-
1
-
-
0028836862
-
Genetics and molecular biology of HD
-
Albin R. L. and Tagle D. A. (1995) Genetics and molecular biology of HD. Trends Neurosci. 18, 11-14.
-
(1995)
Trends Neurosci.
, vol.18
, pp. 11-14
-
-
Albin, R.L.1
Tagle, D.A.2
-
2
-
-
1842334441
-
Anterograde transport of brain-derived neurotrophic factor and its role in the brain
-
Altar C. A., Cai N., Bliven T., et al. (1997) Anterograde transport of brain-derived neurotrophic factor and its role in the brain. Nature 389, 856-860.
-
(1997)
Nature
, vol.389
, pp. 856-860
-
-
Altar, C.A.1
Cai, N.2
Bliven, T.3
-
3
-
-
0028260436
-
Evidence against simple inactivation due to an expanded CAG repeat
-
Ambrose C. M., Duyao M. P., Barnes G., et al. (1994) Evidence against simple inactivation due to an expanded CAG repeat. Somat. Cell Molec. Genet. 20, 27-38.
-
(1994)
Somat. Cell Molec. Genet.
, vol.20
, pp. 27-38
-
-
Ambrose, C.M.1
Duyao, M.P.2
Barnes, G.3
-
4
-
-
0033614770
-
Are there multiple pathways in the pathogenesis of Huntington's disease?
-
Aronin N., Kim M., Laforet G., and DiFiglia M. (1999) Are there multiple pathways in the pathogenesis of Huntington's disease? Phil. Trans. R. Soc. Lond. B. 354, 995-1003.
-
(1999)
Phil. Trans. R. Soc. Lond. B.
, vol.354
, pp. 995-1003
-
-
Aronin, N.1
Kim, M.2
Laforet, G.3
DiFiglia, M.4
-
5
-
-
0035888618
-
The HD mutation causes progressive neurological disease in mice expressing reduced levels of huntingtin
-
Auerbach W., Hurlbert M. S., Hilditsch-Maguire P., et al. (2001) The HD mutation causes progressive neurological disease in mice expressing reduced levels of huntingtin. Hum. Mol. Gen. 10, 2515-2523.
-
(2001)
Hum. Mol. Gen.
, vol.10
, pp. 2515-2523
-
-
Auerbach, W.1
Hurlbert, M.S.2
Hilditsch-Maguire, P.3
-
6
-
-
0035947372
-
Impairment of the ubiquitin-proteosome system by protein aggregation
-
Bence N. F., Sampat R. M., and Kopito R. R. (2001) Impairment of the ubiquitin-proteosome system by protein aggregation. Science 292, 1552-1555.
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
7
-
-
9444286388
-
Expression of normal and mutant Huntingtin in the developing brain
-
Bhide P. G., Day M., Sapp E., et al. (1996) Expression of normal and mutant Huntingtin in the developing brain. J. Neurosci. 16, 5523-5535.
-
(1996)
J. Neurosci.
, vol.16
, pp. 5523-5535
-
-
Bhide, P.G.1
Day, M.2
Sapp, E.3
-
8
-
-
0000970542
-
Huntington's chorea
-
(Vinken P. J., Bruyn G. W., and Klawans H. L., eds.) Elsevier Science Publishers, Amsterdam, Netherlands
-
Bruyn G. W. and Went L. N. (1986) Huntington's chorea, In Handbook of Clinical Neurology, vol. 49 Extrapyramidal Disorders, revised series 5 (Vinken P. J., Bruyn G. W., and Klawans H. L., eds.) Elsevier Science Publishers, Amsterdam, Netherlands, pp. 267-313.
-
(1986)
Handbook of Clinical Neurology, Vol. 49 Extrapyramidal Disorders, Revised Series 5
, vol.49
, pp. 267-313
-
-
Bruyn, G.W.1
Went, L.N.2
-
9
-
-
0035282594
-
Loss of normal huntingtin function: New developments in Huntington's disease research
-
Cattaneo E. Rigamonti D. Goffredo D. Zuccato C. Squitieri F., and Sipione S. (2001) Loss of normal huntingtin function: new developments in Huntington's disease research. Trends Neurosci. 24, 182-188.
-
(2001)
Trends Neurosci.
, vol.24
, pp. 182-188
-
-
Cattaneo, E.1
Rigamonti, D.2
Goffredo, D.3
Zuccato, C.4
Squitieri, F.5
Sipione, S.6
-
10
-
-
0032568517
-
Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene
-
Cha J. H., Kosinski C. M., Kerner J. A., et al. (1998) Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc. Natl. Acad. Sci. USA 95, 6480-6485.
-
(1998)
Proc. Natl. Acad. Sci. USA
, vol.95
, pp. 6480-6485
-
-
Cha, J.H.1
Kosinski, C.M.2
Kerner, J.A.3
-
11
-
-
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., and Paulson H. L. (1999) 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, 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
-
12
-
-
0021260868
-
Huntington Disease: Genetics and epidemiology
-
Conneally P. M. (1984) Huntington Disease: Genetics and epidemiology. Am. J. Hum. Genet. 36, 506-526.
-
(1984)
Am. J. Hum. Genet.
, vol.36
, pp. 506-526
-
-
Conneally, P.M.1
-
13
-
-
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., et al. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
-
14
-
-
0023750525
-
Morphometric demonstrations of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease
-
De La Monte S. M., Vonsattel J. P., and Richardson E. P. Jr. (1988) Morphometric demonstrations of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease. J. Neuropathol. Exp. Neurol. 44, 516-525.
-
(1988)
J. Neuropathol. Exp. Neurol.
, vol.44
, pp. 516-525
-
-
De La Monte, S.M.1
Vonsattel, J.P.2
Richardson Jr., E.P.3
-
15
-
-
0028989602
-
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
-
DiFiglia M., Sapp E., Chase K., et al. (1995) Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14, 1075-1081.
-
(1995)
Neuron
, vol.14
, pp. 1075-1081
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.3
-
16
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia M., Sapp E., Chase K. O., Davies S. W., Bates G. P., Vonsattel J. P., and Aronin J. P. (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993.
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
Davies, S.W.4
Bates, G.P.5
Vonsattel, J.P.6
Aronin, J.P.7
-
17
-
-
0033614774
-
Analysis of the subcellular localization of huntingtin with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin: Comparison with distribution of huntingtin in Huntington's disease autopsy brain
-
Dorsman J. C., Smoor M. A., Maat-Schieman M. L. C, et al. (1999) Analysis of the subcellular localization of huntingtin with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin: comparison with distribution of huntingtin in Huntington's disease autopsy brain. Phil. Trans. R. Soc. Lond. 354, 1061-1067.
-
(1999)
Phil. Trans. R. Soc. Lond.
, vol.354
, pp. 1061-1067
-
-
Dorsman, J.C.1
Smoor, M.A.2
Maat-Schieman, M.L.C.3
-
18
-
-
2642646258
-
Mouse mutant embryos lacking huntingtin are rescued from lethality by wild-type extraembryonic tissues
-
Dragatsis I., Efstratiadis A., and Zeitlin S. (1998) Mouse mutant embryos lacking huntingtin are rescued from lethality by wild-type extraembryonic tissues. Development 125, 1529-1539.
-
(1998)
Development
, vol.125
, pp. 1529-1539
-
-
Dragatsis, I.1
Efstratiadis, A.2
Zeitlin, S.3
-
19
-
-
0033757718
-
Inactivation of the mouse Huntington's disease gene in the brain and testis results in progressive neurodegeneration and sterility
-
Dragatsis I., Levine M., and Zeitlin S. (2000) Inactivation of the mouse Huntington's disease gene in the brain and testis results in progressive neurodegeneration and sterility. Nature Genet. 26, 300-306.
-
(2000)
Nature Genet.
, vol.26
, pp. 300-306
-
-
Dragatsis, I.1
Levine, M.2
Zeitlin, S.3
-
20
-
-
0033624837
-
CaMKllalpha-Cre transgene expression and recombination patterns in the mouse brain
-
Dragatsis I. and Zeitlin S. (2000) CaMKllalpha-Cre transgene expression and recombination patterns in the mouse brain. Genesis 26, 133-135.
-
(2000)
Genesis
, vol.26
, pp. 133-135
-
-
Dragatsis, I.1
Zeitlin, S.2
-
21
-
-
0029082383
-
Inactivation of the mouse HD gene homolog
-
Duyao M. P., Auerbach A. B., Ryan A., et al. (1995) Inactivation of the mouse HD gene homolog Hdh. Science 269, 407-410.
-
(1995)
Hdh. Science
, vol.269
, pp. 407-410
-
-
Duyao, M.P.1
Auerbach, A.B.2
Ryan, A.3
-
22
-
-
0033557661
-
Cellular localization of huntingtin in striatal and cortical neurons in rats: Lack of correlation with neuronal vulnerability in Huntington's disease
-
Fusco F. R., Chen Q., Lamoreaux W. J., et al. (1999) Cellular localization of huntingtin in striatal and cortical neurons in rats: Lack of correlation with neuronal vulnerability in Huntington's disease. J. Neurosci. 19, 1189-1202.
-
(1999)
J. Neurosci.
, vol.19
, pp. 1189-1202
-
-
Fusco, F.R.1
Chen, Q.2
Lamoreaux, W.J.3
-
23
-
-
0036173770
-
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi
-
Gervais F. G., Singaraja R., Xanthoudakis S., et al. (2002) Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nature Cell Biol. 4, 95-105.
-
(2002)
Nature Cell Biol.
, vol.4
, pp. 95-105
-
-
Gervais, F.G.1
Singaraja, R.2
Xanthoudakis, S.3
-
24
-
-
0029873571
-
Trinucleotide instability: A repeating theme in human inherited disorders
-
Gusella J. F. and MacDonald M. E. (1996) Trinucleotide instability: A repeating theme in human inherited disorders. Ann. Rev. Med. 47, 201-209.
-
(1996)
Ann. Rev. Med.
, vol.47
, pp. 201-209
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
25
-
-
0029152808
-
Identification and localization of huntigtin in brain and hman lymphoblastoid cell lines with anti-fusion protein antibodies
-
Gutekunst C. A., Levey A. I., Heilman C. J., et al. (1995) Identification and localization of huntigtin in brain and hman lymphoblastoid cell lines with anti-fusion protein antibodies. Proc. Natl. Acad. Sci. USA 92, 8710-8714.
-
(1995)
Proc. Natl. Acad. Sci. USA
, vol.92
, pp. 8710-8714
-
-
Gutekunst, C.A.1
Levey, A.I.2
Heilman, C.J.3
-
26
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
Gutekunst C. A., Li S. H., Yi H., et al. (1999) Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci. 19, 2522-2534.
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
Li, S.H.2
Yi, H.3
-
27
-
-
0031970977
-
The fatal attraction of polyglutamine-containing proteins
-
Hackam A. S., Wellington C. L., and Hayden M. R. (1998) The fatal attraction of polyglutamine-containing proteins. Clin. Gen. 53, 233-242.
-
(1998)
Clin. Gen.
, vol.53
, pp. 233-242
-
-
Hackam, A.S.1
Wellington, C.L.2
Hayden, M.R.3
-
28
-
-
0034731370
-
Huntingtin interacting protein 1 induces apoptosis via novel caspase-dependent death effector domain
-
Hackam A. S., Yassa A. S., Singaraja R., et al. (2000) Huntingtin interacting protein 1 induces apoptosis via novel caspase-dependent death effector domain. J. Biol Chem. 275, 41,299-41,308.
-
(2000)
J. Biol Chem.
, vol.275
, pp. 41299-41308
-
-
Hackam, A.S.1
Yassa, A.S.2
Singaraja, R.3
-
29
-
-
0033007867
-
Expression of the Huntinton's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats
-
Hebb M. O., Denovan-Wright E. M., and Robertson H. A. (1999) Expression of the Huntinton's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats. FASEB 9, 1099-1106.
-
(1999)
FASEB
, vol.9
, pp. 1099-1106
-
-
Hebb, M.O.1
Denovan-Wright, E.M.2
Robertson, H.A.3
-
30
-
-
0025885733
-
Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease
-
Hedreen J. C., Peyser C. E., Folstein S. E., and Ross C. A. (1991) Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease. Neurosci. Lett. 133, 257-261.
-
(1991)
Neurosci. Lett.
, vol.133
, pp. 257-261
-
-
Hedreen, J.C.1
Peyser, C.E.2
Folstein, S.E.3
Ross, C.A.4
-
31
-
-
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., et al. (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23, 181-192.
-
(1999)
Neuron
, vol.23
, pp. 181-192
-
-
Hodgson, J.G.1
Agopyan, N.2
Gutekunst, C.A.3
-
32
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on the HD chromosome
-
Huntington's Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on the HD chromosome. Cell 72, 971-983.
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
33
-
-
0033168977
-
Expression of the striatal DARPP-32/ARPP-21 phenotype in GABAergic neurons requires neurotrophins in vivo and in vitro
-
Lvkovic S. and Ehrlich M. E. (1999) Expression of the striatal DARPP-32/ARPP-21 phenotype in GABAergic neurons requires neurotrophins in vivo and in vitro. J. Neurosci. 19, 5409-5419.
-
(1999)
J. Neurosci.
, vol.19
, pp. 5409-5419
-
-
Lvkovic, S.1
Ehrlich, M.E.2
-
34
-
-
0030986659
-
HIP1: A human homologue of S. cerevisiae sla2p interacts with membrane-associated huntingtin in the brain
-
Kalchman M. A., Koide H. B., McCutcheon K., et al. (1997) HIP1: A human homologue of S. cerevisiae sla2p interacts with membrane-associated huntingtin in the brain. Nat. Genet. 16, 44-53.
-
(1997)
Nat. Genet.
, vol.16
, pp. 44-53
-
-
Kalchman, M.A.1
Koide, H.B.2
McCutcheon, K.3
-
35
-
-
0034307476
-
Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy
-
Kegel K. B., Kim M., Sapp E., McIntyre C., Castano J. C., Aronin N., and DiFiglia M. (2000) Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. J. Neurosci. 20, 7268-7278.
-
(2000)
J. Neurosci.
, vol.20
, pp. 7268-7278
-
-
Kegel, K.B.1
Kim, M.2
Sapp, E.3
McIntyre, C.4
Castano, J.C.5
Aronin, N.6
DiFiglia, M.7
-
36
-
-
18544368523
-
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
-
Kegel K. B., Meloni A. R., Yi Y., et al. (2002) Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J. Biol. Chem. 277, 7466-7476.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 7466-7476
-
-
Kegel, K.B.1
Meloni, A.R.2
Yi, Y.3
-
37
-
-
0032191848
-
Neuronal intranuclear inclusions in polyglutamine diseases: Nuclear weapons or nuclear fallout
-
Kim T. W. and Tanzi R. E. (1998) Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout. Neuron 21, 657-659.
-
(1998)
Neuron
, vol.21
, pp. 657-659
-
-
Kim, T.W.1
Tanzi, R.E.2
-
38
-
-
0035502934
-
New anti-huntingtin monoclonal antibodies: Implications for huntingtin conformation and its binding proteins
-
Ko J., Ou S., and Patterson P. H. (2001) New anti-huntingtin monoclonal antibodies: Implications for huntingtin conformation and its binding proteins. Brain Res. Bull. 56, 319-329.
-
(2001)
Brain Res. Bull.
, vol.56
, pp. 319-329
-
-
Ko, J.1
Ou, S.2
Patterson, P.H.3
-
39
-
-
0028891145
-
HD gene: Regional and cellular expression in brain of normal and affected individuals
-
Landwehrmeyer G. B., McNeil S. M., Dure IV L. S., et al. (1995) HD gene: regional and cellular expression in brain of normal and affected individuals. Ann. Neurol. 37, 218-230.
-
(1995)
Ann. Neurol.
, vol.37
, pp. 218-230
-
-
Landwehrmeyer, G.B.1
McNeil, S.M.2
Dure IV, L.S.3
-
40
-
-
0035127907
-
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
-
Leavitt B. R., Guttman J. A., Hodgson J. G., et al. (2001) Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. Am. J. Hum. Genet. 68, 313-324.
-
(2001)
Am. J. Hum. Genet.
, vol.68
, pp. 313-324
-
-
Leavitt, B.R.1
Guttman, J.A.2
Hodgson, J.G.3
-
41
-
-
0031945025
-
Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats
-
Li S. H. and Li X. J. (1998) Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats. Human Mol. Gen. 7, 801-806.
-
(1998)
Human Mol. Gen.
, vol.7
, pp. 801-806
-
-
Li, S.H.1
Li, X.J.2
-
42
-
-
0027484673
-
HD gene (IT15) is widely expressed in human and rat tissues
-
Li S. H., Schilling G., Young W. S. III, et al. (1993) HD gene (IT15) is widely expressed in human and rat tissues. Neuron 11, 985-993.
-
(1993)
Neuron
, vol.11
, pp. 985-993
-
-
Li, S.H.1
Schilling, G.2
Young III, W.S.3
-
43
-
-
0037101835
-
Dysregulation of gene expression in the R6/2 model of polyglutamine disease: Parallel changes in muscle and brain
-
Luthi-Carter R., Hanson S., Strand A. D., et al. (2002) Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain. Human Mol. Genet. 11, 1911-1926.
-
(2002)
Human Mol. Genet.
, vol.11
, pp. 1911-1926
-
-
Luthi-Carter, R.1
Hanson, S.2
Strand, A.D.3
-
44
-
-
0033025958
-
Distribution of inclusions in neuronal nuclei and dystrophic neuntes in Huntington's disease brain
-
Maat-Schieman M. L. C., Dorsman J. C., Smoor M. A., et al. (1999) Distribution of inclusions in neuronal nuclei and dystrophic neuntes in Huntington's disease brain. J. Neuropath. Exp. Neurol. 58, 129-137.
-
(1999)
J. Neuropath. Exp. Neurol.
, vol.58
, pp. 129-137
-
-
Maat-Schieman, M.L.C.1
Dorsman, J.C.2
Smoor, M.A.3
-
45
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale D., Hackam A., Wieczorek A., et al. (1998) Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nature Gen. 18, 150-154.
-
(1998)
Nature Gen.
, vol.18
, pp. 150-154
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
-
46
-
-
0034537209
-
Mechanisms underlying neural cell death in neurodegenerative diseases: Alterations of a developmentally mediated cellular rheostat
-
Mehler M. F. and Gokhan S. (2000) Mechanisms underlying neural cell death in neurodegenerative diseases: alterations of a developmentally mediated cellular rheostat. Trends Neurosci. 23, 599-605.
-
(2000)
Trends Neurosci.
, vol.23
, pp. 599-605
-
-
Mehler, M.F.1
Gokhan, S.2
-
47
-
-
0035207082
-
Developmental mechanisms in the pathogenesis of neurodegenerative diseases
-
Mehler M. F. and Gokhan S. (2001) Developmental mechanisms in the pathogenesis of neurodegenerative diseases. Prog. Neurobiol. 63, 337-363.
-
(2001)
Prog. Neurobiol.
, vol.63
, pp. 337-363
-
-
Mehler, M.F.1
Gokhan, S.2
-
48
-
-
0032588968
-
Life without huntingtin: Normal differentiation into functional neurons
-
Metzler M., Chen N., Helgason C. D., et al. (1999) Life without huntingtin: Normal differentiation into functional neurons. J. Neurochem. 72, 1009-1018.
-
(1999)
J. Neurochem.
, vol.72
, pp. 1009-1018
-
-
Metzler, M.1
Chen, N.2
Helgason, C.D.3
-
49
-
-
0031889732
-
Equivalence in the genetic control of hindbrain segmentation in fish and mouse
-
Moens C. B., Cordes S. P., Giorgianni M. W., Barsh G. S., and Kimmel C. B. (1998) Equivalence in the genetic control of hindbrain segmentation in fish and mouse. Development 125, 381-391.
-
(1998)
Development
, vol.125
, pp. 381-391
-
-
Moens, C.B.1
Cordes, S.P.2
Giorgianni, M.W.3
Barsh, G.S.4
Kimmel, C.B.5
-
51
-
-
0032872817
-
A molecular investigation of true dominance in Huntington's disease
-
Narain Y., Wyttenbach A., Rankin J., Furlong R. A., and Rubinsztein D. C. (1999) A molecular investigation of true dominance in Huntington's disease. J. Med. Genet. 36, 739-746.
-
(1999)
J. Med. Genet.
, vol.36
, pp. 739-746
-
-
Narain, Y.1
Wyttenbach, A.2
Rankin, J.3
Furlong, R.A.4
Rubinsztein, D.C.5
-
52
-
-
0029055717
-
Targeted disruption of the HD gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
-
Nasir J., Floresco S. B., O'Kusky J. R., et al. (1995) Targeted disruption of the HD gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81, 811-823.
-
(1995)
Cell
, vol.81
, pp. 811-823
-
-
Nasir, J.1
Floresco, S.B.2
O'Kusky, J.R.3
-
53
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity
-
Nucifora F. C. Jr., Sasaki M., Peters M. F., et al. (2001) Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity. Science 291, 2423-2428.
-
(2001)
Science
, vol.291
, pp. 2423-2428
-
-
Nucifora Jr., F.C.1
Sasaki, M.2
Peters, M.F.3
-
54
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
Ona V. O., Li M., Vonsattel J. P., et al. (1999) Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 399, 263-267.
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.3
-
55
-
-
0030175161
-
Differential expression of normal and mutant Huntington's disease gene alleles
-
Persichetti F., Carlee L., Faber P. W., et al. (1996) Differential expression of normal and mutant Huntington's disease gene alleles. Neurobiol. Dis. 3, 183-190.
-
(1996)
Neurobiol. Dis.
, vol.3
, pp. 183-190
-
-
Persichetti, F.1
Carlee, L.2
Faber, P.W.3
-
56
-
-
0035364748
-
Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration
-
Petersén Å., Larsen K. E., Behr G. G., Romero N., Przedborski S., Brundin P., and Sulzer D. (2001) Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration. Hum. Mol. Genet. 10, 1243-1254.
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 1243-1254
-
-
Petersén, Å.1
Larsen, K.E.2
Behr, G.G.3
Romero, N.4
Przedborski, S.5
Brundin, P.6
Sulzer, D.7
-
57
-
-
0033614768
-
Evidence for a recruitment and sequestration mechanism in Huntinton's disease
-
Preisinger E., Jordan B. M., Kazantsev A., and Housman D. (1999) Evidence for a recruitment and sequestration mechanism in Huntinton's disease. Phil. Trans. Royal Soc. Lond. 354, 1029-1034.
-
(1999)
Phil. Trans. Royal Soc. Lond.
, vol.354
, pp. 1029-1034
-
-
Preisinger, E.1
Jordan, B.M.2
Kazantsev, A.3
Housman, D.4
-
58
-
-
0035478039
-
Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice
-
Reiner A., Del Mar N., Meade C. A., Yang H., Dragatsis I., Zeitlin S., and Goldowitz D. (2001) Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice. J. Neurosci. 21, 7608-7618.
-
(2001)
J. Neurosci.
, vol.21
, pp. 7608-7618
-
-
Reiner, A.1
Del Mar, N.2
Meade, C.A.3
Yang, H.4
Dragatsis, I.5
Zeitlin, S.6
Goldowitz, D.7
-
59
-
-
0034657112
-
Wild-type huntingtin protects from apoptosis upstream of caspase-3
-
Rigamonti D., Bauer J. H., De-Fraja C., et al. (2000) Wild-type huntingtin protects from apoptosis upstream of caspase-3. J. Neurosci. 20, 3705-3713.
-
(2000)
J. Neurosci.
, vol.20
, pp. 3705-3713
-
-
Rigamonti, D.1
Bauer, J.H.2
De-Fraja, C.3
-
60
-
-
0035805504
-
Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing
-
Rigamonti D., Sipione S., Goffredo D., Zuccato C., Fossale E., and Cattaneo E. (2001) Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing. J. Biol. Chem. 276, 14,545-14,548.
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 14545-14548
-
-
Rigamonti, D.1
Sipione, S.2
Goffredo, D.3
Zuccato, C.4
Fossale, E.5
Cattaneo, E.6
-
61
-
-
0000174823
-
Neuropathology of Huntington's Chorea
-
(Vinken P. J., Bruyn G. W., and Klawans H. L., eds.) Elsevier Science Publishers, Amsterdam, Netherlands
-
Roos R. A. C. (1986) Neuropathology of Huntington's Chorea, In: Handbook of Clinical Neurology, vol. 49 Extrapyramidal Disorders, revised series 5 (Vinken P. J., Bruyn G. W., and Klawans H. L., eds.) Elsevier Science Publishers, Amsterdam, Netherlands, pp. 315-326.
-
(1986)
Handbook of Clinical Neurology, Vol. 49 Extrapyramidal Disorders, Revised Series 5
, vol.49
, pp. 315-326
-
-
Roos, R.A.C.1
-
62
-
-
0037066115
-
Regional and progressive thinning of the cortical ribbon in Huntington's disease
-
Rosas H. D., Liu A. K., Hersch S., et al. (2002) Regional and progressive thinning of the cortical ribbon in Huntington's disease. Neurology 58, 695-701.
-
(2002)
Neurology
, vol.58
, pp. 695-701
-
-
Rosas, H.D.1
Liu, A.K.2
Hersch, S.3
-
63
-
-
0037194897
-
Polyglutamine pathogenesis: Emergence of unifying mechanisms for Huntington's disease and related disorders
-
Ross C. A. (2002) Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders. Neuron 35, 819-822.
-
(2002)
Neuron
, vol.35
, pp. 819-822
-
-
Ross, C.A.1
-
64
-
-
0342454322
-
Pathogenesis of polyglutamine neurodegenerative diseases: Toward a unifying hypothesis
-
(Wells R., ed.) Academic Press, New York
-
Ross C. A., Margolis R. L., Belcher M. W., Wood J. D., Engelender S., and Sharp A. H. (1998) Pathogenesis of polyglutamine neurodegenerative diseases: Toward a unifying hypothesis, In: Genetic Instabilities and Hereditary Neurological Disease (Wells R., ed.) Academic Press, New York, pp. 761-776.
-
(1998)
Genetic Instabilities and Hereditary Neurological Disease
, pp. 761-776
-
-
Ross, C.A.1
Margolis, R.L.2
Belcher, M.W.3
Wood, J.D.4
Engelender, S.5
Sharp, A.H.6
-
65
-
-
0033103523
-
Caspase-8 is required for cell death induced by expanded polyglutamine repeats
-
Sanchez I., Xu C. J., Juo P., Kakizaka A., Bienis J., and Yuan J. (1999) Caspase-8 is required for cell death induced by expanded polyglutamine repeats. Neuron 22, 623-633.
-
(1999)
Neuron
, vol.22
, pp. 623-633
-
-
Sanchez, I.1
Xu, C.J.2
Juo, P.3
Kakizaka, A.4
Bienis, J.5
Yuan, J.6
-
66
-
-
0030771894
-
Huntingtin localization in brains of normal and HD patients
-
Sapp E., Schwarz C., Chase K., et al. (1997) Huntingtin localization in brains of normal and HD patients. Ann. Neurol. 42, 604-612.
-
(1997)
Ann. Neurol.
, vol.42
, pp. 604-612
-
-
Sapp, E.1
Schwarz, C.2
Chase, K.3
-
67
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
Saudou F., Finkbeiner S., Devys D., and Greenberg M. E. (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55-66.
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
68
-
-
0029092340
-
Expression of the Huntington's disease (IT15) protein product in HD patients
-
Schilling G., Sharp A. H., Loev S. J., Wagster M. V., Li S. H., Stine O. C., and Ross C. A. (1995) Expression of the Huntington's disease (IT15) protein product in HD patients. Human Molec. Gen. 4, 1365-1371.
-
(1995)
Human Molec. Gen.
, vol.4
, pp. 1365-1371
-
-
Schilling, G.1
Sharp, A.H.2
Loev, S.J.3
Wagster, M.V.4
Li, S.H.5
Stine, O.C.6
Ross, C.A.7
-
69
-
-
0033004063
-
Neurotrophin regulation of synaptic transmission
-
Schuman E. M. (1999) Neurotrophin regulation of synaptic transmission. Curr. Opin. Neurobiol. 9, 105-109.
-
(1999)
Curr. Opin. Neurobiol.
, vol.9
, pp. 105-109
-
-
Schuman, E.M.1
-
70
-
-
0029991245
-
Neurobiology of Huntington's Disease
-
Sharp A. H. and Ross C. A. (1996) Neurobiology of Huntington's Disease. Neurobiol. Dis. 3, 3-15.
-
(1996)
Neurobiol. Dis.
, vol.3
, pp. 3-15
-
-
Sharp, A.H.1
Ross, C.A.2
-
71
-
-
0029034511
-
Widespread expression of HD gene (IT15) protein product
-
Sharp A. H., Loev S. J., Schilling G., et al. (1995) Widespread expression of HD gene (IT15) protein product. Neuron 14, 1065-1074.
-
(1995)
Neuron
, vol.14
, pp. 1065-1074
-
-
Sharp, A.H.1
Loev, S.J.2
Schilling, G.3
-
72
-
-
0032055648
-
Identification of a signaling pathway involved in calcium regulation of BDNF expression
-
Shieh P. B., Hu S. C., Bobb K., Timmusk T., and Ghosh A. (1998) Identification of a signaling pathway involved in calcium regulation of BDNF expression. Neuron 20, 727-740.
-
(1998)
Neuron
, vol.20
, pp. 727-740
-
-
Shieh, P.B.1
Hu, S.C.2
Bobb, K.3
Timmusk, T.4
Ghosh, A.5
-
73
-
-
0032475877
-
Nuclear inclusion in glutamine repeat disorders: Are they pernicious, coincidental or beneficial?
-
Sisodia S. S. (1998) Nuclear inclusion in glutamine repeat disorders: Are they pernicious, coincidental or beneficial? Cell 95, 1-4.
-
(1998)
Cell
, vol.95
, pp. 1-4
-
-
Sisodia, S.S.1
-
74
-
-
0037379416
-
Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course
-
Squitieri F., Gellera C., Cannella M., et al. (2003) Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course. Brain 126, 946-955.
-
(2003)
Brain
, vol.126
, pp. 946-955
-
-
Squitieri, F.1
Gellera, C.2
Cannella, M.3
-
75
-
-
0026793837
-
The cortical lesion of Huntington's disease: Further neurochemical characterization and reproduction of some of the histological and neurochemical features by N-Methyl-D-Aspartate
-
Storey E., Kowall N. W., Finn S. F., Mazurek M. F., and Beal M. F. (1992) The cortical lesion of Huntington's disease: further neurochemical characterization and reproduction of some of the histological and neurochemical features by N-Methyl-D-Aspartate. Ann. Neurol. 32, 526-534.
-
(1992)
Ann. Neurol.
, vol.32
, pp. 526-534
-
-
Storey, E.1
Kowall, N.W.2
Finn, S.F.3
Mazurek, M.F.4
Beal, M.F.5
-
76
-
-
0027432418
-
Widespread expression of the human and rat Huntington's disease gene in brain and non-neural tissues
-
Strong T. V., Tagle D. A., Valdes J. M., et al. (1993) Widespread expression of the human and rat Huntington's disease gene in brain and non-neural tissues. Nat. Genet. 5, 259-265.
-
(1993)
Nat. Genet.
, vol.5
, pp. 259-265
-
-
Strong, T.V.1
Tagle, D.A.2
Valdes, J.M.3
-
77
-
-
0032034262
-
Ca2+ influx regulates BDNF transcription by a CREB family transcription factor-dependent mechanism
-
Tao X., Finkbeiner S., Arnold D. B., Shaywitz A., and Greenberg M. E. (1998) Ca2+ influx regulates BDNF transcription by a CREB family transcription factor-dependent mechanism. Neuron 20, 709-726.
-
(1998)
Neuron
, vol.20
, pp. 709-726
-
-
Tao, X.1
Finkbeiner, S.2
Arnold, D.B.3
Shaywitz, A.4
Greenberg, M.E.5
-
78
-
-
0031867231
-
Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytotic pathways
-
Velier J., Kim M., Schwarz C., Kim T. W., Sapp E., Chase K., Aronin N., and DiFiglia M. (1998) Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytotic pathways. Exp. Neurol. 152, 34-40.
-
(1998)
Exp. Neurol.
, vol.152
, pp. 34-40
-
-
Velier, J.1
Kim, M.2
Schwarz, C.3
Kim, T.W.4
Sapp, E.5
Chase, K.6
Aronin, N.7
DiFiglia, M.8
-
80
-
-
0022395922
-
Neuropathological classification of Huntington's disease
-
Vonsattel J. P., Myers R. H., Stevens T. J., Ferrante R. J., Bird E. D., and Richardson E. P. (1985) Neuropathological classification of Huntington's disease. J. Neuropathol. Exp. Neurol. 44, 559-577.
-
(1985)
J. Neuropathol. Exp. Neurol.
, vol.44
, pp. 559-577
-
-
Vonsattel, J.P.1
Myers, R.H.2
Stevens, T.J.3
Ferrante, R.J.4
Bird, E.D.5
Richardson, E.P.6
-
81
-
-
0023115076
-
Homozygotes for Huntington's Disease
-
Wexler N. S., Young A. B., Tanzi R. E., et al. (1987) Homozygotes for Huntington's Disease. Nature 326, 194-197.
-
(1987)
Nature
, vol.326
, pp. 194-197
-
-
Wexler, N.S.1
Young, A.B.2
Tanzi, R.E.3
-
82
-
-
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., et al. (2000) 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, 503-513.
-
(2000)
Hum. Mol. Genet.
, vol.9
, pp. 503-513
-
-
Wheeler, V.C.1
White, J.K.2
Gutekunst, C.A.3
-
83
-
-
0030613177
-
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
-
White J. K., Auerbach W., Duyao M. P., Vonsattel J. P., Gusella J. F., Joyner A. L., and MacDonald M. E. (1997) Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nature Genet. 17, 404-410.
-
(1997)
Nature Genet.
, vol.17
, pp. 404-410
-
-
White, J.K.1
Auerbach, W.2
Duyao, M.P.3
Vonsattel, J.P.4
Gusella, J.F.5
Joyner, A.L.6
MacDonald, M.E.7
-
84
-
-
0344849472
-
Localization of rabbit huntingtin using a new panel of monoclonal antibodies
-
Wilkinson F. L., Man N. T., Manual S. B., Thomas P., Neal J. W., Harper P. S., Jones A. L., and Morris G. E. (1999) Localization of rabbit huntingtin using a new panel of monoclonal antibodies. Mol. Brain Res. 69, 10-20.
-
(1999)
Mol. Brain Res.
, vol.69
, pp. 10-20
-
-
Wilkinson, F.L.1
Man, N.T.2
Manual, S.B.3
Thomas, P.4
Neal, J.W.5
Harper, P.S.6
Jones, A.L.7
Morris, G.E.8
-
85
-
-
0023118448
-
Memory failure in Huntington's disease
-
Wilson R. S., Como P. G., Garron D. C., Klawans H. L., Barr A., and Klawans D. (1987) Memory failure in Huntington's disease. J. Clin. Exp. Neuropsychol. 9, 147-154.
-
(1987)
J. Clin. Exp. Neuropsychol.
, vol.9
, pp. 147-154
-
-
Wilson, R.S.1
Como, P.G.2
Garron, D.C.3
Klawans, H.L.4
Barr, A.5
Klawans, D.6
-
86
-
-
0029947276
-
Partial characterization of murine huntingtin and apparent variations in the subcellular localization of huntingtin in human, mouse and rat brain
-
Wood J. D., McLaughlin J. C., Harper P. S., Lowenstein P. R., and Jones A. L. (1996) Partial characterization of murine huntingtin and apparent variations in the subcellular localization of huntingtin in human, mouse and rat brain. Hum. Molec. Genet. 5, 481-487.
-
(1996)
Hum. Molec. Genet.
, vol.5
, pp. 481-487
-
-
Wood, J.D.1
McLaughlin, J.C.2
Harper, P.S.3
Lowenstein, P.R.4
Jones, A.L.5
-
87
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the HD gene homologue
-
Zeitlin S., Liu J. P., Chapman D. L., Papaioannou V. E., and Efstratiadis A. (1995) Increased apoptosis and early embryonic lethality in mice nullizygous for the HD gene homologue. Nature Genet. 11, 155-163.
-
(1995)
Nature Genet.
, vol.11
, pp. 155-163
-
-
Zeitlin, S.1
Liu, J.P.2
Chapman, D.L.3
Papaioannou, V.E.4
Efstratiadis, A.5
-
88
-
-
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., and Raymond L. A. (2002) Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 33, 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
-
89
-
-
0035919701
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
-
Zuccato C., Ciammola A., Rigamonti D., et al. (2001) Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293, 493-498.
-
(2001)
Science
, vol.293
, pp. 493-498
-
-
Zuccato, C.1
Ciammola, A.2
Rigamonti, D.3
|