-
2
-
-
0000510404
-
Huntington's disease
-
Wells RD, Warren ST, eds. San Diego: Academic Press
-
Myers RH, Marans KS, MacDonald ME. Huntington's disease. In: Wells RD, Warren ST, eds. Genetic instabilities and hereditary neurological diseases. San Diego: Academic Press, 1998:301-323.
-
(1998)
Genetic Instabilities and Hereditary Neurological Diseases
, pp. 301-323
-
-
Myers, R.H.1
Marans, K.S.2
MacDonald, M.E.3
-
3
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia M, Sapp E, Chase KO, et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997;277:1990-1993.
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
-
4
-
-
0031918640
-
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length
-
Becher MW, Kotzuk JA, Sharp AH, et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998;4:387-397.
-
(1998)
Neurobiol Dis
, vol.4
, pp. 387-397
-
-
Becher, M.W.1
Kotzuk, J.A.2
Sharp, A.H.3
-
5
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Gutekunst CA, Li SH, Yi H, et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci 1999;19:2522-2534.
-
(1999)
J Neurosci
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
-
6
-
-
0033560924
-
Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
-
Carter RJ, Lione LA, Humby T, et al. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 1999;19:3248-3257.
-
(1999)
J Neurosci
, vol.19
, pp. 3248-3257
-
-
Carter, R.J.1
Lione, L.A.2
Humby, T.3
-
7
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini L, Sathasivam K, Seller M, et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996;87:493-506.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
-
8
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies SW, Turmaine M, Cozens BA, et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997;90:537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
-
9
-
-
0036523110
-
Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease
-
Ferrante RJ, Andreassen OA, Dedeoglu A, et al. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci 2002;22:1592-1599.
-
(2002)
J Neurosci
, vol.22
, pp. 1592-1599
-
-
Ferrante, R.J.1
Andreassen, O.A.2
Dedeoglu, A.3
-
10
-
-
0037109665
-
Therapeutic effects of cystamine in a murine model of Huntington's disease
-
Dedeoglu A, Kubilus JK, Jeitner TM, et al. Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 2002;22:8942-8950.
-
(2002)
J Neurosci
, vol.22
, pp. 8942-8950
-
-
Dedeoglu, A.1
Kubilus, J.K.2
Jeitner, T.M.3
-
11
-
-
0036652765
-
Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease
-
Schiefer J, Landwehrmeyer GB, Luesse HG, et al. Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease. Mov Disord 2002;17:748-757.
-
(2002)
Mov Disord
, vol.17
, pp. 748-757
-
-
Schiefer, J.1
Landwehrmeyer, G.B.2
Luesse, H.G.3
-
12
-
-
0034660457
-
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
-
Ferrante RJ, Andreassen OA, Jenkins BG, et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci 2000;20:4389-4397.
-
(2000)
J Neurosci
, vol.20
, pp. 4389-4397
-
-
Ferrante, R.J.1
Andreassen, O.A.2
Jenkins, B.G.3
-
13
-
-
0033912716
-
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
-
Chen M, Ona VO, Li M, et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med 2000;6:797-801.
-
(2000)
Nat Med
, vol.6
, pp. 797-801
-
-
Chen, M.1
Ona, V.O.2
Li, M.3
-
14
-
-
0035968856
-
Lipoic acid improves survival in transgenic mouse models of Huntington's disease
-
Andreassen OA, Ferrante RJ, Dedeoglu A, Beal MF. Lipoic acid improves survival in transgenic mouse models of Huntington's disease. Neuroreport 2001;12:3371-3373.
-
(2001)
Neuroreport
, vol.12
, pp. 3371-3373
-
-
Andreassen, O.A.1
Ferrante, R.J.2
Dedeoglu, A.3
Beal, M.F.4
-
15
-
-
0035969336
-
Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: Behavioral testing and impact of diabetes mellitus
-
Luesse HG, Schiefer J, Spruenken A, et al. Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: behavioral testing and impact of diabetes mellitus. Behav Brain Res 2001;126:185-195.
-
(2001)
Behav Brain Res
, vol.126
, pp. 185-195
-
-
Luesse, H.G.1
Schiefer, J.2
Spruenken, A.3
-
16
-
-
0034955984
-
Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease
-
Andreassen OA, Ferrante RJ, Huang HM, et al. Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. Ann Neurol 2001;50:112-117.
-
(2001)
Ann Neurol
, vol.50
, pp. 112-117
-
-
Andreassen, O.A.1
Ferrante, R.J.2
Huang, H.M.3
-
17
-
-
0034743672
-
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease
-
Andreassen OA, Dedeoglu A, Ferrante RJ, et al. Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease. Neurobiol Dis 2001;8:479-491.
-
(2001)
Neurobiol Dis
, vol.8
, pp. 479-491
-
-
Andreassen, O.A.1
Dedeoglu, A.2
Ferrante, R.J.3
-
18
-
-
0033054555
-
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
-
Schilling G, Becher MW, Sharp AH, et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 1999;8:397-407.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 397-407
-
-
Schilling, G.1
Becher, M.W.2
Sharp, A.H.3
-
19
-
-
17344367977
-
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
-
Reddy PH, Williams M, Charles V, et al. Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. Nat Genet 1998;20:198-202.
-
(1998)
Nat Genet
, vol.20
, pp. 198-202
-
-
Reddy, P.H.1
Williams, M.2
Charles, V.3
-
20
-
-
0035575858
-
Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease
-
Laforet GA, Sapp E, Chase K, et al. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci 2001;21:9112-9123.
-
(2001)
J Neurosci
, vol.21
, pp. 9112-9123
-
-
Laforet, G.A.1
Sapp, E.2
Chase, K.3
-
21
-
-
0033136692
-
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
-
Hodgson JG, Agopyan N, Gutekunst CA, et al. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 1999;23:181-192.
-
(1999)
Neuron
, vol.23
, pp. 181-192
-
-
Hodgson, J.G.1
Agopyan, N.2
Gutekunst, C.A.3
-
22
-
-
0035862896
-
Neurological abnormalities in a knock-in mouse model of Huntington's disease
-
Lin CH, Tallaksen-Greene S, Chien WM, et al. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 2001;10:137-144.
-
(2001)
Hum Mol Genet
, vol.10
, pp. 137-144
-
-
Lin, C.H.1
Tallaksen-Greene, S.2
Chien, W.M.3
-
23
-
-
0032949459
-
A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
-
Shelbourne PF, Killeen N, Hevner RF, et al. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Hum Mol Genet 1999;8:763-774.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 763-774
-
-
Shelbourne, P.F.1
Killeen, N.2
Hevner, R.F.3
-
24
-
-
0032938295
-
Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
-
Wheeler VC, Auerbach W, White JK, et al. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 1999;8:115-122.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 115-122
-
-
Wheeler, V.C.1
Auerbach, W.2
White, J.K.3
-
25
-
-
0033571743
-
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
-
Levine MS, Klapstein GJ, Koppel A, et al. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res 1999;58:515-532.
-
(1999)
J Neurosci Res
, vol.58
, pp. 515-532
-
-
Levine, M.S.1
Klapstein, G.J.2
Koppel, A.3
-
26
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
Yamamoto A, Lucas JJ, Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000;101:57-66.
-
(2000)
Cell
, vol.101
, pp. 57-66
-
-
Yamamoto, A.1
Lucas, J.J.2
Hen, R.3
-
27
-
-
0032198241
-
Inhibition of MMP synthesis by doxycycline and chemically modified tetracyclines (CMTs) in human endothelial cells
-
Hanemaaijer R, Visser H, Koolwijk P, et al. Inhibition of MMP synthesis by doxycycline and chemically modified tetracyclines (CMTs) in human endothelial cells. Adv Dent Res 1998;12:114-118.
-
(1998)
Adv Dent Res
, vol.12
, pp. 114-118
-
-
Hanemaaijer, R.1
Visser, H.2
Koolwijk, P.3
-
28
-
-
0032442475
-
Tetracyclines inhibit microglial activation and are neuroprotective in global brain ischemia
-
Yrjanheikki J, Keinanen R, Pellikka M, et al. Tetracyclines inhibit microglial activation and are neuroprotective in global brain ischemia. Proc Natl Acad Sci USA 1998;95:15769-15774.
-
(1998)
Proc Natl Acad Sci USA
, vol.95
, pp. 15769-15774
-
-
Yrjanheikki, J.1
Keinanen, R.2
Pellikka, M.3
-
29
-
-
0035807904
-
Minocycline prevents nigrostriatal dopaminergic neurodegeneration in the MPTP model of Parkinson's disease
-
Du Y, Ma Z, Lin S, et al. Minocycline prevents nigrostriatal dopaminergic neurodegeneration in the MPTP model of Parkinson's disease. Proc Natl Acad Sci USA 2001;98:14669-14674.
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 14669-14674
-
-
Du, Y.1
Ma, Z.2
Lin, S.3
-
30
-
-
0033539522
-
A tetracycline derivative, minocycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window
-
Yrjanheikki J, Tikka T, Keinanen R, et al. A tetracycline derivative, minocycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window. Proc Nati Acad Sci USA 1999;96:13496-13500.
-
(1999)
Proc Nati Acad Sci USA
, vol.96
, pp. 13496-13500
-
-
Yrjanheikki, J.1
Tikka, T.2
Keinanen, R.3
-
31
-
-
0036406903
-
Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis
-
Kriz J, Nguyen MD, Julien JP. Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 2002;10:268-278.
-
(2002)
Neurobiol Dis
, vol.10
, pp. 268-278
-
-
Kriz, J.1
Nguyen, M.D.2
Julien, J.P.3
-
32
-
-
0035876916
-
Minocycline provides neuroprotection against N-methyl-D-aspartate neurotoxicity by inhibiting microglia
-
Tikka TM, Koistinaho JE. Minocycline provides neuroprotection against N-methyl-D-aspartate neurotoxicity by inhibiting microglia. J Immunol 2001;166:7527-7533.
-
(2001)
J Immunol
, vol.166
, pp. 7527-7533
-
-
Tikka, T.M.1
Koistinaho, J.E.2
-
33
-
-
0034725536
-
Tetracycline affects abnormal properties of synthetic PrP peptides and PrP(Sc) in vitro
-
Tagliavini F, Forloni G, Colombo L, et al. Tetracycline affects abnormal properties of synthetic PrP peptides and PrP(Sc) in vitro. J Mol Biol 2000;300:1309-1322.
-
(2000)
J Mol Biol
, vol.300
, pp. 1309-1322
-
-
Tagliavini, F.1
Forloni, G.2
Colombo, L.3
-
34
-
-
0035808264
-
Anti-amyloidogenic activity of tetracyclines: Studies in vitro
-
Forloni G, Colombo L, Girola L, et al. Anti-amyloidogenic activity of tetracyclines: studies in vitro. FEBS Lett 2001;487:404-407.
-
(2001)
FEBS Lett
, vol.487
, pp. 404-407
-
-
Forloni, G.1
Colombo, L.2
Girola, L.3
-
36
-
-
0032502715
-
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
-
Wellington CL, Ellerby LM, Hackam AS, et al. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem 1998;273:9158-9167.
-
(1998)
J Biol Chem
, vol.273
, pp. 9158-9167
-
-
Wellington, C.L.1
Ellerby, L.M.2
Hackam, A.S.3
-
37
-
-
0033953015
-
Caspases and neurodegeneration: On the cutting edge of new therapeutic approaches
-
Wellington CL, Hayden MR. Caspases and neurodegeneration: on the cutting edge of new therapeutic approaches. Clin Genet 2000;57:1-10.
-
(2000)
Clin Genet
, vol.57
, pp. 1-10
-
-
Wellington, C.L.1
Hayden, M.R.2
-
38
-
-
0034888540
-
Caspase inhibition: A potential therapeutic strategy in neurological diseases
-
Rideout HJ, Stefanis L. Caspase inhibition: a potential therapeutic strategy in neurological diseases. Histol Histopathol 2001;16:895-908.
-
(2001)
Histol Histopathol
, vol.16
, pp. 895-908
-
-
Rideout, H.J.1
Stefanis, L.2
-
39
-
-
0035668573
-
Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles
-
Smith DL, Portier R, Woodman B, et al. Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles. Neurobiol Dis 2001;8:1017-1026.
-
(2001)
Neurobiol Dis
, vol.8
, pp. 1017-1026
-
-
Smith, D.L.1
Portier, R.2
Woodman, B.3
-
40
-
-
0031056685
-
Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation
-
Mangiarini L, Sathasivam K, Mahal A, et al. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat Genet 1997;15:197-200.
-
(1997)
Nat Genet
, vol.15
, pp. 197-200
-
-
Mangiarini, L.1
Sathasivam, K.2
Mahal, A.3
-
41
-
-
0025066941
-
More powerful procedures for multiple significance testing
-
Hochberg Y, Benjamini Y. More powerful procedures for multiple significance testing. Stat Med 1990;9:811-818.
-
(1990)
Stat Med
, vol.9
, pp. 811-818
-
-
Hochberg, Y.1
Benjamini, Y.2
-
42
-
-
0034698254
-
Simple and reliable method of doxycycline determination in human plasma and biological tissues
-
Axisa B, Naylor AR, Bell PR, Thompson MM. Simple and reliable method of doxycycline determination in human plasma and biological tissues. J Chromatogr B Biomed Sci Appl 2000;744:359-65.
-
(2000)
J Chromatogr B Biomed Sci Appl
, vol.744
, pp. 359-365
-
-
Axisa, B.1
Naylor, A.R.2
Bell, P.R.3
Thompson, M.M.4
-
43
-
-
0141678246
-
Standardization and statistical approaches to therapeutic trials in the R6/2 mouse
-
in press
-
Hockly E, Woodman B, Mahal A, et al. Standardization and statistical approaches to therapeutic trials in the R6/2 mouse. Brain Res Bull (in press).
-
Brain Res Bull
-
-
Hockly, E.1
Woodman, B.2
Mahal, A.3
-
44
-
-
0035504960
-
Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients
-
Sathasivam K, Woodman B, Mahal A, et al. Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients. Hum Mol Genet 2001;10:2425-2435.
-
(2001)
Hum Mol Genet
, vol.10
, pp. 2425-2435
-
-
Sathasivam, K.1
Woodman, B.2
Mahal, A.3
-
45
-
-
0032919205
-
Formation of polyglutamine inclusions in non-CNS tissue
-
Sathasivam K, Hobbs C, Turmaine M, et al. Formation of polyglutamine inclusions in non-CNS tissue. Hum Mol Genet 1999;8:813-822.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 813-822
-
-
Sathasivam, K.1
Hobbs, C.2
Turmaine, M.3
-
46
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
Ona VO, Li M, Vonsattel JP, et al. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 1999;399:263-267.
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.3
-
47
-
-
85009226418
-
A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease
-
Huntington's Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001;57:397-404.
-
(2001)
Neurology
, vol.57
, pp. 397-404
-
-
|