-
1
-
-
33847290297
-
Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes
-
Bowman, A. B., Lam, Y. C., Jafar-Nejad, P., Chen, H. K., Richman, R., Samaco, R. C., Fryer, J. D., Kahle, J. J., Orr, H. T. & Zoghbi, H. Y. (2007). Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes. Nat. Genet, 39, 373-379.
-
(2007)
Nat. Genet
, vol.39
, pp. 373-379
-
-
Bowman, A.B.1
Lam, Y.C.2
Jafar-Nejad, P.3
Chen, H.K.4
Richman, R.5
Samaco, R.C.6
Fryer, J.D.7
Kahle, J.J.8
Orr, H.T.9
Zoghbi, H.Y.10
-
2
-
-
0029163222
-
SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat
-
Burright, E. N., Clark, H. B., Servadio, A., Matilla, T., Feddersen, R. M., Yunis, W. S., Duvick, L. A., Zoghbi, H. Y. & Orr, H. T. (1995). SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell, 82, 937-948.
-
(1995)
Cell
, vol.82
, pp. 937-948
-
-
Burright, E.N.1
Clark, H.B.2
Servadio, A.3
Matilla, T.4
Feddersen, R.M.5
Yunis, W.S.6
Duvick, L.A.7
Zoghbi, H.Y.8
Orr, H.T.9
-
3
-
-
0037726598
-
Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1
-
DOI 10.1016/S0092-8674(03)00349-0
-
Chen, H. K., Fernandez-Funez, P., Acevedo, S. F., Lam, Y. C., Kaytor, M. D., Fernandez, M. H., Aitken, A., Skoulakis, E. M., Orr, H. T., Botas, J. & Zoghbi, H. Y. (2003). Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell, 113, 457-468. (Pubitemid 36588749)
-
(2003)
Cell
, vol.113
, Issue.4
, pp. 457-468
-
-
Chen, H.-K.1
Fernandez-Funez, P.2
Acevedo, S.F.3
Lam, Y.C.4
Kaytor, M.D.5
Fernandez, M.H.6
Aitken, A.7
Skoulakis, E.M.C.8
Orr, H.T.9
Botas, J.10
Zoghbi, H.Y.11
-
4
-
-
0942276360
-
The structure of the AXH domain of spinocerebellar ataxin-1
-
Chen, Y. W., Allen, M. D., Veprintsev, D. B., Lowe, J. & Bycroft, M. (2004). The structure of the AXH domain of spinocerebellar ataxin-1. J. Biol. Chem, 279, 3758-3765.
-
(2004)
J. Biol. Chem
, vol.279
, pp. 3758-3765
-
-
Chen, Y.W.1
Allen, M.D.2
Veprintsev, D.B.3
Lowe, J.4
Bycroft, M.5
-
5
-
-
0142039037
-
The AXH module: An independently folded domain common to ataxin-1 and HBP1
-
DOI 10.1016/S0014-5793(03)00818-4
-
de Chiara, C., Giannini, C., Adinolfi, S., de Boer, J., Guida, S., Ramos, A., Jodice, C., Kioussis, D. & Pastore, A. (2003). The AXH module: an independently folded domain common to ataxin-1 and HBP1. FEBS Lett, 551, 107-112. (Pubitemid 37281336)
-
(2003)
FEBS Letters
, vol.551
, Issue.1-3
, pp. 107-112
-
-
De Chiara, C.1
Giannini, C.2
Adinolfi, S.3
De Boer, J.4
Guida, S.5
Ramos, A.6
Jodice, C.7
Kioussis, D.8
Pastore, A.9
-
6
-
-
0037846441
-
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice
-
DOI 10.1016/S0896-6273(03)00258-7
-
Emamian, E. S., Kaytor, M. D., Duvick, L. A., Zu, T., Tousey, S. K., Zoghbi, H. Y., Clark, H. B. & Orr, H. T. (2003). Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron, 38, 375-387. (Pubitemid 36579141)
-
(2003)
Neuron
, vol.38
, Issue.3
, pp. 375-387
-
-
Emamian, E.S.1
Kaytor, M.D.2
Duvick, L.A.3
Zu, T.4
Tousey, S.K.5
Zoghbi, H.Y.6
Clark, H.B.7
Orr, H.T.8
-
7
-
-
17744393249
-
A cell-based screen for modulators of ataxin-1 phosphorylation
-
Kaytor, M. D., Byam, C. E., Tousey, S. K., Stevens, S. D., Zoghbi, H. Y. & Orr, H. T. (2005). A cell-based screen for modulators of ataxin-1 phosphorylation.Hum. Mol. Genet, 14, 1095-1105.
-
(2005)
Hum. Mol. Genet
, vol.14
, pp. 1095-1105
-
-
Kaytor, M.D.1
Byam, C.E.2
Tousey, S.K.3
Stevens, S.D.4
Zoghbi, H.Y.5
Orr, H.T.6
-
8
-
-
0029045033
-
Stages of embryonic development of the zebrafish
-
Kimmel, C. B., Ballard, W. W., Kimmel, S. R., Ullmann, B. & Schilling, T. F. (1995). Stages of embryonic development of the zebrafish. Dev. Dyn, 203, 253-310.
-
(1995)
Dev. Dyn
, vol.203
, pp. 253-310
-
-
Kimmel, C.B.1
Ballard, W.W.2
Kimmel, S.R.3
Ullmann, B.4
Schilling, T.F.5
-
9
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: Role in polyglutarnine- Induced disease in SCA1 transgenic mice
-
DOI 10.1016/S0092-8674(00)81781-X
-
Klement, I. A., Skinner, P. J., Kaytor, M. D., Yi, H., Hersch, S. M., Clark, H. B., Zoghbi, H. Y. & Orr, H. T. (1998). Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell, 95, 41-53. (Pubitemid 28458023)
-
(1998)
Cell
, vol.95
, Issue.1
, pp. 41-53
-
-
Klement, I.A.1
Skinner, P.J.2
Kaytor, M.D.3
Yi, H.4
Hersch, S.M.5
Clark, H.B.6
Zoghbi, H.Y.7
Orr, H.T.8
-
10
-
-
33845657872
-
ATAXIN-1 Interacts with the Repressor Capicua in Its Native Complex to Cause SCA1 Neuropathology
-
DOI 10.1016/j.cell.2006.11.038, PII S0092867406015431
-
Lam, Y. C., Bowman, A. B., Jafar-Nejad, P., Lim, J., Richman, R., Fryer, J. D., Hyun, E. D., Duvick, L. A., Orr, H. T., Botas, J. & Zoghbi, H. Y. (2006). ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell, 127, 1335-1347. (Pubitemid 44960413)
-
(2006)
Cell
, vol.127
, Issue.7
, pp. 1335-1347
-
-
Lam, Y.C.1
Bowman, A.B.2
Jafar-Nejad, P.3
Lim, J.4
Richman, R.5
Fryer, J.D.6
Hyun, E.D.7
Duvick, L.A.8
Orr, H.T.9
Botas, J.10
Zoghbi, H.Y.11
-
11
-
-
34247186766
-
Animal models of human disease: Zebrafish swim into view
-
Lieschke, G. J. & Currie, P. D. (2007). Animal models of human disease: zebrafish swim into view. Nat. Rev. Genet, 8, 353-367.
-
(2007)
Nat. Rev. Genet
, vol.8
, pp. 353-367
-
-
Lieschke, G.J.1
Currie, P.D.2
-
12
-
-
0032528167
-
Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation
-
Matilla, A., Roberson, E. D., Banfi, S., Morales, J., Armstrong, D. L., Burright, E. N., Orr, H. T., Sweatt, J. D., Zoghbi, H. Y. & Matzuk, M. M. (1998). Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation. J. Neurosci, 18, 5508-5516. (Pubitemid 28311965)
-
(1998)
Journal of Neuroscience
, vol.18
, Issue.14
, pp. 5508-5516
-
-
Matilla, A.1
Roberson, E.D.2
Banfi, S.3
Morales, J.4
Armstrong, D.L.5
Burright, E.N.6
Orr, H.T.7
Sweatt, J.D.8
Zoghbi, H.Y.9
Matzuk, M.M.10
-
13
-
-
27144558034
-
CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo
-
DOI 10.1523/JNEUROSCI.3001-05.2005
-
Miller, V. M., Nelson, R. F., Gouvion, C. M., Williams, A., Rodriguez-Lebron, E., Harper, S. Q., Davidson, B. L., Rebagliati, M. R. & Paulson, H. L. (2005). CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J. Neurosci, 25, 9152-9161. (Pubitemid 41500823)
-
(2005)
Journal of Neuroscience
, vol.25
, Issue.40
, pp. 9152-9161
-
-
Miller, V.M.1
Nelson, R.F.2
Gouvion, C.M.3
Williams, A.4
Rodriguez-Lebron, E.5
Harper, S.Q.6
Davidson, B.L.7
Rebagliati, M.R.8
Paulson, H.L.9
-
14
-
-
0035725202
-
Zonal distribution of Purkinje cells in the zebrafish cerebellum: Analysis by means of a specific monoclonal antibody
-
DOI 10.1007/s004410100421
-
Miyamura, Y. & Nakayasu, H. (2001). Zonal distribution of Purkinje cells in the zebrafish cerebellum: analysis by means of a specific monoclonal antibody. Cell Tissue Res, 305, 299-305. (Pubitemid 34212123)
-
(2001)
Cell and Tissue Research
, vol.305
, Issue.3
, pp. 299-305
-
-
Miyamura, Y.1
Nakayasu, H.2
-
15
-
-
25144468986
-
Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1
-
Mizutani, A., Wang, L., Rajan, H., Vig, P. J., Alaynick, W. A., Thaler, J. P. & Tsai, C. C. (2005). Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1. Embo. J, 24, 3339-3351.
-
(2005)
Embo. J
, vol.24
, pp. 3339-3351
-
-
Mizutani, A.1
Wang, L.2
Rajan, H.3
Vig, P.J.4
Alaynick, W.A.5
Thaler, J.P.6
Tsai, C.C.7
-
16
-
-
0030915681
-
Positionally cloned human disease genes: Patterns of evolutionary conservation and functional motifs
-
DOI 10.1073/pnas.94.11.5831
-
Mushegian, A. R., Bassett, D. E., Jr., Boguski, M. S., Bork, P. & Koonin, E. V. (1997). Positionally cloned human disease genes: patterns of evolutionary conservation and functional motifs. Proc Natl Acad Sci U S A, 94, 5831-5836. (Pubitemid 27241857)
-
(1997)
Proceedings of the National Academy of Sciences of the United States of America
, vol.94
, Issue.11
, pp. 5831-5836
-
-
Mushegian, A.R.1
Bassett Jr., D.E.2
Boguski, M.S.3
Bork, P.4
Koonin, E.V.5
-
17
-
-
0035393427
-
SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein
-
Nakamura, K., Jeong, S. Y., Uchihara, T., Anno, M., Nagashima, K., Nagashima, T., Ikeda, S., Tsuji, S. & Kanazawa, I. (2001). SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum. Mol. Genet, 10, 1441-1448.
-
(2001)
Hum. Mol. Genet
, vol.10
, pp. 1441-1448
-
-
Nakamura, K.1
Jeong, S.Y.2
Uchihara, T.3
Anno, M.4
Nagashima, K.5
Nagashima, T.6
Ikeda, S.7
Tsuji, S.8
Kanazawa, I.9
-
18
-
-
0027164698
-
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
-
Orr, H. T., Chung, M. Y., Banfi, S., Kwiatkowski, T. J., Jr., Servadio, A., Beaudet, A. L., McCall, A. E., Duvick, L. A., Ranum, L. P. & Zoghbi, H. Y. (1993). Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat. Genet, 4, 221-226.
-
(1993)
Nat. Genet
, vol.4
, pp. 221-226
-
-
Orr, H.T.1
Chung, M.Y.2
Banfi, S.3
Kwiatkowski Jr., T.J.4
Servadio, A.5
Beaudet, A.L.6
McCall, A.E.7
Duvick, L.A.8
Ranum, L.P.9
Zoghbi, H.Y.10
-
19
-
-
33750815242
-
RORalpha-Mediated Purkinje Cell Development Determines Disease Severity in Adult SCA1 Mice
-
DOI 10.1016/j.cell.2006.09.036, PII S0092867406012967
-
Serra, H. G., Duvick, L., Zu, T., Carlson, K., Stevens, S., Jorgensen, N., Lysholm, A., Burright, E., Zoghbi, H. Y., Clark, H. B., Andresen, J. M. & Orr, H. T. (2006). RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell, 127, 697-708. (Pubitemid 44716252)
-
(2006)
Cell
, vol.127
, Issue.4
, pp. 697-708
-
-
Serra, H.G.1
Duvick, L.2
Zu, T.3
Carlson, K.4
Stevens, S.5
Jorgensen, N.6
Lysholm, A.7
Burright, E.8
Zoghbi, H.Y.9
Clark, H.B.10
Andresen, J.M.11
Orr, H.T.12
-
20
-
-
0029014180
-
Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals
-
Servadio, A., Koshy, B., Armstrong, D., Antalffy, B., Orr, H. T. & Zoghbi, H. Y. (1995). Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nat. Genet, 10, 94-98.
-
(1995)
Nat. Genet
, vol.10
, pp. 94-98
-
-
Servadio, A.1
Koshy, B.2
Armstrong, D.3
Antalffy, B.4
Orr, H.T.5
Zoghbi, H.Y.6
-
21
-
-
0008740028
-
Clinical aspects of spinocerebellar ataxia
-
R. D. Wells and S. T. Warren (Eds.), San Diego: Academic Press
-
Subramony, S. H. and Vig, P. J. (1998). Clinical aspects of spinocerebellar ataxia. In R. D. Wells and S. T. Warren (Eds.), Genetic instabilities and hereditary neurological diseases (pp. 231-239). San Diego: Academic Press.
-
(1998)
Genetic Instabilities and Hereditary Neurological Diseases
, pp. 231-239
-
-
Subramony, S.H.1
Vig, P.J.2
-
22
-
-
0037351989
-
Genome duplication, a trait shared by 22000 species of ray-finned fish
-
Taylor, J. S., Braasch, I., Frickey, T., Meyer, A. & Van de Peer, Y. (2003). Genome duplication, a trait shared by 22000 species of ray-finned fish. Gen. Res, 13, 382-390.
-
(2003)
Gen. Res
, vol.13
, pp. 382-390
-
-
Taylor, J.S.1
Braasch, I.2
Frickey, T.3
Meyer, A.4
Van De Peer, Y.5
-
23
-
-
1642447764
-
Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors
-
Tsai, C. C., Kao, H. Y., Mitzutani, A., Banayo, E., Rajan, H., McKeown, M. & Evans, R. M. (2004). Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors. Proc. Natl. Acad, Sci. U. S. A., 101, 4047-4052.
-
(2004)
Proc. Natl. Acad, Sci. U. S. A.
, vol.101
, pp. 4047-4052
-
-
Tsai, C.C.1
Kao, H.Y.2
Mitzutani, A.3
Banayo, E.4
Rajan, H.5
McKeown, M.6
Evans, R.M.7
-
24
-
-
23944438950
-
The AXH domain of ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/senseless proteins
-
DOI 10.1016/j.cell.2005.06.012, PII S0092867405005957
-
Tsuda, H., Jafar-Nejad, H., Patel, A. J., Sun, Y., Chen, H. K., Rose, M. F., Venken, K. J., Botas, J., Orr, H. T., Bellen, H. J. & Zoghbi, H. Y. (2005). The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell, 122, 633-644. (Pubitemid 41191160)
-
(2005)
Cell
, vol.122
, Issue.4
, pp. 633-644
-
-
Tsuda, H.1
Jafar-Nejad, H.2
Patel, A.J.3
Sun, Y.4
Chen, H.-K.5
Rose, M.F.6
Venken, K.J.T.7
Botas, J.8
Orr, H.T.9
Bellen, H.J.10
Zoghbi, H.Y.11
-
25
-
-
18844447814
-
Identification of a novel phosphorylation site in ataxin-1
-
Vierra-Green, C. A., Orr, H. T., Zoghbi, H. Y. & Ferrington, D. A. (2005). Identification of a novel phosphorylation site in ataxin-1. Biochim. Biophys. Acta, 1744, 11-18.
-
(2005)
Biochim. Biophys. Acta
, vol.1744
, pp. 11-18
-
-
Vierra-Green, C.A.1
Orr, H.T.2
Zoghbi, H.Y.3
Ferrington, D.A.4
-
27
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi, H. Y. & Orr, H. T. (2000). Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci, 23, 217-247.
-
(2000)
Annu. Rev. Neurosci
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
-
28
-
-
12144273121
-
In vivo drug discovery in the zebrafish
-
Zon, L. I. & Peterson, R. T. (2005). In vivo drug discovery in the zebrafish. Nat. Rev. Drug Discov, 4, 35-44.
-
(2005)
Nat. Rev. Drug Discov
, vol.4
, pp. 35-44
-
-
Zon, L.I.1
Peterson, R.T.2
|