
-
Rothstein, J.D.1
Bristol, L.A.2
Hosler, B.3
Brown Jr., R.H.4
Kuncl, R.W.5
-
11
-
-
0037168643
-
Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: Decreased stability of the apo state
-
Lindberg, M. J., Tibell, L., and Oliveberg, M. (2002) Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: decreased stability of the apo state, Proc. Natl. Acad. Sci. U.S.A. 99, 16607-16612.
-
(2002)
Proc. Natl. Acad. Sci. U.S.A.
, vol.99
, pp. 16607-16612
-
-
Lindberg, M.J.1
Tibell, L.2
Oliveberg, M.3
-
12
-
-
0037013264
-
Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis
-
Hayward, L. J., Rodriguez, J. A., Kim, J. W., Tiwari, A., Goto, J. J., Cabelli, D. E., Valentine, J. S., and Brown, R. H., Jr. (2002) Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis, J. Biol. Chem. 277, 15923-15931.
-
(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
-
13
-
-
0030596537
-
Instability of mutant Cu/Zn superoxide dismutase (Ala4Thr) associated with familial amyotrophic lateral sclerosis
-
Nakano, R., Inuzuka, T., Kikugawa, K., Takahashi, H., Sakimura, K., Fujii, J., Taniguchi, N., and Tsuji, S. (1996) Instability of mutant Cu/Zn superoxide dismutase (Ala4Thr) associated with familial amyotrophic lateral sclerosis, Neurosci. Lett. 211, 129-131.
-
(1996)
Neurosci. Lett.
, vol.211
, pp. 129-131
-
-
Nakano, R.1
Inuzuka, T.2
Kikugawa, K.3
Takahashi, H.4
Sakimura, K.5
Fujii, J.6
Taniguchi, N.7
Tsuji, S.8
-
14
-
-
0027426169
-
Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase
-
Deng, H. X., Hentati, A., Tainer, J. A., Iqbal, Z., Cayabyab, A., Hung, W. Y., Getzoff, E. D., Hu, P., Herzfeldt, B., Rood, R. P., Warner, C., Deng, G., Soriano, E., Smyth, C., Parge, H. E., Ahmed, A., Roses, A. D., Hallewell, R. A., Perciak-Vance, M. A., and Siddique, T. (1993) Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase, Science 261, 1047-1051.
-
(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
Rood, R.P.10
Warner, C.11
Deng, G.12
Soriano, E.13
Smyth, C.14
Parge, H.E.15
Ahmed, A.16
Roses, A.D.17
Hallewell, R.A.18
Perciak-Vance, M.A.19
Siddique, T.20
more..
-
15
-
-
0035936804
-
Amyotrophic lateral sclerosis. Unfolding the toxicity of the misfolded
-
Julien, J. P. (2001) Amyotrophic lateral sclerosis. Unfolding the toxicity of the misfolded, Cell 104, 581-591.
-
(2001)
Cell
, vol.104
, pp. 581-591
-
-
Julien, J.P.1
-
16
-
-
0037130174
-
Neurodegenerative disease: Amyloid pores from pathogenic mutations
-
Lashuel, H. A., Hartley, D., Petre, B. M., Walz, T., and Lansbury, P. T., Jr. (2002) Neurodegenerative disease: amyloid pores from pathogenic mutations, Nature 418, 291.
-
(2002)
Nature
, vol.418
, pp. 291
-
-
Lashuel, H.A.1
Hartley, D.2
Petre, B.M.3
Walz, T.4
Lansbury Jr., P.T.5
-
17
-
-
0038004727
-
Superoxide dismutase folding/unfolding pathway: Role of the metal ions in modulating structural and dynamical features
-
Assfalg, M., Banci, L., Bertini, I., Turano, P., and Vasos, P. R. (2003) Superoxide dismutase folding/unfolding pathway: role of the metal ions in modulating structural and dynamical features, J. Mol. Biol. 330, 145-158.
-
(2003)
J. Mol. Biol.
, vol.330
, pp. 145-158
-
-
Assfalg, M.1
Banci, L.2
Bertini, I.3
Turano, P.4
Vasos, P.R.5
-
18
-
-
0037795635
-
Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro
-
Stathopulos, P. B., Rumfeldt, J. A., Scholz, G. A., Irani, R. A., Frey, H. E., Hallewell, R. A., Lepock, J. R., and Meiering, E. M. (2003) Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro, Proc. Natl. Acad. Sci. U.S.A. 100, 7021-7026.
-
(2003)
Proc. Natl. Acad. Sci. U.S.A.
, vol.100
, pp. 7021-7026
-
-
Stathopulos, P.B.1
Rumfeldt, J.A.2
Scholz, G.A.3
Irani, R.A.4
Frey, H.E.5
Hallewell, R.A.6
Lepock, J.R.7
Meiering, E.M.8
-
19
-
-
0036440686
-
Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase
-
Cardoso, R. M., Thayer, M. M., DiDonato, M., Lo, T. P., Bruns, C. K., Getzoff, E. D., and Tainer, J. A. (2002) Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase, J. Mol. Biol. 324, 247-256.
-
(2002)
J. Mol. Biol.
, vol.324
, pp. 247-256
-
-
Cardoso, R.M.1
Thayer, M.M.2
DiDonato, M.3
Lo, T.P.4
Bruns, C.K.5
Getzoff, E.D.6
Tainer, J.A.7
-
20
-
-
0031577722
-
Stability of mutant superoxide dismutase-1 associated with familial amyotrophic lateral sclerosis determines the manner of copper release and induction of thioredoxin in erythrocytes
-
Ogawa, Y., Kosaka, H., Nakanishi, T., Shimizu, A., Ohoi, N., Shouji, H., Yanagihara, T., and Sakoda, S. (1997) Stability of mutant superoxide dismutase-1 associated with familial amyotrophic lateral sclerosis determines the manner of copper release and induction of thioredoxin in erythrocytes, Biochem. Biophys. Res. Commun. 241, 251-257.
-
(1997)
Biochem. Biophys. Res. Commun.
, vol.241
, pp. 251-257
-
-
Ogawa, Y.1
Kosaka, H.2
Nakanishi, T.3
Shimizu, A.4
Ohoi, N.5
Shouji, H.6
Yanagihara, T.7
Sakoda, S.8
-
21
-
-
0031025061
-
Instability of expressed Cu/Zn superoxide dismutase with 2 bp deletion found in familial amyotrophic lateral sclerosis
-
Watanabe, Y., Kono, Y., Nanba, E., Ohama, E., and Nakashima, K. (1997) Instability of expressed Cu/Zn superoxide dismutase with 2 bp deletion found in familial amyotrophic lateral sclerosis, FEBS Lett. 400, 108-112.
-
(1997)
FEBS Lett.
, vol.400
, pp. 108-112
-
-
Watanabe, Y.1
Kono, Y.2
Nanba, E.3
Ohama, E.4
Nakashima, K.5
-
22
-
-
0025929954
-
Advances in the understanding of the structure-function relationship in Cu,Zn superoxide dismutase
-
Banci, L., Bertini, I., Cabelli, D. E., Hallewell, R. A., Luchinat, C., and Viezzoli, M. S. (1991) Advances in the understanding of the structure-function relationship in Cu,Zn superoxide dismutase, Free Radical Res. Commun. 12-13 (Part 1), 239-251.
-
(1991)
Free Radical Res. Commun.
, vol.12-13
, Issue.PART 1
, pp. 239-251
-
-
Banci, L.1
Bertini, I.2
Cabelli, D.E.3
Hallewell, R.A.4
Luchinat, C.5
Viezzoli, M.S.6
-
23
-
-
0025074777
-
Changes in crystallographic structure and thermostability of a Cu,Zn superoxide dismutase mutant resulting from the removal of a buried cysteine
-
McRee, D. E., Redford, S. M., Getzoff, E. D., Lepock, J. R., Hallewell, R. A., and Tainer, J. A. (1990) Changes in crystallographic structure and thermostability of a Cu,Zn superoxide dismutase mutant resulting from the removal of a buried cysteine, J. Biol. Chem. 265, 14234-14241.
-
(1990)
J. Biol. Chem.
, vol.265
, pp. 14234-14241
-
-
McRee, D.E.1
Redford, S.M.2
Getzoff, E.D.3
Lepock, J.R.4
Hallewell, R.A.5
Tainer, J.A.6
-
24
-
-
0038627546
-
The perplexing role of copper-zinc superoxide dismutase in amyotrophic lateral sclerosis (Lou Gehrig's disease)
-
Potter, S. Z., and Valentine, J. S. (2003) The perplexing role of copper-zinc superoxide dismutase in amyotrophic lateral sclerosis (Lou Gehrig's disease), J. Biol. Inorg. Chem. 8, 373-380.
-
(2003)
J. Biol. Inorg. Chem.
, vol.8
, pp. 373-380
-
-
Potter, S.Z.1
Valentine, J.S.2
-
25
-
-
0028915976
-
Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: Studies in yeast and neural cells
-
Rabizadeh, S., Gralla, E. B., Borchelt, D. R., Gwinn, R., Valentine, J. S., Sisodia, S., Wong, P., Lee, M., Hahn, H., and Bredesen, D. E. (1995) Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells, Proc. Natl. Acad. Sci. U.S.A. 92, 3024-3028.
-
(1995)
Proc. Natl. Acad. Sci. U.S.A.
, vol.92
, pp. 3024-3028
-
-
Rabizadeh, S.1
Gralla, E.B.2
Borchelt, D.R.3
Gwinn, R.4
Valentine, J.S.5
Sisodia, S.6
Wong, P.7
Lee, M.8
Hahn, H.9
Bredesen, D.E.10
-
26
-
-
0028149084
-
Characterization of three yeast copper-zinc superoxide dismutase mutants analogous to those coded for in familial amyotrophic lateral sclerosis
-
Nishida, C. R., Gralla, E. B., and Valentine, J. S. (1994) Characterization of three yeast copper-zinc superoxide dismutase mutants analogous to those coded for in familial amyotrophic lateral sclerosis, Proc. Natl. Acad. Sci. U.S.A. 91, 9906-9910.
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 9906-9910
-
-
Nishida, C.R.1
Gralla, E.B.2
Valentine, J.S.3
-
27
-
-
0031015422
-
Prognosis in familial amyotrophic lateral sclerosis: Progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase
-
Juneja, T., Pericak-Vance, M. A., Laing, N. G., Dave, S., and Siddique, T. (1997) Prognosis in familial amyotrophic lateral sclerosis: progression and survival in patients with glu100gly and ala4val mutations in Cu,Zn superoxide dismutase, Neurology 48, 55-57.
-
(1997)
Neurology
, vol.48
, pp. 55-57
-
-
Juneja, T.1
Pericak-Vance, M.A.2
Laing, N.G.3
Dave, S.4
Siddique, T.5
-
28
-
-
0035892642
-
Assay of superoxide dismutase: Cautions relevant to the use of cytochrome c, a sulfonated tetrazolium, and cyanide
-
Okado-Matsumoto, A., and Fridovich, I. (2001) Assay of superoxide dismutase: cautions relevant to the use of cytochrome c, a sulfonated tetrazolium, and cyanide, Anal. Biochem. 298, 337-342.
-
(2001)
Anal. Biochem.
, vol.298
, pp. 337-342
-
-
Okado-Matsumoto, A.1
Fridovich, I.2
-
29
-
-
0026054085
-
Mechanism and atomic structure of superoxide dismutase
-
Roberts, V. A., Fisher, C. L., Redford, S. M., McRee, D. E., Parge, H. E., Getzoff, E. D., and Tainer, J. A. (1991) Mechanism and atomic structure of superoxide dismutase, Free Radical Res. Commun. 12-13 (Part 1), 269-278.
-
(1991)
Free Radical Res. Commun.
, vol.12-13
, Issue.PART 1
, pp. 269-278
-
-
Roberts, V.A.1
Fisher, C.L.2
Redford, S.M.3
McRee, D.E.4
Parge, H.E.5
Getzoff, E.D.6
Tainer, J.A.7
-
30
-
-
0030900245
-
A familial amyotrophic lateral sclerosis-associated A4V Cu, Zn-superoxide dismutase mutant has a lower Km for hydrogen peroxide. Correlation between clinical severity and the Km value
-
Yim, H. S., Kang, J. H., Chock, P. B., Stadtman, E. R., and Yim, M. B. (1997) A familial amyotrophic lateral sclerosis-associated A4V Cu, Zn-superoxide dismutase mutant has a lower Km for hydrogen peroxide. Correlation between clinical severity and the Km value, J. Biol. Chem. 272, 8861-8863.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 8861-8863
-
-
Yim, H.S.1
Kang, J.H.2
Chock, P.B.3
Stadtman, E.R.4
Yim, M.B.5
-
31
-
-
0036415838
-
Alpha-synuclein, especially the Parkinson's disease-associated mutants, forms pore-like annular and tubular protofibrils
-
Lashuel, H. A., Petre, B. M., Wall, J., Simon, M., Nowak, R. J., Walz, T., and Lansbury, P. T., Jr. (2002) Alpha-synuclein, especially the Parkinson's disease-associated mutants, forms pore-like annular and tubular protofibrils, J. Mol. Biol. 322, 1089-1102.
-
(2002)
J. Mol. Biol.
, vol.322
, pp. 1089-1102
-
-
Lashuel, H.A.1
Petre, B.M.2
Wall, J.3
Simon, M.4
Nowak, R.J.5
Walz, T.6
Lansbury Jr., P.T.7
-
32
-
-
0024997658
-
Conformations of disulfide bridges in proteins
-
Srinivasan, N., Sowdhamini, R., Ramakrishnan, C., and Balaram, P. (1990) Conformations of disulfide bridges in proteins, Int. J. Pept. Protein Res. 36, 147-155.
-
(1990)
Int. J. Pept. Protein Res.
, vol.36
, pp. 147-155
-
-
Srinivasan, N.1
Sowdhamini, R.2
Ramakrishnan, C.3
Balaram, P.4
-
33
-
-
0024818499
-
Stereochemical modeling of disulfide bridges. Criteria for introduction into proteins by site-directed mutagenesis
-
Sowdhamini, R., Srinivasan, N., Shoichet, B., Santi, D. V., Ramakrishnan, C., and Balaram, P. (1989) Stereochemical modeling of disulfide bridges. Criteria for introduction into proteins by site-directed mutagenesis, Protein Eng. 3, 95-103.
-
(1989)
Protein Eng.
, vol.3
, pp. 95-103
-
-
Sowdhamini, R.1
Srinivasan, N.2
Shoichet, B.3
Santi, D.V.4
Ramakrishnan, C.5
Balaram, P.6
-
34
-
-
0027489388
-
Modelling multiple disulphide loop containing polypeptides by random conformation generation. The test cases of alpha-conotoxin GI and endothelin I
-
Sowdhamini, R., Ramakrishnan, C., and Balaram, P. (1993) Modelling multiple disulphide loop containing polypeptides byrandom conformation generation. The test cases of alpha-conotoxin GI and endothelin I, Protein Eng. 6, 873-882.
-
(1993)
Protein Eng.
, vol.6
, pp. 873-882
-
-
Sowdhamini, R.1
Ramakrishnan, C.2
Balaram, P.3
-
35
-
-
0028111860
-
X-ray, NMR and molecular dynamics studies on reduced bovine superoxide dismutase: Implications for the mechanism
-
Banci, L., Bertini, I., Bruni, B., Carloni, P., Luchinat, C., Mangani, S., Orioli, P. L., Piccioli, M., Ripniewski, W., and Wilson, K. S. (1994) X-ray, NMR and molecular dynamics studies on reduced bovine superoxide dismutase: implications for the mechanism, Biochem. Biophys. Res. Commun. 202, 1088-1095.
-
(1994)
Biochem. Biophys. Res. Commun.
, vol.202
, pp. 1088-1095
-
-
Banci, L.1
Bertini, I.2
Bruni, B.3
Carloni, P.4
Luchinat, C.5
Mangani, S.6
Orioli, P.L.7
Piccioli, M.8
Ripniewski, W.9
Wilson, K.S.10
-
36
-
-
0344495441
-
Cu/Zn superoxide dismutase can form pore-like structures
-
Chung, J., Yang, H., de Beus, M. D., Ryu, C. Y., Cho, K., and Colon, W. (2003) Cu/Zn superoxide dismutase can form pore-like structures, Biochem. Biophys. Res. Commun. 312, 873-876.
-
(2003)
Biochem. Biophys. Res. Commun.
, vol.312
, pp. 873-876
-
-
Chung, J.1
Yang, H.2
De Beus, M.D.3
Ryu, C.Y.4
Cho, K.5
Colon, W.6
-
37
-
-
0037742610
-
An alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase: Rates enhanced via proposed enzyme-associated peroxycarbonate intermediate
-
Elam, J. S., Malek, K., Rodriguez, J. A., Doucette, P. A., Taylor, A. B., Hayward, L. J., Cabelli, D. E., Valentine, J. S., and Hart, P. J. (2003) An alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase: rates enhanced via proposed enzyme-associated peroxycarbonate intermediate, J. Biol. Chem. 278, 21032-21039.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 21032-21039
-
-
Elam, J.S.1
Malek, K.2
Rodriguez, J.A.3
Doucette, P.A.4
Taylor, A.B.5
Hayward, L.J.6
Cabelli, D.E.7
Valentine, J.S.8
Hart, P.J.9
-
38
-
-
0038442784
-
Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS
-
Elam, J. S., Taylor, A. B., Strange, R., Antonyuk, S., Doucette, P. A., Rodriguez, J. A., Hasnain, S. S., Hayward, L. J., Valentine, J. S., Yeates, T. O., and Hart, P. J. (2003) Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS, Nat. Struct. Biol. 10, 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
-
39
-
-
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, No. RC215.
-
(2002)
J. Neurosci.
, vol.22
, Issue.RC215
-
-
Higgins, C.M.1
Jung, C.2
Ding, H.3
Xu, Z.4
-
40
-
-
27244462026
-
Dynamic features of the subunit interface of Cu,Zn superoxide dismutase as probed by tryptophan phosphorescence
-
Cioni, P., Stroppolo, M. E., Desideri, A., and Strambini, G. B. (2001) Dynamic features of the subunit interface of Cu,Zn superoxide dismutase as probed by tryptophan phosphorescence, Arch. Biochem. Biophys. 391, 111-118.
-
(2001)
Arch. Biochem. Biophys.
, vol.391
, pp. 111-118
-
-
Cioni, P.1
Stroppolo, M.E.2
Desideri, A.3
Strambini, G.B.4
-
41
-
-
0034720474
-
Single mutation induces a metal-dependent subunit association in dimeric Cu,Zn superoxide dismutase
-
D'Orazio, M., Battistoni, A., Stroppolo, M. E., and Desideri, A. (2000) Single mutation induces a metal-dependent subunit association in dimeric Cu,Zn superoxide dismutase, Biochem. Biophys. Res. Commun. 272, 81-83.
-
(2000)
Biochem. Biophys. Res. Commun.
, vol.272
, pp. 81-83
-
-
D'Orazio, M.1
Battistoni, A.2
Stroppolo, M.E.3
Desideri, A.4
-
42
-
-
0032575482
-
On the coordination and oxidation states of the active-site copper ion in prokaryotic Cu,Zn superoxide dismutases
-
Stroppolo, M. E., Nuzzo, S., Pesce, A., Rosano, C., Battistoni, A., Bolognesi, M., Mobilio, S., and Desideri, A. (1998) On the coordination and oxidation states of the active-site copper ion in prokaryotic Cu,Zn superoxide dismutases, Biochem. Biophys. Res. Commun. 249, 579-582.
-
(1998)
Biochem. Biophys. Res. Commun.
, vol.249
, pp. 579-582
-
-
Stroppolo, M.E.1
Nuzzo, S.2
Pesce, A.3
Rosano, C.4
Battistoni, A.5
Bolognesi, M.6
Mobilio, S.7
Desideri, A.8
-
43
-
-
0034657260
-
Role of the tertiary and quaternary structures in the stability of dimeric copper, zinc superoxide dismutases
-
Stroppolo, M. E., Malvezzi-Campeggi, F., Mei, G., Rosato, N., and Desideri, A. (2000) Role of the tertiary and quaternary structures in the stability of dimeric copper, zinc superoxide dismutases, Arch. Biochem. Biophys. 377, 215-218.
-
(2000)
Arch. Biochem. Biophys.
, vol.377
, pp. 215-218
-
-
Stroppolo, M.E.1
Malvezzi-Campeggi, F.2
Mei, G.3
Rosato, N.4
Desideri, A.5
-
44
-
-
0017034938
-
Aggregation of deoxyhemoglobin subunits
-
McGovern, P., Reisberg, P., and Olson, J. S. (1976) Aggregation of deoxyhemoglobin subunits, J. Biol. Chem. 251, 7871-7879.
-
(1976)
J. Biol. Chem.
, vol.251
, pp. 7871-7879
-
-
McGovern, P.1
Reisberg, P.2
Olson, J.S.3
-
45
-
-
0022628340
-
Shy-Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons
-
Konno, H., Yamamoto, T., Iwasaki, Y., and Iizuka, H. (1986) Shy-Drager syndrome and amyotrophic lateral sclerosis. Cytoarchitectonic and morphometric studies of sacral autonomic neurons, J. Neurol. Sci. 73, 193-204.
-
(1986)
J. Neurol. Sci.
, vol.73
, pp. 193-204
-
-
Konno, H.1
Yamamoto, T.2
Iwasaki, Y.3
Iizuka, H.4
-
46
-
-
0030814099
-
Quaternary structure regulates hemin dissociation from human hemoglobin
-
Hargrove, M. S., Whitaker, T., Olson, J. S., Vali, R. J., and Mathews, A. J. (1997) Quaternary structure regulates hemin dissociation from human hemoglobin, J. Biol. Chem. 272, 17385-17389.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 17385-17389
-
-
Hargrove, M.S.1
Whitaker, T.2
Olson, J.S.3
Vali, R.J.4
Mathews, A.J.5
-
47
-
-
0024432915
-
Urea-induced inactivation, dissociation, and unfolding of the allosteric phosphofructokinase from Escherichia coli
-
Bras, G. L., Teschner, W., Deville-Bonne, D., and Garel, J. R. (1989) Urea-induced inactivation, dissociation, and unfolding of the allosteric phosphofructokinase from Escherichia coli, Biochemistry 28, 6836-6841.
-
(1989)
Biochemistry
, vol.28
, pp. 6836-6841
-
-
Bras, G.L.1
Teschner, W.2
Deville-Bonne, D.3
Garel, J.R.4
-
48
-
-
0033580635
-
Thermodynamic analysis of unfolding and dissociation in lactose repressor protein
-
Barry, J. K., and Matthews, K. S. (1999) Thermodynamic analysis of unfolding and dissociation in lactose repressor protein, Biochemistry 38, 6520-6528.
-
(1999)
Biochemistry
, vol.38
, pp. 6520-6528
-
-
Barry, J.K.1
Matthews, K.S.2
-
49
-
-
0030041283
-
Covalent tethering of the dimer interface annuls aggregation in thymidylate synthase
-
Agarwalla, S., Gokhale, R. S., Santi, D. V., and Balaram, P. (1996) Covalent tethering of the dimer interface annuls aggregation in thymidylate synthase, Protein Sci. 5, 270-277.
-
(1996)
Protein Sci.
, vol.5
, pp. 270-277
-
-
Agarwalla, S.1
Gokhale, R.S.2
Santi, D.V.3
Balaram, P.4
-
50
-
-
0029982719
-
Covalent reinforcement of a fragile region in the dimeric enzyme thymidylate synthase stabilizes the protein against chaotrope-induced unfolding
-
Gokhale, R. S., Agarwalla, S., Santi, D. V., and Balaram, P. (1996) Covalent reinforcement of a fragile region in the dimeric enzyme thymidylate synthase stabilizes the protein against chaotrope-induced unfolding, Biochemistry 35, 7150-7158.
-
(1996)
Biochemistry
, vol.35
, pp. 7150-7158
-
-
Gokhale, R.S.1
Agarwalla, S.2
Santi, D.V.3
Balaram, P.4
-
51
-
-
0036785613
-
Structure and dynamics of copper-free SOD: The protein before binding copper
-
Banci, L., Bertini, I., Cantini, F., D'Onofrio, M., and Viezzoli, M. S. (2002) Structure and dynamics of copper-free SOD: The protein before binding copper, Protein Sci. 11, 2479-2492.
-
(2002)
Protein Sci.
, vol.11
, pp. 2479-2492
-
-
Banci, L.1
Bertini, I.2
Cantini, F.3
D'Onofrio, M.4
Viezzoli, M.S.5
-
52
-
-
0036231449
-
The solution structure of reduced dimeric copper zinc superoxide dismutase. The structural effects of dimerization
-
Banci, L., Bertini, I., Cramaro, F., Del Conte, R., and Viezzoli, M. S. (2002) The solution structure of reduced dimeric copper zinc superoxide dismutase. The structural effects of dimerization, Eur. J. Biochem. 269, 1905-1915.
-
(2002)
Eur. J. Biochem.
, vol.269
, pp. 1905-1915
-
-
Banci, L.1
Bertini, I.2
Cramaro, F.3
Del Conte, R.4
Viezzoli, M.S.5
-
53
-
-
0034687667
-
Commitment to folded and aggregated states occurs late in interleukin-1 beta folding
-
Finke, J. M., Gross, L. A., Ho, H. M., Sept, D., Zimm, B. H., and Jennings, P. A. (2000) Commitment to folded and aggregated states occurs late in interleukin-1 beta folding, Biochemistry 39, 15633-15642.
-
(2000)
Biochemistry
, vol.39
, pp. 15633-15642
-
-
Finke, J.M.1
Gross, L.A.2
Ho, H.M.3
Sept, D.4
Zimm, B.H.5
Jennings, P.A.6
-
54
-
-
0031570336
-
Amyloid fibril formation and protein misassembly: A structural quest for insights into amyloid and prion diseases
-
Kelly, J. W. (1997) Amyloid fibril formation and protein misassembly: a structural quest for insights into amyloid and prion diseases, Structure 5, 595-600.
-
(1997)
Structure
, vol.5
, pp. 595-600
-
-
Kelly, J.W.1
-
55
-
-
0030830019
-
Transthyretin quaternary and tertiary structural changes facilitate misassembly into amyloid
-
Kelly, J. W., Colon, W., Lai, Z., Lashuel, H. A., McCulloch, J., McCutchen, S. L., Miroy, G. J., and Peterson, S. A. (1997) Transthyretin quaternary and tertiary structural changes facilitate misassembly into amyloid, Adv. Protein Chem. 50, 161-181.
-
(1997)
Adv. Protein Chem.
, vol.50
, pp. 161-181
-
-
Kelly, J.W.1
Colon, W.2
Lai, Z.3
Lashuel, H.A.4
McCulloch, J.5
McCutchen, S.L.6
Miroy, G.J.7
Peterson, S.A.8
-
56
-
-
0034919395
-
Transthyretin slowly exchanges subunits under physiological conditions: A convenient chromatographic method to study subunit exchange in oligomeric proteins
-
Schneider, F., Hammarstrom, P., and Kelly, J. W. (2001) Transthyretin slowly exchanges subunits under physiological conditions: A convenient chromatographic method to study subunit exchange in oligomeric proteins, Protein Sci. 10, 1606-1613.
-
(2001)
Protein Sci.
, vol.10
, pp. 1606-1613
-
-
Schneider, F.1
Hammarstrom, P.2
Kelly, J.W.3
-
57
-
-
0031684499
-
Discovering transthyretin amyloid fibril inhibitors by limited screening
-
Baures, P. W., Peterson, S. A., and Kelly, J. W. (1998) Discovering transthyretin amyloid fibril inhibitors by limited screening, Bioorg. Med. Chem. 6, 1389-1401.
-
(1998)
Bioorg. Med. Chem.
, vol.6
, pp. 1389-1401
-
-
Baures, P.W.1
Peterson, S.A.2
Kelly, J.W.3
-
58
-
-
0030447882
-
Inhibiting transthyretin amyloid fibril formation via protein stabilization
-
Miroy, G. J., Lai, Z., Lashuel, H. A., Peterson, S. A., Strang, C., and Kelly, J. W. (1996) Inhibiting transthyretin amyloid fibril formation via protein stabilization, Proc. Natl. Acad. Sci. U.S.A. 93, 15051-15056.
-
(1996)
Proc. Natl. Acad. Sci. U.S.A.
, vol.93
, pp. 15051-15056
-
-
Miroy, G.J.1
Lai, Z.2
Lashuel, H.A.3
Peterson, S.A.4
Strang, C.5
Kelly, J.W.6
-
59
-
-
0035949632
-
Anion shielding of electrostatic repulsions in transthyretin modulates stability and amyloidosis: Insight into the chaotrope unfolding dichotomy
-
Hammarstrom, P., Jiang, X., Deechongkit, S., and Kelly, J. W. (2001) Anion shielding of electrostatic repulsions in transthyretin modulates stability and amyloidosis: insight into the chaotrope unfolding dichotomy, Biochemistry 40, 11453-11459.
-
(2001)
Biochemistry
, vol.40
, pp. 11453-11459
-
-
Hammarstrom, P.1
Jiang, X.2
Deechongkit, S.3
Kelly, J.W.4
-
60
-
-
0035964955
-
Transsuppression of misfolding in an amyloid disease
-
Hammarstrom, P., Schneider, F., and Kelly, J. W. (2001) Transsuppression of misfolding in an amyloid disease, Science 293, 2459-2462.
-
(2001)
Science
, vol.293
, pp. 2459-2462
-
-
Hammarstrom, P.1
Schneider, F.2
Kelly, J.W.3
-
61
-
-
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., and 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
-
62
-
-
0042736853
-
ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization
-
DiDonato, M., Craig, L., Huff, M. E., Thayer, M. M., Cardoso, R. M., Kassmann, C. J., Lo, T. P., Bruns, C. K., Powers, E. T., Kelly, J. W., Getzoff, E. D., and Tainer, J. A. (2003) ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization, J. Mol. Biol. 332, 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
-
63
-
-
0033524469
-
Unfolding of Plasmodium falciparum triosephosphate isomerase in urea and guanidinium chloride: Evidence for a novel disulfide exchange reaction in a covalently cross-linked mutant
-
Gokhale, R. S., Ray, S. S., Balaram, H., and Balaram, P. (1999) Unfolding of Plasmodium falciparum triosephosphate isomerase in urea and guanidinium chloride: evidence for a novel disulfide exchange reaction in a covalently cross-linked mutant, Biochemistry 38, 423-431.
-
(1999)
Biochemistry
, vol.38
, pp. 423-431
-
-
Gokhale, R.S.1
Ray, S.S.2
Balaram, H.3
Balaram, P.4
-
64
-
-
0033524490
-
Cavity-creating mutation at the dimer interface of Plasmodium falciparum triosephosphate isomerase: Restoration of stability by disulfide cross-linking of subunits
-
Gopal, B., Ray, S. S., Gokhale, R. S., Balaram, H., Murthy, M. R., and Balaram, P. (1999) Cavity-creating mutation at the dimer interface of Plasmodium falciparum triosephosphate isomerase: restoration of stability by disulfide cross-linking of subunits, Biochemistry 38, 478-486.
-
(1999)
Biochemistry
, vol.38
, pp. 478-486
-
-
Gopal, B.1
Ray, S.S.2
Gokhale, R.S.3
Balaram, H.4
Murthy, M.R.5
Balaram, P.6
-
65
-
-
0032891249
-
Disulfide engineering at the dimer interface of Lactobacillus casei thymidylate synthase: Crystal structure of the T155C/E188C/C244T mutant
-
Velanker, S. S., Gokhale, R. S., Ray, S. S., Gopal, B., Parthasarathy, S., Santi, D. V., Balaram, P., and Murthy, M. R. (1999) Disulfide engineering at the dimer interface of Lactobacillus casei thymidylate synthase: crystal structure of the T155C/E188C/C244T mutant, Protein Sci. 8, 930-933.
-
(1999)
Protein Sci.
, vol.8
, pp. 930-933
-
-
Velanker, S.S.1
Gokhale, R.S.2
Ray, S.S.3
Gopal, B.4
Parthasarathy, S.5
Santi, D.V.6
Balaram, P.7
Murthy, M.R.8
-
66
-
-
0028064007
-
Thermal stabilization of thymidylate synthase by engineering two disulfide bridges across the dimer interface
-
Gokhale, R. S., Agarwalla, S., Francis, V. S., Santi, D. V., and Balaram, P. (1994) Thermal stabilization of thymidylate synthase by engineering two disulfide bridges across the dimer interface, J. Mol. Biol. 235, 89-94.
-
(1994)
J. Mol. Biol.
, vol.235
, pp. 89-94
-
-
Gokhale, R.S.1
Agarwalla, S.2
Francis, V.S.3
Santi, D.V.4
Balaram, P.5
-
67
-
-
0027052444
-
Structure of a stabilizing disulfide bridge mutant that closes the active-site cleft of T4 lysozyme
-
Jacobson, R. H., Matsumura, M., Faber, H. R., and Matthews, B. W. (1992) Structure of a stabilizing disulfide bridge mutant that closes the active-site cleft of T4 lysozyme, Protein Sci. 1, 46-57.
-
(1992)
Protein Sci.
, vol.1
, pp. 46-57
-
-
Jacobson, R.H.1
Matsumura, M.2
Faber, H.R.3
Matthews, B.W.4
-
68
-
-
0025319751
-
Structure of a thermostable disulfide-bridge mutant of phage T4 lysozyme shows that an engineered cross-link in a flexible region does not increase the rigidity of the folded protein
-
Pjura, P. E., Matsumura, M., Wozniak, J. A., and Matthews, B. W. (1990) Structure of a thermostable disulfide-bridge mutant of phage T4 lysozyme shows that an engineered cross-link in a flexible region does not increase the rigidity of the folded protein, Biochemistry 29, 2592-2598.
-
(1990)
Biochemistry
, vol.29
, pp. 2592-2598
-
-
Pjura, P.E.1
Matsumura, M.2
Wozniak, J.A.3
Matthews, B.W.4
-
69
-
-
0026319603
-
Stabilization of functional proteins by introduction of multiple disulfide bonds
-
Matsumura, M., and Matthews, B. W. (1991) Stabilization of functional proteins by introduction of multiple disulfide bonds, Methods Enzymol. 202, 336-356.
-
(1991)
Methods Enzymol.
, vol.202
, pp. 336-356
-
-
Matsumura, M.1
Matthews, B.W.2
-
70
-
-
0031012304
-
Mutations in SOD1 associated with amyotrophic lateral sclerosis cause novel protein interactions
-
Kunst, C. B., Mezey, E., Brownstein, M. J., and Patterson, D. (1997) Mutations in SOD1 associated with amyotrophic lateral sclerosis cause novel protein interactions, Nat. Genet. 15, 91-94.
-
(1997)
Nat. Genet.
, vol.15
, pp. 91-94
-
-
Kunst, C.B.1
Mezey, E.2
Brownstein, M.J.3
Patterson, D.4
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