-
1
-
-
33750947173
-
Retinitis pigmentosa
-
Hartong, D.T., Berson, E.L. and Dryja, T.P. (2006) Retinitis pigmentosa. Lancet, 368, 1795-1809.
-
(2006)
Lancet
, vol.368
, pp. 1795-1809
-
-
Hartong, D.T.1
Berson, E.L.2
Dryja, T.P.3
-
2
-
-
34347246364
-
RNA interference-mediated suppression and replacement of human rhodopsin in vivo
-
O'Reilly, M., Palfi, A., Chadderton, N., Millington-Ward, S., Ader, M., Cronin, T., Tuohy, T., Auricchio, A., Hildinger, M., Tivnan, A. et al. (2007) RNA interference-mediated suppression and replacement of human rhodopsin in vivo. Am. J. Hum. Genet., 81, 127-135.
-
(2007)
Am. J. Hum. Genet.
, vol.81
, pp. 127-135
-
-
O'Reilly, M.1
Palfi, A.2
Chadderton, N.3
Millington-Ward, S.4
Ader, M.5
Cronin, T.6
Tuohy, T.7
Auricchio, A.8
Hildinger, M.9
Tivnan, A.10
-
3
-
-
46249091156
-
Therapeutic benefit derived from RNAi-mediated ablation of IMPDH1 transcripts in a murine model of autosomal dominant retinitis pigmentosa (RP10)
-
Tam, L.C., Kiang, A.S., Kennan, A., Kenna, P.F., Chadderton, N., Ader, M., Palfi, A., Aherne, A., Ayuso, C., Campbell, M., Reynolds, A. et al. (2008) Therapeutic benefit derived from RNAi-mediated ablation of IMPDH1 transcripts in a murine model of autosomal dominant retinitis pigmentosa (RP10). Hum. Mol. Genet., 17, 2084-2100.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 2084-2100
-
-
Tam, L.C.1
Kiang, A.S.2
Kennan, A.3
Kenna, P.F.4
Chadderton, N.5
Ader, M.6
Palfi, A.7
Aherne, A.8
Ayuso, C.9
Campbell, M.10
Reynolds, A.11
-
4
-
-
44249120315
-
Effect of gene therapy on visual function in Leber's congenital amaurosis
-
Bainbridge, J.W., Smith, A.J., Barker, S.S., Robbie, S., Henderson, R., Balaggan, K., Viswanathan, A., Holder, G.E., Stockman, A., Tyler, N. et al. (2008) Effect of gene therapy on visual function in Leber's congenital amaurosis. N. Engl. J. Med., 358, 2231-2239.
-
(2008)
N. Engl. J. Med.
, vol.358
, pp. 2231-2239
-
-
Bainbridge, J.W.1
Smith, A.J.2
Barker, S.S.3
Robbie, S.4
Henderson, R.5
Balaggan, K.6
Viswanathan, A.7
Holder, G.E.8
Stockman, A.9
Tyler, N.10
-
5
-
-
70349105559
-
Human RPE65 gene therapy for Leber congenital amaurosis: persistence of early visual improvements and safety at 1 year
-
Cideciyan, A.V., Hauswirth, W.W., Aleman, T.S., Kaushal, S., Schwartz, S.B., Boye, S.L., Windsor, E.A., Conlon, T.J., Sumaroka, A., Pang, J.J. et al. (2009) Human RPE65 gene therapy for Leber congenital amaurosis: persistence of early visual improvements and safety at 1 year. Hum. Gene Ther., 20, 999-1004.
-
(2009)
Hum. Gene Ther.
, vol.20
, pp. 999-1004
-
-
Cideciyan, A.V.1
Hauswirth, W.W.2
Aleman, T.S.3
Kaushal, S.4
Schwartz, S.B.5
Boye, S.L.6
Windsor, E.A.7
Conlon, T.J.8
Sumaroka, A.9
Pang, J.J.10
-
6
-
-
70350620424
-
Age-dependent effects of RPE65 gene therapy for Leber's congenital amaurosis: a phase 1 dose-escalation trial
-
Maguire, A.M., High, K.A., Auricchio, A., Wright, J.F., Pierce, E.A., Testa, F., Mingozzi, F., Bennicelli, J.L., Ying, G.S., Rossi, S. et al. (2009) Age-dependent effects of RPE65 gene therapy for Leber's congenital amaurosis: a phase 1 dose-escalation trial. Lancet, 374, 1597-1605.
-
(2009)
Lancet
, vol.374
, pp. 1597-1605
-
-
Maguire, A.M.1
High, K.A.2
Auricchio, A.3
Wright, J.F.4
Pierce, E.A.5
Testa, F.6
Mingozzi, F.7
Bennicelli, J.L.8
Ying, G.S.9
Rossi, S.10
-
7
-
-
0025105161
-
A point mutation of the rhodopsin gene in one form of retinitis pigmentosa
-
Dryja, T.P., McGee, T.L., Reichel, E., Hahn, L.B., Cowley, G.S., Yandell, D.W., Sandberg, M.A. and Berson, E.L. (1990) A point mutation of the rhodopsin gene in one form of retinitis pigmentosa. Nature, 343, 364-366.
-
(1990)
Nature
, vol.343
, pp. 364-366
-
-
Dryja, T.P.1
McGee, T.L.2
Reichel, E.3
Hahn, L.B.4
Cowley, G.S.5
Yandell, D.W.6
Sandberg, M.A.7
Berson, E.L.8
-
8
-
-
0025721075
-
A three-base-pair deletion in the peripherin-RDS gene in one form of retinitis pigmentosa
-
Farrar, G.J., Kenna, P., Jordan, S.A., Kumar-Singh, R., Humphries, M.M., Sharp, E.M., Sheils, D.M. and Humphries, P. (1991) A three-base-pair deletion in the peripherin-RDS gene in one form of retinitis pigmentosa. Nature, 354, 478-480.
-
(1991)
Nature
, vol.354
, pp. 478-480
-
-
Farrar, G.J.1
Kenna, P.2
Jordan, S.A.3
Kumar-Singh, R.4
Humphries, M.M.5
Sharp, E.M.6
Sheils, D.M.7
Humphries, P.8
-
9
-
-
0036501462
-
Identification of an IMPDH1 mutation in autosomal dominant retinitis pigmentosa (RP10) revealed following comparative microarray analysis of transcripts derived from retinas of wild-type and Rho(2/2) mice
-
Kennan, A., Aherne, A., Palfi, A., Humphries, M.M., McKee, A., Stitt, A., Simpson, D.A., Demtroder, K., Orntoft, T., Ayuso, C. et al. (2002) Identification of an IMPDH1 mutation in autosomal dominant retinitis pigmentosa (RP10) revealed following comparative microarray analysis of transcripts derived from retinas of wild-type and Rho(2/2) mice. Hum. Mol. Genet., 11, 547-557.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 547-557
-
-
Kennan, A.1
Aherne, A.2
Palfi, A.3
Humphries, M.M.4
McKee, A.5
Stitt, A.6
Simpson, D.A.7
Demtroder, K.8
Orntoft, T.9
Ayuso, C.10
-
10
-
-
0036501591
-
Mutations in the inosine monophosphate dehydrogenase 1 gene (IMPDH1) cause the RP10 form of autosomal dominant retinitis pigmentosa
-
Bowne, S.J., Sullivan, L.S., Blanton, S.H., Cepko, C.L., Blackshaw, S., Birch, D.G., Hughbanks-Wheaton, D., Heckenlively, J.R. and Daiger, S.P. (2002) Mutations in the inosine monophosphate dehydrogenase 1 gene (IMPDH1) cause the RP10 form of autosomal dominant retinitis pigmentosa. Hum. Mol. Genet., 11, 559-568.
-
(2002)
Hum. Mol. Genet.
, vol.11
, pp. 559-568
-
-
Bowne, S.J.1
Sullivan, L.S.2
Blanton, S.H.3
Cepko, C.L.4
Blackshaw, S.5
Birch, D.G.6
Hughbanks-Wheaton, D.7
Heckenlively, J.R.8
Daiger, S.P.9
-
11
-
-
17044363529
-
Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy
-
Mendes, H.F., van der Spuy, J., Chapple, J.P. and Cheetham, M.E. (2005) Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy. Trends Mol. Med., 11, 177-185.
-
(2005)
Trends Mol. Med.
, vol.11
, pp. 177-185
-
-
Mendes, H.F.1
van der Spuy, J.2
Chapple, J.P.3
Cheetham, M.E.4
-
12
-
-
0037072934
-
A rhodopsin mutant linked to autosomal dominant RP is prone to aggregate and interacts with the ubiquitin proteasome system
-
Illing, M.E., Rajan, R.S., Bence, N.F. and Kopito, R.R. (2002) A rhodopsin mutant linked to autosomal dominant RP is prone to aggregate and interacts with the ubiquitin proteasome system. J. Biol. Chem., 277, 34150-34160.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 34150-34160
-
-
Illing, M.E.1
Rajan, R.S.2
Bence, N.F.3
Kopito, R.R.4
-
13
-
-
0037099080
-
The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation
-
Saliba, R.S., Munro, P.M., Luthert, P.J. and Cheetham, M.E. (2002) The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation. J. Cell Sci., 115, 2907-2918.
-
(2002)
J. Cell Sci.
, vol.115
, pp. 2907-2918
-
-
Saliba, R.S.1
Munro, P.M.2
Luthert, P.J.3
Cheetham, M.E.4
-
14
-
-
75749112504
-
Biochemical analysis of phenotypic diversity associated with mutations in codon 244 of the retinal degeneration slow gene
-
Conley, S.M., Stricker, H.M. and Naash, M.I. (2010) Biochemical analysis of phenotypic diversity associated with mutations in codon 244 of the retinal degeneration slow gene. Biochemistry, 49, 905-911.
-
(2010)
Biochemistry
, vol.49
, pp. 905-911
-
-
Conley, S.M.1
Stricker, H.M.2
Naash, M.I.3
-
15
-
-
12144287403
-
On the molecular pathology of neurodegeneration in IMPDH1-based retinitis pigmentosa
-
Aherne, A., Kennan, A., Kenna, P.F., McNally, N., Lloyd, D.G., Alberts, I.L., Kiang, A.S., Humphries, M.M., Ayuso, C., Engel, P.C. et al. (2004) On the molecular pathology of neurodegeneration in IMPDH1-based retinitis pigmentosa. Hum. Mol. Genet., 13, 641-650.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 641-650
-
-
Aherne, A.1
Kennan, A.2
Kenna, P.F.3
McNally, N.4
Lloyd, D.G.5
Alberts, I.L.6
Kiang, A.S.7
Humphries, M.M.8
Ayuso, C.9
Engel, P.C.10
-
16
-
-
0026463972
-
Transgenic mice with a rhodopsin mutation (Pro23His): a mouse model of autosomal dominant retinitis pigmentosa
-
Olsson, J.E., Gordon, J.W., Pawlyk, B.S., Roof, D., Hayes, A., Molday, R.S., Mukai, S., Cowley, G.S., Berson, E.L. and Dryja, T.P. (1992) Transgenic mice with a rhodopsin mutation (Pro23His): a mouse model of autosomal dominant retinitis pigmentosa. Neuron, 9, 815-830.
-
(1992)
Neuron
, vol.9
, pp. 815-830
-
-
Olsson, J.E.1
Gordon, J.W.2
Pawlyk, B.S.3
Roof, D.4
Hayes, A.5
Molday, R.S.6
Mukai, S.7
Cowley, G.S.8
Berson, E.L.9
Dryja, T.P.10
-
17
-
-
48449096008
-
Molecular chaperones and photoreceptor function
-
Kosmaoglou, M., Schwarz, N., Bett, J.S. and Cheetham, M.E. (2008) Molecular chaperones and photoreceptor function. Prog. Retin. Eye Res., 27, 434-449.
-
(2008)
Prog. Retin. Eye Res.
, vol.27
, pp. 434-449
-
-
Kosmaoglou, M.1
Schwarz, N.2
Bett, J.S.3
Cheetham, M.E.4
-
18
-
-
12144282296
-
Genetic disorders involving molecular-chaperone genes: a perspective
-
Macario, A.J., Grippo, T.M. and de Macario, E.C. (2005) Genetic disorders involving molecular-chaperone genes: a perspective. Genet. Med., 7, 3-12.
-
(2005)
Genet. Med.
, vol.7
, pp. 3-12
-
-
Macario, A.J.1
Grippo, T.M.2
de Macario, E.C.3
-
19
-
-
30044446463
-
A crucial role of mitochondria Hsp40 in preventing dilated cardiomyopathy
-
Hayashi, M., Imanaka-Yoshida, T., Wood, M., Fearns, C., Tatake, R.J. and Lee, J.D. (2006) A crucial role of mitochondria Hsp40 in preventing dilated cardiomyopathy. Nat. Med., 12, 128-132.
-
(2006)
Nat. Med.
, vol.12
, pp. 128-132
-
-
Hayashi, M.1
Imanaka-Yoshida, T.2
Wood, M.3
Fearns, C.4
Tatake, R.J.5
Lee, J.D.6
-
20
-
-
0042825251
-
Heat shock protein 27 gene: chromosomal and molecular location and relationship to Williams syndrome
-
Stock, A.D., Spallone, P.A., Dennis, T.R., Netski, D., Morris, C.A., Mervis, C.B. and Hobart, H.H. (2003) Heat shock protein 27 gene: chromosomal and molecular location and relationship to Williams syndrome. Am. J. Med. Genet. A., 120A, 320-325.
-
(2003)
Am. J. Med. Genet. A.
, vol.120 A
, pp. 320-325
-
-
Stock, A.D.1
Spallone, P.A.2
Dennis, T.R.3
Netski, D.4
Morris, C.A.5
Mervis, C.B.6
Hobart, H.H.7
-
21
-
-
48249119911
-
The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex
-
Hidalgo-de-Quintana, J., Evans, R.J., Cheetham, M.E. and van der Spuy, J. (2008) The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex. Invest. Ophthalmol. Vis. Sci., 49, 2878-2887.
-
(2008)
Invest. Ophthalmol. Vis. Sci.
, vol.49
, pp. 2878-2887
-
-
Hidalgo-de-Quintana, J.1
Evans, R.J.2
Cheetham, M.E.3
van der Spuy, J.4
-
22
-
-
0027135501
-
The function of heat-shock proteins in stress tolerance: degradation and reactivation of damaged proteins
-
Parsell, D.A. and Lindquist, S. (1993) The function of heat-shock proteins in stress tolerance: degradation and reactivation of damaged proteins. Annu. Rev. Genet., 27, 437-96.
-
(1993)
Annu. Rev. Genet.
, vol.27
, pp. 437-496
-
-
Parsell, D.A.1
Lindquist, S.2
-
23
-
-
0037315208
-
Regulation of signaling protein function and trafficking by the hsp90/hsp70-based chaperone machinery
-
Pratt, W.B. and Toft, D.O. (2003) Regulation of signaling protein function and trafficking by the hsp90/hsp70-based chaperone machinery. Exp. Biol. Med., 228, 111-133.
-
(2003)
Exp. Biol. Med.
, vol.228
, pp. 111-133
-
-
Pratt, W.B.1
Toft, D.O.2
-
24
-
-
17044387386
-
Hsp70 chaperones: cellular functions and molecular mechanism
-
Mayer, M.P. and Bukau, B. (2005) Hsp70 chaperones: cellular functions and molecular mechanism. Cell. Mol. Life Sci., 62, 670-684.
-
(2005)
Cell. Mol. Life Sci.
, vol.62
, pp. 670-684
-
-
Mayer, M.P.1
Bukau, B.2
-
25
-
-
5044239107
-
Pathways of chaperone-mediated protein folding in the cytosol
-
Young, J.C., Agashe, V.R., Seigers, K. and Hartl, F.U. (2004) Pathways of chaperone-mediated protein folding in the cytosol. Nat. Rev. Mol. Cell Biol., 5, 781-791.
-
(2004)
Nat. Rev. Mol. Cell Biol.
, vol.5
, pp. 781-791
-
-
Young, J.C.1
Agashe, V.R.2
Seigers, K.3
Hartl, F.U.4
-
26
-
-
41649083178
-
MKKS is a centrosome-shuttling protein degraded by disease-causing mutations via CHIP-mediated ubiquitination
-
Hirayama, S., Yamazaki, Y., Kitamura, A., Oda, Y., Morito, D., Okawa, K., Kimura, H., Cyr, D.M., Kubota, H. and Nagata, K. (2008) MKKS is a centrosome-shuttling protein degraded by disease-causing mutations via CHIP-mediated ubiquitination. Mol. Biol. Cell, 19, 899-911.
-
(2008)
Mol. Biol. Cell
, vol.19
, pp. 899-911
-
-
Hirayama, S.1
Yamazaki, Y.2
Kitamura, A.3
Oda, Y.4
Morito, D.5
Okawa, K.6
Kimura, H.7
Cyr, D.M.8
Kubota, H.9
Nagata, K.10
-
27
-
-
0035363805
-
Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease
-
Sittler, A., Lurz, R., Lueder, G., Priller, J., Lehrach, H., Hayer-Hartl, M.K., Hartl, F.U. and Wanker, E.E. (2001) Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Hum. Mol. Genet., 10, 1307-1315.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1307-1315
-
-
Sittler, A.1
Lurz, R.2
Lueder, G.3
Priller, J.4
Lehrach, H.5
Hayer-Hartl, M.K.6
Hartl, F.U.7
Wanker, E.E.8
-
28
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
-
Warrick, J.M., Chan, H.Y., Gray-Board, G.L., Chai, Y., Paulson, H.L. and Bonini, N.M. (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet., 23, 425-428.
-
(1999)
Nat. Genet.
, vol.23
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
29
-
-
0034703863
-
Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila
-
Chan, H.Y., Warrick, J.M., Gray-Board, G.L., Paulson, H.L. and Bonini, N.M. (2000) Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Hum. Mol. Genet., 9, 2811-2820.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 2811-2820
-
-
Chan, H.Y.1
Warrick, J.M.2
Gray-Board, G.L.3
Paulson, H.L.4
Bonini, N.M.5
-
30
-
-
77950532428
-
Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78
-
Gorbatyuk, M.S., Knox, T., LaVail, M.M., Gorbatyuk, O.S., Noorwez, S.M., Hauswirth, W.W., Lin, J.H., Muzyczka, N. and Lewin, A.S. (2010) Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78. Proc. Natl Acad. Sci. USA, 107, 5961-5966.
-
(2010)
Proc. Natl Acad. Sci. USA
, vol.107
, pp. 5961-5966
-
-
Gorbatyuk, M.S.1
Knox, T.2
LaVail, M.M.3
Gorbatyuk, O.S.4
Noorwez, S.M.5
Hauswirth, W.W.6
Lin, J.H.7
Muzyczka, N.8
Lewin, A.S.9
-
31
-
-
70149093873
-
Small-molecule modulation of cellular chaperones to treat protein misfolding disorders
-
Sloan, L.A., Fillmore, M.C. and Churcher, I. (2009) Small-molecule modulation of cellular chaperones to treat protein misfolding disorders. Curr. Opin. Drug Discov. Dev., 12, 666-681.
-
(2009)
Curr. Opin. Drug Discov. Dev.
, vol.12
, pp. 666-681
-
-
Sloan, L.A.1
Fillmore, M.C.2
Churcher, I.3
-
32
-
-
0027467764
-
Heat shock protein (hsx70) mRNA expression in human brain: effects of neurodegenerative disease and agonal state
-
Harrison, P.J., Procter, A.W., Exworthy, T., Roberts, G.W., Najlerahim, A., Barton, A.J. and Pearson, R.C. (1993) Heat shock protein (hsx70) mRNA expression in human brain: effects of neurodegenerative disease and agonal state. Neuropathol. Appl. Neurobiol., 19, 10-21.
-
(1993)
Neuropathol. Appl. Neurobiol.
, vol.19
, pp. 10-21
-
-
Harrison, P.J.1
Procter, A.W.2
Exworthy, T.3
Roberts, G.W.4
Najlerahim, A.5
Barton, A.J.6
Pearson, R.C.7
-
33
-
-
77249121048
-
Geldanamycin and its anti-cancer activities
-
Fukuyo, Y., Hunt, C.R. and Horikoshi, N. (2010) Geldanamycin and its anti-cancer activities. Cancer Lett., 290, 24-35.
-
(2010)
Cancer Lett
, vol.290
, pp. 24-35
-
-
Fukuyo, Y.1
Hunt, C.R.2
Horikoshi, N.3
-
34
-
-
0032555685
-
Repression of heat shock transcription factor HSF1 activation by HSP90 (HSP90 complex) that forms a stress-sensitive complex with HSF1
-
Zou, J., Guo, Y., Guettouche, T., Smith, D.F. and Voellmy, R. (1998) Repression of heat shock transcription factor HSF1 activation by HSP90 (HSP90 complex) that forms a stress-sensitive complex with HSF1. Cell, 94, 471-480.
-
(1998)
Cell
, vol.94
, pp. 471-480
-
-
Zou, J.1
Guo, Y.2
Guettouche, T.3
Smith, D.F.4
Voellmy, R.5
-
35
-
-
0034743361
-
Plasma pharmacokinetics and tissue distribution of 17-(allylamino)-17-demethoxygeldanamycin (NSC 330507) in CD2F1 mice1
-
Egorin, M.J., Zuhowski, E.G., Rosen, D.M., Sentz, D.L., Covey, J.M. and Eiseman, J.L. (2001) Plasma pharmacokinetics and tissue distribution of 17-(allylamino)-17-demethoxygeldanamycin (NSC 330507) in CD2F1 mice1. Cancer Chemother. Pharmacol., 47, 291-302.
-
(2001)
Cancer Chemother. Pharmacol.
, vol.47
, pp. 291-302
-
-
Egorin, M.J.1
Zuhowski, E.G.2
Rosen, D.M.3
Sentz, D.L.4
Covey, J.M.5
Eiseman, J.L.6
-
36
-
-
0141596326
-
Clinical development of 17-allylamino 17-demethoxygeldanamycin
-
Sausville, E.A., Tomaszewski, J.E. and Ivy, P. (2003) Clinical development of 17-allylamino, 17-demethoxygeldanamycin. Curr. Cancer Drug Targets, 3, 377-383.
-
(2003)
Curr. Cancer Drug Targets
, vol.3
, pp. 377-383
-
-
Sausville, E.A.1
Tomaszewski, J.E.2
Ivy, P.3
-
37
-
-
54449101793
-
Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones
-
Fujikake, N., Nagai, Y., Popiel, H.A., Okamoto, Y., Yamaguchi, M. and Toda, T. (2008) Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones. J. Biol. Chem., 283, 26188-26189.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 26188-26189
-
-
Fujikake, N.1
Nagai, Y.2
Popiel, H.A.3
Okamoto, Y.4
Yamaguchi, M.5
Toda, T.6
-
38
-
-
27144503120
-
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration
-
Waza, M., Adachi, H., Katsuno, M., Minamiyama, M., Sang, C., Tanaka, F., Inukai, A., Doyu, M. and Sobue, G. (2005) 17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat. Med., 11, 1088-1095.
-
(2005)
Nat. Med.
, vol.11
, pp. 1088-1095
-
-
Waza, M.1
Adachi, H.2
Katsuno, M.3
Minamiyama, M.4
Sang, C.5
Tanaka, F.6
Inukai, A.7
Doyu, M.8
Sobue, G.9
-
39
-
-
52949134162
-
Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa
-
Mendes, H.F. and Cheetham, M.E. (2008) Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa. Hum. Mol. Genet., 17, 3043-3054.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 3043-3054
-
-
Mendes, H.F.1
Cheetham, M.E.2
-
40
-
-
33746191768
-
Inhibitors of the HSP90 molecular chaperone: current status
-
Sharp, S. and Workman, P. (2006) Inhibitors of the HSP90 molecular chaperone: current status. Adv. Cancer Res., 95, 323-348.
-
(2006)
Adv. Cancer Res.
, vol.95
, pp. 323-348
-
-
Sharp, S.1
Workman, P.2
-
41
-
-
33947517558
-
AGGRESCAN: a server for the prediction and evaluation of "hot spots" of aggregation in polypeptides
-
Conchillo-Solé, O., de Groot, N.S., Avilés, F.X., Vendrell, J., Daura, X. and Ventura, S. (2007) AGGRESCAN: a server for the prediction and evaluation of "hot spots" of aggregation in polypeptides. BMC Bioinformatics, 8, 65.
-
(2007)
BMC Bioinformatics
, vol.8
, pp. 65
-
-
Conchillo-Solé, O.1
de Groot, N.S.2
Avilés, F.X.3
Vendrell, J.4
Daura, X.5
Ventura, S.6
-
42
-
-
70349847872
-
Fast and accurate predictions of protein stability changes upon mutations using statistical potentials and neural networks: PoPMuSiC-2 0
-
Dehouck, Y., Grosfils, A., Folch, B., Gilis, D., Bogaerts, P. and Rooman, M. (2009) Fast and accurate predictions of protein stability changes upon mutations using statistical potentials and neural networks: PoPMuSiC-2.0. Bioinformatics, 25, 2537-2543.
-
(2009)
Bioinformatics
, vol.25
, pp. 2537-2543
-
-
Dehouck, Y.1
Grosfils, A.2
Folch, B.3
Gilis, D.4
Bogaerts, P.5
Rooman, M.6
-
43
-
-
0027973061
-
CPP32, a novel human apoptotic protein with homology to Caenorhabditis elegans cell death protein Ced-3 and mammalian interleukin-1 beta-converting enzyme
-
Fernandes-Alnemri, T., Litwack, G. and Alnemri, E.S. (1994) CPP32, a novel human apoptotic protein with homology to Caenorhabditis elegans cell death protein Ced-3 and mammalian interleukin-1 beta-converting enzyme. J. Biol. Chem., 269, 30761-30764.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 30761-30764
-
-
Fernandes-Alnemri, T.1
Litwack, G.2
Alnemri, E.S.3
-
44
-
-
0029068871
-
Identification and inhibition of the ICE/CED-3 protease necessary for mammalian apoptosis
-
Nicholson, D.W., Ali, A., Thornberry, N.A., Vaillancourt, J.P., Ding, C.K., Gallant, M., Gareau, Y., Griffin, P.R., Labelle, M., Lazebnik, Y.A. et al. (1995) Identification and inhibition of the ICE/CED-3 protease necessary for mammalian apoptosis. Nature, 376, 37-43.
-
(1995)
Nature
, vol.376
, pp. 37-43
-
-
Nicholson, D.W.1
Ali, A.2
Thornberry, N.A.3
Vaillancourt, J.P.4
Ding, C.K.5
Gallant, M.6
Gareau, Y.7
Griffin, P.R.8
Labelle, M.9
Lazebnik, Y.A.10
-
45
-
-
33847369469
-
The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins
-
Dickey, C.A., Kamal, A., Lundgren, K., Klosak, N., Bailey, R.M., Dunmore, J., Ash, P., Shoraka, S., Zlatkovic, J., Eckman, C.B. et al. (2007) The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins. J. Clin. Invest., 117, 648-658.
-
(2007)
J. Clin. Invest.
, vol.117
, pp. 648-658
-
-
Dickey, C.A.1
Kamal, A.2
Lundgren, K.3
Klosak, N.4
Bailey, R.M.5
Dunmore, J.6
Ash, P.7
Shoraka, S.8
Zlatkovic, J.9
Eckman, C.B.10
-
46
-
-
70449577160
-
An experimental platform for systemic drug delivery to the retina
-
Campbell, M., Nguyen, A.T., Kiang, A.S., Tam, L.C., Gobbo, O.L., Kerskens, C., Ni Dhubhghaill, S., Humphries, M.M., Farrar, G.J., Kenna
-
(2009)
Proc. Natl Acad. Sci. USA
, vol.106
, pp. 17817-17822
-
-
Campbell, M.1
Nguyen, A.T.2
Kiang, A.S.3
Tam, L.C.4
Gobbo, O.L.5
Kerskens, C.6
Ni Dhubhghaill, S.7
Humphries, M.M.8
Farrar, G.J.9
Kenna, P.F.10
Humphries, P.11
-
47
-
-
49349115993
-
RNAi-mediated reversible opening of the blood-brain barrier
-
P.F. and Humphries, P. (2009) An experimental platform for systemic drug delivery to the retina. Proc. Natl Acad. Sci. USA, 106, 17817-17822.
-
(2008)
J. Gene Med.
, vol.10
, pp. 930-947
-
-
Campbell, M.1
Kiang, A.S.2
Kenna, P.F.3
Kerskens, C.4
Blau, C.5
O'Dwyer, L.6
Tivnan, A.7
Kelly, J.A.8
Brankin, B.9
Farrar, G.J.10
Humphries, P.11
-
48
-
-
3142514201
-
Protein aggregation and neurodegenerative disease
-
Campbell, M., Kiang, A.S., Kenna, P.F., Kerskens, C., Blau, C., O'Dwyer, L., Tivnan, A., Kelly, J.A., Brankin, B., Farrar, G.J. and Humphries, P. (2008) RNAi-mediated reversible opening of the blood-brain barrier. J. Gene Med., 10, 930-947.
-
(2004)
Nat. Med.
, vol.10
-
-
Ross, C.A.1
Poirier, M.A.2
-
49
-
-
71349083669
-
Conformational diseases: Looking into the eyes
-
Ross, C.A. and Poirier, M.A. (2004) Protein aggregation and neurodegenerative disease. Nat. Med., 10, S10-S17.
-
(2009)
Brain Res. Bull.
, vol.81
, pp. 12-24
-
-
Surguchev, A.1
Surguchov, A.2
-
50
-
-
57449087203
-
Preventing beta-amyloid fibrillization and deposition: beta-sheet breakers and pathological chaperone inhibitors
-
Surguchev, A. and Surguchov, A. (2009) Conformational diseases: Looking into the eyes. Brain Res. Bull., 81, 12-24.
-
(2008)
BMC Neurosci
, vol.9
-
-
Wisniewski, T.1
Sadowski, M.2
-
51
-
-
34347258879
-
Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation A possible therapeutic strategy
-
Wisniewski, T. and Sadowski, M. (2008) Preventing beta-amyloid fibrillization and deposition: beta-sheet breakers and pathological chaperone inhibitors. BMC Neurosci., 9, S5.
-
(2007)
Neurodegener. Dis.
, vol.4
, pp. 254-260
-
-
Herbst, M.1
Wanker, E.E.2
-
52
-
-
0036852712
-
Pharmacological prevention of Parkinson disease in Drosophila
-
Herbst, M. and Wanker, E.E. (2007) Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy. Neurodegener. Dis., 4, 254-260.
-
(2002)
Nat. Med.
, vol.8
, pp. 1185-1186
-
-
Auluck, P.K.1
Bonini, N.M.2
-
53
-
-
0036319568
-
Geldanamycin induces heat shock proteins in brain and protects against focal cerebral ischemia
-
Auluck, P.K. and Bonini, N.M. (2002) Pharmacological prevention of Parkinson disease in Drosophila. Nat. Med., 8, 1185-1186.
-
(2002)
J. Neurochem.
, vol.81
, pp. 355-364
-
-
Lu, A.1
Ran, R.2
Parmentier-Batteur, S.3
Nee, A.4
Sharp, F.R.5
-
54
-
-
0037102362
-
Getting out of shape
-
Lu, A., Ran, R., Parmentier-Batteur, S., Nee, A. and Sharp, F.R. (2002) Geldanamycin induces heat shock proteins in brain and protects against focal cerebral ischemia. J. Neurochem., 81, 355-364.
-
(2002)
Nature
, vol.418
, pp. 729-730
-
-
Dobson, C.M.1
-
55
-
-
61349192627
-
IMP dehydrogenase type 1 associates with polyribosomes translating rhodopsin mRNA
-
Dobson, C.M. (2002) Getting out of shape. Nature, 418, 729-730.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 36354-36360
-
-
Mortimer, S.E.1
Xu, D.2
McGrew, D.3
Hamaguchi, N.4
Lim, H.C.5
Bowne, S.J.6
Daiger, S.P.7
Hedstrom, L.8
-
56
-
-
3242695184
-
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
-
Mortimer, S.E., Xu, D., McGrew, D., Hamaguchi, N., Lim, H.C., Bowne, S.J., Daiger, S.P. and Hedstrom, L. (2008) IMP dehydrogenase type 1 associates with polyribosomes translating rhodopsin mRNA. J. Biol. Chem., 283, 36354-36360.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 1389-1405
-
-
Hay, D.G.1
Sathasivam, K.2
Tobaben, S.3
Stahl, B.4
Marber, M.5
Mestril, R.6
Mahal, A.7
Smith, D.L.8
Woodman, B.9
Bates, G.P.10
-
57
-
-
60549084901
-
17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse
-
Hay, D.G., Sathasivam, K., Tobaben, S., Stahl, B., Marber, M., Mestril, R., Mahal, A., Smith, D.L., Woodman, B. and Bates, G.P. (2004) Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Hum. Mol. Genet., 13, 1389-1405.
-
(2009)
Hum. Mol. Genet.
, vol.18
, pp. 898-910
-
-
Tokui, K.1
Adachi, H.2
Waza, M.3
Katsuno, M.4
Minamiyama, M.5
Doi, H.6
Tanaka, K.7
Hamazaki, J.8
Murata, S.9
-
58
-
-
20944444881
-
Phase I pharmacokinetic-pharmacodynamic study of 17-(allylamino)-17-demethoxygeldanamycin (17AAG, NSC 330507), a novel inhibitor of heat shock protein 90, in patients with refractory advanced cancers
-
Tokui, K., Adachi, H., Waza, M., Katsuno, M., Minamiyama, M., Doi, H., Tanaka, K., Hamazaki, J., Murata, S. et al. (2009) 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse. Hum. Mol. Genet., 18, 898-910.
-
(2005)
Clin. Cancer Res.
, vol.11
, pp. 3385-3391
-
-
Ramanathan, R.K.1
Trump, D.L.2
Eiseman, J.L.3
Belani, C.P.4
Agarwala, S.S.5
Zuhowski, E.G.6
Lan, J.7
Potter, D.M.8
Ivy, S.P.9
Ramalingam, S.10
-
59
-
-
20044384168
-
Phase I trial of 17-allylamino-17-demethoxygeldanamycin in patients with advanced cancer
-
Ramanathan, R.K., Trump, D.L., Eiseman, J.L., Belani, C.P., Agarwala, S.S., Zuhowski, E.G., Lan, J., Potter, D.M., Ivy, S.P., Ramalingam, S. et al. (2005) Phase I pharmacokinetic-pharmacodynamic study of 17-(allylamino)-17-demethoxygeldanamycin (17AAG, NSC 330507), a novel inhibitor of heat shock protein 90, in patients with refractory advanced cancers. Clin. Cancer Res., 11, 3385-3391.
-
(2005)
J. Clin. Oncol.
, vol.23
, pp. 1078-1087
-
-
Goetz, M.P.1
Toft, D.2
Reid, J.3
Ames, M.4
Stensgard, B.5
Safgren, S.6
Adjei, A.A.7
Sloan, J.8
Atherton, P.9
Vasile, V.10
-
60
-
-
21244462689
-
In vivo antitumor efficacy of 17-DMAG (17-dimethylaminoethylamino-17-demethoxygeldanamycin hydrochloride), a water-soluble geldanamycin derivative
-
Goetz, M.P., Toft, D., Reid, J., Ames, M., Stensgard, B., Safgren, S., Adjei, A.A., Sloan, J., Atherton, P., Vasile, V. et al. (2005) Phase I trial of 17-allylamino-17-demethoxygeldanamycin in patients with advanced cancer. J. Clin. Oncol., 23, 1078-1087.
-
(2005)
Cancer Chemother. Pharmacol.
, vol.56
, pp. 115-125
-
-
Hollingshead, M.1
Alley, M.2
Burger, A.M.3
Borgel, S.4
Pacula-Cox, C.5
Fiebig, H.H.6
Sausville, E.A.7
-
61
-
-
33745888997
-
17-dimethylaminoethylamino-17-demethoxygeldanamycin in patients with advanced-stage solid tumors and lymphoma: a phase I study
-
Hollingshead, M., Alley, M., Burger, A.M., Borgel, S., Pacula-Cox, C., Fiebig, H.H. and Sausville, E.A. (2005) In vivo antitumor efficacy of 17-DMAG (17-dimethylaminoethylamino-17-demethoxygeldanamycin hydrochloride), a water-soluble geldanamycin derivative. Cancer Chemother. Pharmacol., 56, 115-125.
-
(2006)
Clin. Lymphoma Myeloma
, vol.6
, pp. 500-501
-
-
Shadad, F.N.1
Ramanathan, R.K.2
-
62
-
-
77950929054
-
Phase I study of the heat shock protein 90 inhibitor alvespimycin (KOS-1022 17-DMAG) administered intravenously twice weekly to patients with acute myeloid leukemia
-
Shadad, F.N. and Ramanathan, R.K. (2006) 17-dimethylaminoethylamino-17-demethoxygeldanamycin in patients with advanced-stage solid tumors and lymphoma: a phase I study. Clin. Lymphoma Myeloma, 6, 500-501.
-
(2010)
Leukemia
, vol.24
, pp. 699-705
-
-
Lancet, J.E.1
Gojo, I.2
Burton, M.3
Quinn, M.4
Tighe, S.M.5
Kersey, K.6
Zhong, Z.7
Albitar, M.X.8
Bhalla, K.9
Hannah, A.L.10
-
63
-
-
34247498083
-
Synergism between arsenic trioxide and heat shock protein 90 inhibitors on signal transducer and activator of transcription protein 3 activity-pharmacodynamic drug-drug interaction modeling
-
Lancet, J.E., Gojo, I., Burton, M., Quinn, M., Tighe, S.M., Kersey, K., Zhong, Z., Albitar, M.X., Bhalla, K., Hannah, A.L. et al. (2010) Phase I study of the heat shock protein 90 inhibitor alvespimycin (KOS-1022, 17-DMAG) administered intravenously twice weekly to patients with acute myeloid leukemia. Leukemia, 24, 699-705.
-
(2007)
Clin. Cancer Res.
, vol.13
, pp. 2261-2270
-
-
Wetzler, M.1
Earp, J.C.2
Brady, M.T.3
Keng, M.K.4
Jusko, W.J.5
-
64
-
-
0031045876
-
Retinopathy induced in mice by targeted disruption of the rhodopsin gene
-
Wetzler, M., Earp, J.C., Brady, M.T., Keng, M.K. and Jusko, W.J. (2007) Synergism between arsenic trioxide and heat shock protein 90 inhibitors on signal transducer and activator of transcription protein 3 activity-pharmacodynamic drug-drug interaction modeling. Clin. Cancer Res., 13, 2261-2270.
-
(1997)
Nat. Genet.
, vol.15
, pp. 216-219
-
-
Humphries, M.M.1
Rancourt, D.2
Farrar, G.J.3
Kenna, P.4
Hazel, M.5
Bush, R.A.6
Sieving, P.A.7
Sheils, D.M.8
McNally, N.9
Creighton, P.10
-
65
-
-
23844529037
-
Toward a gene therapy for dominant disease: validation of an RNA interference-based mutation-independent approach
-
Humphries, M.M., Rancourt, D., Farrar, G.J., Kenna, P., Hazel, M., Bush, R.A., Sieving, P.A., Sheils, D.M., McNally, N., Creighton, P. et al. (1997) Retinopathy induced in mice by targeted disruption of the rhodopsin gene. Nat. Genet., 15, 216-219.
-
(2005)
Mol. Ther.
, vol.12
, pp. 555-561
-
-
Kiang, A.S.1
Palfi, A.2
Ader, M.3
Kenna, P.F.4
Millington-Ward, S.5
Clark, G.6
Kennan, A.7
O'Reilly, M.8
Tam, L.C.9
Aherne, A.10
|