-
1
-
-
0033624419
-
Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease
-
Albers D.S., Beal M.F. Mitochondrial dysfunction and oxidative stress in aging and neurodegenerative disease. J Neural Transm Suppl. 59:2000;133-154.
-
(2000)
J Neural Transm Suppl
, vol.59
, pp. 133-154
-
-
Albers, D.S.1
Beal, M.F.2
-
2
-
-
17044446594
-
Antagonist properties of the stereoisomers of ifenprodil at NR1A/NR2A and NR1A/NR2B subtypes of the NMDA receptor expressed in Xenopus oocytes
-
Avenet P., Leonardon J., Besnard F., Graham D., Frost J., Depoortere H., Langer S.Z., Scatton B. Antagonist properties of the stereoisomers of ifenprodil at NR1A/NR2A and NR1A/NR2B subtypes of the NMDA receptor expressed in Xenopus oocytes. Eur J Pharmacol. 296:1996;209-213.
-
(1996)
Eur J Pharmacol
, vol.296
, pp. 209-213
-
-
Avenet, P.1
Leonardon, J.2
Besnard, F.3
Graham, D.4
Frost, J.5
Depoortere, H.6
Langer, S.Z.7
Scatton, B.8
-
3
-
-
0022446150
-
Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
-
Beal M.F., Kowall N.W., Ellison D.W., Mazurek M.F., Swartz K.J., Martin J.B. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature. 321:1986;168-171.
-
(1986)
Nature
, vol.321
, pp. 168-171
-
-
Beal, M.F.1
Kowall, N.W.2
Ellison, D.W.3
Mazurek, M.F.4
Swartz, K.J.5
Martin, J.B.6
-
4
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence N.F., Sampat R.M., Kopito R.R. Impairment of the ubiquitin-proteasome system by protein aggregation. Science. 292:2001;1552-1555.
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
5
-
-
0031797267
-
Increased vulnerability to 3-nitropropionic acid in an animal model of Huntington's disease
-
Bogdanov M.B., Ferrante R.J., Kuemmerle S., Klivenyi P., Beal M.F. Increased vulnerability to 3-nitropropionic acid in an animal model of Huntington's disease. J Neurochem. 71:1998;2642-2644.
-
(1998)
J Neurochem
, vol.71
, pp. 2642-2644
-
-
Bogdanov, M.B.1
Ferrante, R.J.2
Kuemmerle, S.3
Klivenyi, P.4
Beal, M.F.5
-
6
-
-
0035668684
-
Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease
-
Bogdanov M.B., Andreassen O.A., Dedeoglu A., Ferrante R.J., Beal M.F. Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease. J Neurochem. 79:2001;1246-1249.
-
(2001)
J Neurochem
, vol.79
, pp. 1246-1249
-
-
Bogdanov, M.B.1
Andreassen, O.A.2
Dedeoglu, A.3
Ferrante, R.J.4
Beal, M.F.5
-
8
-
-
0030919567
-
Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia
-
Browne S.E., Bowling A.C., MacGarvey U., Baik M.J., Berger S.C., Muqit M.M., Bird E.D., Beal M.F. Oxidative damage and metabolic dysfunction in Huntington's disease selective vulnerability of the basal ganglia . Ann Neurol. 41:1997;646-653.
-
(1997)
Ann Neurol
, vol.41
, pp. 646-653
-
-
Browne, S.E.1
Bowling, A.C.2
MacGarvey, U.3
Baik, M.J.4
Berger, S.C.5
Muqit, M.M.6
Bird, E.D.7
Beal, M.F.8
-
9
-
-
0016153291
-
Superoxide- and singlet oxygen-catalyzed lipid peroxidation as a possible mechanism for paraquat (methyl viologen) toxicity
-
Bus J.S., Aust S.D., Gibson J.E. Superoxide- and singlet oxygen-catalyzed lipid peroxidation as a possible mechanism for paraquat (methyl viologen) toxicity. Biochem Biophys Res Commun. 58:1974;749-755.
-
(1974)
Biochem Biophys Res Commun
, vol.58
, pp. 749-755
-
-
Bus, J.S.1
Aust, S.D.2
Gibson, J.E.3
-
11
-
-
0032581167
-
Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum
-
Butterworth N.J., Williams L., Bullock J.Y., Love D.R., Faull R.L., Dragunow M. Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum. Neuroscience. 87:1998;49-53.
-
(1998)
Neuroscience
, vol.87
, pp. 49-53
-
-
Butterworth, N.J.1
Williams, L.2
Bullock, J.Y.3
Love, D.R.4
Faull, R.L.5
Dragunow, M.6
-
12
-
-
0035889535
-
NMDA receptor function in mouse models of Huntington disease
-
Cepeda C., Ariano M.A., Calvert C.R., Flores-Hernandez J., Chandler S.H., Leavitt B.R., Hayden M.R., Levine M.S. NMDA receptor function in mouse models of Huntington disease. J Neurosci Res. 66:2001;525-539.
-
(2001)
J Neurosci Res
, vol.66
, pp. 525-539
-
-
Cepeda, C.1
Ariano, M.A.2
Calvert, C.R.3
Flores-Hernandez, J.4
Chandler, S.H.5
Leavitt, B.R.6
Hayden, M.R.7
Levine, M.S.8
-
13
-
-
0033499931
-
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
-
Chai Y., Koppenhafer S.L., Bonini N.M., Paulson H.L. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J Neurosci. 19:1999;10338-10347.
-
(1999)
J Neurosci
, vol.19
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
14
-
-
0033030565
-
Evidence for proteasome involvement in polyglutamine disease: Localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
-
Chai Y., Koppenhafer S.L., Shoesmith S.J., Perez M.K., Paulson H.L. Evidence for proteasome involvement in polyglutamine disease localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro . Hum Mol Genet. 8:1999;673-682.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 673-682
-
-
Chai, Y.1
Koppenhafer, S.L.2
Shoesmith, S.J.3
Perez, M.K.4
Paulson, H.L.5
-
15
-
-
0033566918
-
Subtype-dependence of NMDA receptor channel open probability
-
Chen N., Luo T., Raymond L.A. Subtype-dependence of NMDA receptor channel open probability. J Neurosci. 19:1999;6844-6854.
-
(1999)
J Neurosci
, vol.19
, pp. 6844-6854
-
-
Chen, N.1
Luo, T.2
Raymond, L.A.3
-
16
-
-
0023137252
-
Ionic dependence of glutamate neurotoxicity
-
Choi D.W. Ionic dependence of glutamate neurotoxicity. J Neurosci. 7:1987;369-379.
-
(1987)
J Neurosci
, vol.7
, pp. 369-379
-
-
Choi, D.W.1
-
17
-
-
0037181474
-
Mutant huntingtin aggregates do not sensitize cells to apoptotic stressors
-
Chun W., Lesort M., Lee M., Johnson G.V. Mutant huntingtin aggregates do not sensitize cells to apoptotic stressors. FEBS Lett. 515:2002;61-65.
-
(2002)
FEBS Lett
, vol.515
, pp. 61-65
-
-
Chun, W.1
Lesort, M.2
Lee, M.3
Johnson, G.V.4
-
18
-
-
7144253143
-
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture
-
Cooper J.K., Schilling G., Peters M.F., Herring W.J., Sharp A.H., Kaminsky Z., Masone J., Khan F.A., Delanoy M., Borchelt D.R., Dawson V.L., Dawson T.M., Ross C.A. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum Mol Genet. 7:1998;783-790.
-
(1998)
Hum Mol Genet
, vol.7
, pp. 783-790
-
-
Cooper, J.K.1
Schilling, G.2
Peters, M.F.3
Herring, W.J.4
Sharp, A.H.5
Kaminsky, Z.6
Masone, J.7
Khan, F.A.8
Delanoy, M.9
Borchelt, D.R.10
Dawson, V.L.11
Dawson, T.M.12
Ross, C.A.13
-
19
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies S.W., Turmaine M., Cozens B.A., DiFiglia M., Sharp A.H., Ross C.A., Scherzinger E., Wanker E.E., Mangiarini L., Bates G.P. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell. 90:1997;537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
DiFiglia, M.4
Sharp, A.H.5
Ross, C.A.6
Scherzinger, E.7
Wanker, E.E.8
Mangiarini, L.9
Bates, G.P.10
-
20
-
-
0023750525
-
Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease
-
de la Monte S.M., Vonsattel J.P., Richardson E.P. Jr. Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease. J Neuropathol Exp Neurol. 47:1988;516-525.
-
(1988)
J Neuropathol Exp Neurol
, vol.47
, pp. 516-525
-
-
De la Monte, S.M.1
Vonsattel, J.P.2
Richardson E.P., Jr.3
-
22
-
-
0029040355
-
In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes
-
Dragunow M., Faull R.L., Lawlor P., Beilharz E.J., Singleton K., Walker E.B., Mee E. In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes. Neuroreport. 6:1995;1053-1057.
-
(1995)
Neuroreport
, vol.6
, pp. 1053-1057
-
-
Dragunow, M.1
Faull, R.L.2
Lawlor, P.3
Beilharz, E.J.4
Singleton, K.5
Walker, E.B.6
Mee, E.7
-
23
-
-
0027396021
-
Excitotoxin lesions in primates as a model for Huntington's disease: Histopathologic and neurochemical characterization
-
Ferrante R.J., Kowall N.W., Cipolloni P.B., Storey E., Beal M.F. Excitotoxin lesions in primates as a model for Huntington's disease histopathologic and neurochemical characterization . Exp Neurol. 119:1993;46-71.
-
(1993)
Exp Neurol
, vol.119
, pp. 46-71
-
-
Ferrante, R.J.1
Kowall, N.W.2
Cipolloni, P.B.3
Storey, E.4
Beal, M.F.5
-
24
-
-
0033510014
-
Mitochondria in neurodegeneration: Acute ischemia and chronic neurodegenerative diseases
-
Fiskum G., Murphy A.N., Beal M.F. Mitochondria in neurodegeneration acute ischemia and chronic neurodegenerative diseases . J Cereb Blood Flow Metab. 19:1999;351-369.
-
(1999)
J Cereb Blood Flow Metab
, vol.19
, pp. 351-369
-
-
Fiskum, G.1
Murphy, A.N.2
Beal, M.F.3
-
25
-
-
0029871365
-
Interactions between ifenprodil and the NR2B subunit of the N-methyl-D-aspartate receptor
-
Gallagher M.J., Huang H., Pritchett D.B., Lynch D.R. Interactions between ifenprodil and the NR2B subunit of the N-methyl-D-aspartate receptor. J Biol Chem. 271:1996;9603-9611.
-
(1996)
J Biol Chem
, vol.271
, pp. 9603-9611
-
-
Gallagher, M.J.1
Huang, H.2
Pritchett, D.B.3
Lynch, D.R.4
-
26
-
-
0030879848
-
Caspase inhibition selectively reduces the apoptotic component of oxygen-glucose deprivation-induced cortical neuronal cell death
-
Gottron F.J., Ying H.S., Choi D.W. Caspase inhibition selectively reduces the apoptotic component of oxygen-glucose deprivation-induced cortical neuronal cell death. Mol Cell Neurosci. 9:1997;159-169.
-
(1997)
Mol Cell Neurosci
, vol.9
, pp. 159-169
-
-
Gottron, F.J.1
Ying, H.S.2
Choi, D.W.3
-
27
-
-
0031041530
-
Slowly triggered excitotoxicity occurs by necrosis in cortical cultures
-
Gwag B.J., Koh J.Y., DeMaro J.A., Ying H.S., Jacquin M., Choi D.W. Slowly triggered excitotoxicity occurs by necrosis in cortical cultures. Neuroscience. 77:1997;393-401.
-
(1997)
Neuroscience
, vol.77
, pp. 393-401
-
-
Gwag, B.J.1
Koh, J.Y.2
DeMaro, J.A.3
Ying, H.S.4
Jacquin, M.5
Choi, D.W.6
-
28
-
-
0032101287
-
The influence of huntingtin protein size on nuclear localization and cellular toxicity
-
Hackam A.S., Singaraja R., Wellington C.L., Metzler M., McCutcheon K., Zhang T., Kalchman M., Hayden M.R. The influence of huntingtin protein size on nuclear localization and cellular toxicity. J Cell Biol. 141:1998;1097-1105.
-
(1998)
J Cell Biol
, vol.141
, pp. 1097-1105
-
-
Hackam, A.S.1
Singaraja, R.2
Wellington, C.L.3
Metzler, M.4
McCutcheon, K.5
Zhang, T.6
Kalchman, M.7
Hayden, M.R.8
-
29
-
-
0032424883
-
Regional specificity of brain atrophy in Huntington's disease
-
Halliday G.M., McRitchie D.A., Macdonald V., Double K.L., Trent R.J., McCusker E. Regional specificity of brain atrophy in Huntington's disease. Exp Neurol. 154:1998;663-672.
-
(1998)
Exp Neurol
, vol.154
, pp. 663-672
-
-
Halliday, G.M.1
McRitchie, D.A.2
Macdonald, V.3
Double, K.L.4
Trent, R.J.5
McCusker, E.6
-
30
-
-
0034889544
-
Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length
-
Hansson O., Castilho R.F., Korhonen L., Lindholm D., Bates G.P., Brundin P. Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length. J Neurochem. 78:2001;694-703.
-
(2001)
J Neurochem
, vol.78
, pp. 694-703
-
-
Hansson, O.1
Castilho, R.F.2
Korhonen, L.3
Lindholm, D.4
Bates, G.P.5
Brundin, P.6
-
31
-
-
0033784437
-
Mice transgenic for the Huntington's disease mutation are resistant to chronic 3-nitropropionic acid-induced striatal toxicity
-
Hickey M.A., Morton A.J. Mice transgenic for the Huntington's disease mutation are resistant to chronic 3-nitropropionic acid-induced striatal toxicity. J Neurochem. 75:2000;2163-2171.
-
(2000)
J Neurochem
, vol.75
, pp. 2163-2171
-
-
Hickey, M.A.1
Morton, A.J.2
-
32
-
-
0033136692
-
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
-
Hodgson J.G., Agopyan N., Gutekunst C.A., Leavitt B.R., LePiane F., Lingaraja R., Smith D.J., Bissada N., McCutcheon K., Nasir J., Jamot L., Li X.J., Stevens M.E., Rosemond E., Roder J.C., Phillips A.G., Rubin E.M., Hersch S.M., Hayden M.R. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron. 23:1999;181-192.
-
(1999)
Neuron
, vol.23
, pp. 181-192
-
-
Hodgson, J.G.1
Agopyan, N.2
Gutekunst, C.A.3
Leavitt, B.R.4
LePiane, F.5
Lingaraja, R.6
Smith, D.J.7
Bissada, N.8
McCutcheon, K.9
Nasir, J.10
Jamot, L.11
Li, X.J.12
Stevens, M.E.13
Rosemond, E.14
Roder, J.C.15
Phillips, A.G.16
Rubin, E.M.17
Hersch, S.M.18
Hayden, M.R.19
-
33
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell. 72:1993;971-983.
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
34
-
-
0032827758
-
N-methyl-D-aspartate receptor blockade induces neuronal apoptosis in cortical culture
-
Hwang J.Y., Kim Y.H., Ahn Y.H., Wie M.B., Koh J.Y. N-methyl-D-aspartate receptor blockade induces neuronal apoptosis in cortical culture. Exp Neurol. 159:1999;124-130.
-
(1999)
Exp Neurol
, vol.159
, pp. 124-130
-
-
Hwang, J.Y.1
Kim, Y.H.2
Ahn, Y.H.3
Wie, M.B.4
Koh, J.Y.5
-
35
-
-
0035336658
-
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release
-
Jana N.R., Zemskov E.A., Wang G., Nukina N. Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Hum Mol Genet. 10:2001;1049-1059.
-
(2001)
Hum Mol Genet
, vol.10
, pp. 1049-1059
-
-
Jana, N.R.1
Zemskov, E.A.2
Wang, G.3
Nukina, N.4
-
36
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
Kim M., Lee H.S., LaForet G., McIntyre C., Martin E.J., Chang P., Kim T.W., Williams M., Reddy P.H., Tagle D., Boyce F.M., Won L., Heller A., Aronin N., DiFiglia M. Mutant huntingtin expression in clonal striatal cells dissociation of inclusion formation and neuronal survival by caspase inhibition . J Neurosci. 19:1999;964-973.
-
(1999)
J Neurosci
, vol.19
, pp. 964-973
-
-
Kim, M.1
Lee, H.S.2
LaForet, G.3
McIntyre, C.4
Martin, E.J.5
Chang, P.6
Kim, T.W.7
Williams, M.8
Reddy, P.H.9
Tagle, D.10
Boyce, F.M.11
Won, L.12
Heller, A.13
Aronin, N.14
DiFiglia, M.15
-
37
-
-
0034708793
-
Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract
-
Kobayashi Y., Kume A., Li M., Doyu M., Hata M., Ohtsuka K., Sobue G. Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract. J Biol Chem. 275:2000;8772-8778.
-
(2000)
J Biol Chem
, vol.275
, pp. 8772-8778
-
-
Kobayashi, Y.1
Kume, A.2
Li, M.3
Doyu, M.4
Hata, M.5
Ohtsuka, K.6
Sobue, G.7
-
38
-
-
0028824798
-
Staurosporine-induced neuronal apoptosis
-
Koh J.Y., Wie M.B., Gwag B.J., Sensi S.L., Canzoniero L.M., Demaro J., Csernansky C., Choi D.W. Staurosporine-induced neuronal apoptosis. Exp Neurol. 135:1995;153-159.
-
(1995)
Exp Neurol
, vol.135
, pp. 153-159
-
-
Koh, J.Y.1
Wie, M.B.2
Gwag, B.J.3
Sensi, S.L.4
Canzoniero, L.M.5
Demaro, J.6
Csernansky, C.7
Choi, D.W.8
-
39
-
-
0023221068
-
Quantitative determination of glutamate mediated cortical neuronal injury in cell culture by lactate dehydrogenase efflux assay
-
Koh J.-Y., Choi D.W. Quantitative determination of glutamate mediated cortical neuronal injury in cell culture by lactate dehydrogenase efflux assay. J Neurosci Methods. 20:1987;83-90.
-
(1987)
J Neurosci Methods
, vol.20
, pp. 83-90
-
-
Koh, J.-Y.1
Choi, D.W.2
-
40
-
-
0025800526
-
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
-
La Spada A.R., Wilson E.M., Lubahn D.B., Harding A.E., Fischbeck K.H. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature. 352:1991;77-79.
-
(1991)
Nature
, vol.352
, pp. 77-79
-
-
La Spada, A.R.1
Wilson, E.M.2
Lubahn, D.B.3
Harding, A.E.4
Fischbeck, K.H.5
-
41
-
-
0035575858
-
Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease
-
Laforet G.A., Sapp E., Chase K., McIntyre C., Boyce F.M., Campbell M., Cadigan B.A., Warzecki L., Tagle D.A., Reddy P.H., Cepeda C., Calvert C.R., Jokel E.S., Klapstein G.J., Ariano M.A., Levine M.S., DiFiglia M., Aronin N. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci. 21:2001;9112-9123.
-
(2001)
J Neurosci
, vol.21
, pp. 9112-9123
-
-
Laforet, G.A.1
Sapp, E.2
Chase, K.3
McIntyre, C.4
Boyce, F.M.5
Campbell, M.6
Cadigan, B.A.7
Warzecki, L.8
Tagle, D.A.9
Reddy, P.H.10
Cepeda, C.11
Calvert, C.R.12
Jokel, E.S.13
Klapstein, G.J.14
Ariano, M.A.15
Levine, M.S.16
DiFiglia, M.17
Aronin, N.18
-
42
-
-
0033571743
-
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
-
Levine M.S., Klapstein G.J., Koppel A., Gruen E., Cepeda C., Vargas M.E., Jokel E.S., Carpenter E.M., Zanjani H., Hurst R.S., Efstratiadis A., Zeitlin S., Chesselet M.F. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res. 58:1999;515-532.
-
(1999)
J Neurosci Res
, vol.58
, pp. 515-532
-
-
Levine, M.S.1
Klapstein, G.J.2
Koppel, A.3
Gruen, E.4
Cepeda, C.5
Vargas, M.E.6
Jokel, E.S.7
Carpenter, E.M.8
Zanjani, H.9
Hurst, R.S.10
Efstratiadis, A.11
Zeitlin, S.12
Chesselet, M.F.13
-
43
-
-
0033168302
-
Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin
-
Li S.H., Cheng A.L., Li H., Li X.J. Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin. J Neurosci. 19:1999;5159-5172.
-
(1999)
J Neurosci
, vol.19
, pp. 5159-5172
-
-
Li, S.H.1
Cheng, A.L.2
Li, H.3
Li, X.J.4
-
44
-
-
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., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S.W., Bates G.P. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell. 87:1996;493-506.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
Cozens, B.4
Harper, A.5
Hetherington, C.6
Lawton, M.7
Trottier, Y.8
Lehrach, H.9
Davies, S.W.10
Bates, G.P.11
-
45
-
-
0027231818
-
The topographic distribution of brain atrophy in Huntington's disease and progressive supranuclear palsy
-
Mann D.M., Oliver R., Snowden J.S. The topographic distribution of brain atrophy in Huntington's disease and progressive supranuclear palsy. Acta Neuropathol (Berl). 85:1993;553-559.
-
(1993)
Acta Neuropathol (Berl)
, vol.85
, pp. 553-559
-
-
Mann, D.M.1
Oliver, R.2
Snowden, J.S.3
-
46
-
-
0034194616
-
Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity
-
Morton A.J., Leavens W. Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity. Brain Res Bull. 52:2000;51-59.
-
(2000)
Brain Res Bull
, vol.52
, pp. 51-59
-
-
Morton, A.J.1
Leavens, W.2
-
47
-
-
0036451840
-
The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia
-
Namura S., Hirt L., Wheeler V.C., McGinnis K.M., Hilditch-Maguire P., Moskowitz M.A., MacDonald M.E., Persichetti F. The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia. Neurobiol Dis. 11:2002;147-154.
-
(2002)
Neurobiol Dis
, vol.11
, pp. 147-154
-
-
Namura, S.1
Hirt, L.2
Wheeler, V.C.3
McGinnis, K.M.4
Hilditch-Maguire, P.5
Moskowitz, M.A.6
MacDonald, M.E.7
Persichetti, F.8
-
48
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
Ona V.O., Li M., Vonsattel J.P., Andrews L.J., Khan S.Q., Chung W.M., Frey A.S., Menon A.S., Li X.J., Stieg P.E., Yuan J., Penney J.B., Young A.B., Cha J.H., Friedlander R.M. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature. 399:1999;263-267.
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.3
Andrews, L.J.4
Khan, S.Q.5
Chung, W.M.6
Frey, A.S.7
Menon, A.S.8
Li, X.J.9
Stieg, P.E.10
Yuan, J.11
Penney, J.B.12
Young, A.B.13
Cha, J.H.14
Friedlander, R.M.15
-
49
-
-
0033071176
-
Protein fate in neurodegenerative proteinopathies: Polyglutamine diseases join the (mis)fold
-
Paulson H.L. Protein fate in neurodegenerative proteinopathies polyglutamine diseases join the (mis)fold . Am J Hum Genet. 64:1999;339-345.
-
(1999)
Am J Hum Genet
, vol.64
, pp. 339-345
-
-
Paulson, H.L.1
-
50
-
-
0029072690
-
Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
-
Portera-Cailliau C., Hedreen J.C., Price D.L., Koliatsos V.E. Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models. J Neurosci. 15:1995;3775-3787.
-
(1995)
J Neurosci
, vol.15
, pp. 3775-3787
-
-
Portera-Cailliau, C.1
Hedreen, J.C.2
Price, D.L.3
Koliatsos, V.E.4
-
51
-
-
0028823737
-
Pharmacological properties of recombinant human N-methyl-D-aspartate receptors comprising NR1a/NR2A and NR1a/NR2B subunit assemblies expressed in permanently transfected mouse fibroblast cells
-
Priestley T., Laughton P., Myers J., Le Bourdelles B., Kerby J., Whiting P.J. Pharmacological properties of recombinant human N-methyl-D-aspartate receptors comprising NR1a/NR2A and NR1a/NR2B subunit assemblies expressed in permanently transfected mouse fibroblast cells. Mol Pharmacol. 48:1995;841-848.
-
(1995)
Mol Pharmacol
, vol.48
, pp. 841-848
-
-
Priestley, T.1
Laughton, P.2
Myers, J.3
Le Bourdelles, B.4
Kerby, J.5
Whiting, P.J.6
-
52
-
-
0000991680
-
Proteasome inhibitors induce cytochrome c-caspase-3-like protease- mediated apoptosis in cultured cortical neurons
-
Qiu J.H., Asai A., Chi S., Saito N., Hamada H., Kirino T. Proteasome inhibitors induce cytochrome c-caspase-3-like protease- mediated apoptosis in cultured cortical neurons. J Neurosci. 20:2000;259-265.
-
(2000)
J Neurosci
, vol.20
, pp. 259-265
-
-
Qiu, J.H.1
Asai, A.2
Chi, S.3
Saito, N.4
Hamada, H.5
Kirino, T.6
-
53
-
-
17344367977
-
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
-
Reddy P.H., Williams M., Charles V., Garrett L., Pike-Buchanan L., Whetsell W.O. Jr, Miller G., Tagle D.A. Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. Nat Genet. 20:1998;198-202.
-
(1998)
Nat Genet
, vol.20
, pp. 198-202
-
-
Reddy, P.H.1
Williams, M.2
Charles, V.3
Garrett, L.4
Pike-Buchanan, L.5
Whetsell W.O., Jr.6
Miller, G.7
Tagle, D.A.8
-
54
-
-
0037066115
-
Regional and progressive thinning of the cortical ribbon in Huntington's disease
-
Rosas H.D., Liu A.K., Hersch S., Glessner M., Ferrante R.J., Salat D.H., van der Kouwe A., Jenkins B.G., Dale A.M., Fischl B. Regional and progressive thinning of the cortical ribbon in Huntington's disease. Neurology. 58:2002;695-701.
-
(2002)
Neurology
, vol.58
, pp. 695-701
-
-
Rosas, H.D.1
Liu, A.K.2
Hersch, S.3
Glessner, M.4
Ferrante, R.J.5
Salat, D.H.6
Van der Kouwe, A.7
Jenkins, B.G.8
Dale, A.M.9
Fischl, B.10
-
55
-
-
0001884447
-
Cytotoxicity in murine neocortical cell culture
-
C. Tyson, & J. Frazier. San Diego, CA: Academic Press
-
Rose K., Choi D.W., Goldberg M.P. Cytotoxicity in murine neocortical cell culture. Tyson C., Frazier J. In vitro biological methods, Vol. 1A. 1993;46-60 Academic Press, San Diego, CA.
-
(1993)
In vitro biological methods, Vol. 1A
, pp. 46-60
-
-
Rose, K.1
Choi, D.W.2
Goldberg, M.P.3
-
56
-
-
0033103523
-
Caspase-8 is required for cell death induced by expanded polyglutamine repeats
-
Sanchez I., Xu C.J., Juo P., Kakizaka A., Blenis J., Yuan J. Caspase-8 is required for cell death induced by expanded polyglutamine repeats. Neuron. 22:1999;623-633.
-
(1999)
Neuron
, vol.22
, pp. 623-633
-
-
Sanchez, I.1
Xu, C.J.2
Juo, P.3
Kakizaka, A.4
Blenis, J.5
Yuan, J.6
-
58
-
-
0030887032
-
Determination of the time course and extent of neurotoxicity at defined temperatures in cultured neurons using a modified multiwell plate fluorescence scanner
-
Sattler R., Charlton M.P., Hafner M., Tymianski M. Determination of the time course and extent of neurotoxicity at defined temperatures in cultured neurons using a modified multiwell plate fluorescence scanner. J Cereb Blood Flow Metab. 17:1997;455-463.
-
(1997)
J Cereb Blood Flow Metab
, vol.17
, pp. 455-463
-
-
Sattler, R.1
Charlton, M.P.2
Hafner, M.3
Tymianski, M.4
-
59
-
-
0030887853
-
Genetic determinants of susceptibility to excitotoxic cell death: Implications for gene targeting approaches
-
Schauwecker P.E., Steward O. Genetic determinants of susceptibility to excitotoxic cell death implications for gene targeting approaches . Proc Natl Acad Sci USA. 94:1997;4103-4108.
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 4103-4108
-
-
Schauwecker, P.E.1
Steward, O.2
-
60
-
-
0029079428
-
Expression of the Huntington disease gene in rodents: Cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development
-
Schmitt I., Bachner D., Megow D., Henklein P., Hameister H., Epplen J.T., Riess O. Expression of the Huntington disease gene in rodents cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development . Hum Mol Genet. 4:1995;1173-1182.
-
(1995)
Hum Mol Genet
, vol.4
, pp. 1173-1182
-
-
Schmitt, I.1
Bachner, D.2
Megow, D.3
Henklein, P.4
Hameister, H.5
Epplen, J.T.6
Riess, O.7
-
61
-
-
0027010449
-
A primate model of Huntington's disease: Functional neural transplantation and CT-guided stereotactic procedures
-
Schumacher J.M., Hantraye P., Brownell A.L., Riche D., Madras B.K., Davenport P.D., Maziere M., Elmaleh D.R., Brownell G.L., Isacson O. A primate model of Huntington's disease functional neural transplantation and CT-guided stereotactic procedures . Cell Transplant. 1:1992;313-322.
-
(1992)
Cell Transplant
, vol.1
, pp. 313-322
-
-
Schumacher, J.M.1
Hantraye, P.2
Brownell, A.L.3
Riche, D.4
Madras, B.K.5
Davenport, P.D.6
Maziere, M.7
Elmaleh, D.R.8
Brownell, G.L.9
Isacson, O.10
-
62
-
-
0018069998
-
Comparison of the expression of the src gene of rous sarcoma virus in vitro and in vivo
-
Sefton B.M., Beemon K., Hunter T. Comparison of the expression of the src gene of rous sarcoma virus in vitro and in vivo. J Virol. 28:1978;957-971.
-
(1978)
J Virol
, vol.28
, pp. 957-971
-
-
Sefton, B.M.1
Beemon, K.2
Hunter, T.3
-
63
-
-
0022186670
-
Measurement of protein using bicinchoninic acid
-
Smith P.K., Krohn R.I., Hermanson G.T., Mallia A.K., Gartner F.H., Provenzano M.D., Fujimoto E.K., Goeke N.M., Olson B.J., Klenk D.C. Measurement of protein using bicinchoninic acid. Anal Biochem. 150:1985;76-85.
-
(1985)
Anal Biochem
, vol.150
, pp. 76-85
-
-
Smith, P.K.1
Krohn, R.I.2
Hermanson, G.T.3
Mallia, A.K.4
Gartner, F.H.5
Provenzano, M.D.6
Fujimoto, E.K.7
Goeke, N.M.8
Olson, B.J.9
Klenk, D.C.10
-
64
-
-
0036454320
-
NMDA antagonists exacerbate neuronal death caused by proteasome inhibition in cultured cortical and striatal neurons
-
Snider B.J., Tee L.Y., Canzoniero L.M., Babcock D.J., Choi D.W. NMDA antagonists exacerbate neuronal death caused by proteasome inhibition in cultured cortical and striatal neurons. Eur J Neurosci. 15:2002;419-428.
-
(2002)
Eur J Neurosci
, vol.15
, pp. 419-428
-
-
Snider, B.J.1
Tee, L.Y.2
Canzoniero, L.M.3
Babcock, D.J.4
Choi, D.W.5
-
65
-
-
0032945938
-
Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
-
Stenoien D.L., Cummings C.J., Adams H.P., Mancini M.G., Patel K., DeMartino G.N., Marcelli M., Weigel N.L., Mancini M.A. Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum Mol Genet. 8:1999;731-741.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 731-741
-
-
Stenoien, D.L.1
Cummings, C.J.2
Adams, H.P.3
Mancini, M.G.4
Patel, K.5
DeMartino, G.N.6
Marcelli, M.7
Weigel, N.L.8
Mancini, M.A.9
-
66
-
-
0035816627
-
Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95
-
Sun Y., Savanenin A., Reddy P.H., Liu Y.F. Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem. 276:2001;24713-24718.
-
(2001)
J Biol Chem
, vol.276
, pp. 24713-24718
-
-
Sun, Y.1
Savanenin, A.2
Reddy, P.H.3
Liu, Y.F.4
-
68
-
-
0020440918
-
The metabolism of menadione (2-methyl-1,4-naphthoquinone) by isolated hepatocytes: A study of the implications of oxidative stress in intact cells
-
Thor H., Smith M.T., Hartzell P., Bellomo G., Jewell S.A., Orrenius S. The metabolism of menadione (2-methyl-1,4-naphthoquinone) by isolated hepatocytes a study of the implications of oxidative stress in intact cells . J Biol Chem. 257:1982;12419-12425.
-
(1982)
J Biol Chem
, vol.257
, pp. 12419-12425
-
-
Thor, H.1
Smith, M.T.2
Hartzell, P.3
Bellomo, G.4
Jewell, S.A.5
Orrenius, S.6
-
69
-
-
0028364337
-
A cytotoxicity assay for tumor necrosis factor employing a multiwell fluorescence scanner
-
Trost L.C., Lemasters J.J. A cytotoxicity assay for tumor necrosis factor employing a multiwell fluorescence scanner. Anal Biochem. 220:1994;149-153.
-
(1994)
Anal Biochem
, vol.220
, pp. 149-153
-
-
Trost, L.C.1
Lemasters, J.J.2
-
70
-
-
0034608857
-
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
-
Turmaine M., Raza A., Mahal A., Mangiarini L., Bates G.P., Davies S.W. Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci USA. 97:2000;8093-8097.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 8093-8097
-
-
Turmaine, M.1
Raza, A.2
Mahal, A.3
Mangiarini, L.4
Bates, G.P.5
Davies, S.W.6
-
71
-
-
0034733607
-
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
-
Wellington C.L., Singaraja R., Ellerby L., Savill J., Roy S., Leavitt B., Cattaneo E., Hackam A., Sharp A., Thornberry N., Nicholson D.W., Bredesen D.E., Hayden M.R. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. J Biol Chem. 275:2000;19831-19838.
-
(2000)
J Biol Chem
, vol.275
, pp. 19831-19838
-
-
Wellington, C.L.1
Singaraja, R.2
Ellerby, L.3
Savill, J.4
Roy, S.5
Leavitt, B.6
Cattaneo, E.7
Hackam, A.8
Sharp, A.9
Thornberry, N.10
Nicholson, D.W.11
Bredesen, D.E.12
Hayden, M.R.13
-
72
-
-
0037087771
-
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
-
Wheeler V.C., Gutekunst C.A., Vrbanac V., Lebel L.A., Schilling G., Hersch S., Friedlander R.M., Gusella J.F., Vonsattel J.P., Borchelt D.R., MacDonald M.E. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet. 11:2002;633-640.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 633-640
-
-
Wheeler, V.C.1
Gutekunst, C.A.2
Vrbanac, V.3
Lebel, L.A.4
Schilling, G.5
Hersch, S.6
Friedlander, R.M.7
Gusella, J.F.8
Vonsattel, J.P.9
Borchelt, D.R.10
MacDonald, M.E.11
-
73
-
-
0032938295
-
Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
-
Wheeler V.C., Auerbach W., White J.K., Srinidhi J., Auerbach A., Ryan A., Duyao M.P., Vrbanac V., Weaver M., Gusella J.F., Joyner A.L., MacDonald M.E. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet. 8:1999;115-122.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 115-122
-
-
Wheeler, V.C.1
Auerbach, W.2
White, J.K.3
Srinidhi, J.4
Auerbach, A.5
Ryan, A.6
Duyao, M.P.7
Vrbanac, V.8
Weaver, M.9
Gusella, J.F.10
Joyner, A.L.11
MacDonald, M.E.12
-
74
-
-
0034163497
-
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
-
Wheeler V.C., White J.K., Gutekunst C.A., Vrbanac V., Weaver M., Li X.J., Li S.H., Yi H., Vonsattel J.P., Gusella J.F., Hersch S., Auerbach W., Joyner A.L., MacDonald M.E. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet. 9:2000;503-513.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 503-513
-
-
Wheeler, V.C.1
White, J.K.2
Gutekunst, C.A.3
Vrbanac, V.4
Weaver, M.5
Li, X.J.6
Li, S.H.7
Yi, H.8
Vonsattel, J.P.9
Gusella, J.F.10
Hersch, S.11
Auerbach, W.12
Joyner, A.L.13
MacDonald, M.E.14
-
75
-
-
0035154912
-
Mutant huntingtin enhances excitotoxic cell death
-
Zeron M.M., Chen N., Moshaver A., Lee A.T., Wellington C.L., Hayden M.R., Raymond L.A. Mutant huntingtin enhances excitotoxic cell death. Mol Cell Neurosci. 17:2001;41-53.
-
(2001)
Mol Cell Neurosci
, vol.17
, pp. 41-53
-
-
Zeron, M.M.1
Chen, N.2
Moshaver, A.3
Lee, A.T.4
Wellington, C.L.5
Hayden, M.R.6
Raymond, L.A.7
-
76
-
-
0037075624
-
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease
-
Zeron M.M., Hansson O., Chen N., Wellington C.L., Leavitt B.R., Brundin P., Hayden M.R., Raymond L.A. Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron. 33:2002;849-860.
-
(2002)
Neuron
, vol.33
, pp. 849-860
-
-
Zeron, M.M.1
Hansson, O.2
Chen, N.3
Wellington, C.L.4
Leavitt, B.R.5
Brundin, P.6
Hayden, M.R.7
Raymond, L.A.8
-
77
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi H.Y., Orr H.T. Glutamine repeats and neurodegeneration. Annu Rev Neurosci. 23:2000;217-247.
-
(2000)
Annu Rev Neurosci
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
|