-
1
-
-
84883465132
-
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons
-
Almeida, S., Gascon, E., Tran, H., Chou, H.J., Gendron, T.F., Degroot, S., Tapper, A.R., Sellier, C., Charlet-Berguerand, N., Karydas, A., et al. Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol. 126 (2013), 385–399.
-
(2013)
Acta Neuropathol.
, vol.126
, pp. 385-399
-
-
Almeida, S.1
Gascon, E.2
Tran, H.3
Chou, H.J.4
Gendron, T.F.5
Degroot, S.6
Tapper, A.R.7
Sellier, C.8
Charlet-Berguerand, N.9
Karydas, A.10
-
2
-
-
33750716074
-
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
-
Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., Mann, D., Tsuchiya, K., Yoshida, M., Hashizume, Y., Oda, T., TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 351 (2006), 602–611.
-
(2006)
Biochem. Biophys. Res. Commun.
, vol.351
, pp. 602-611
-
-
Arai, T.1
Hasegawa, M.2
Akiyama, H.3
Ikeda, K.4
Nonaka, T.5
Mori, H.6
Mann, D.7
Tsuchiya, K.8
Yoshida, M.9
Hashizume, Y.10
Oda, T.11
-
3
-
-
84874272095
-
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS
-
Ash, P.E., Bieniek, K.F., Gendron, T.F., Caulfield, T., Lin, W.L., Dejesus-Hernandez, M., van Blitterswijk, M.M., Jansen-West, K., Paul, J.W. 3rd, Rademakers, R., et al. Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron 77 (2013), 639–646.
-
(2013)
Neuron
, vol.77
, pp. 639-646
-
-
Ash, P.E.1
Bieniek, K.F.2
Gendron, T.F.3
Caulfield, T.4
Lin, W.L.5
Dejesus-Hernandez, M.6
van Blitterswijk, M.M.7
Jansen-West, K.8
Paul, J.W.9
Rademakers, R.10
-
4
-
-
33749563294
-
Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1
-
Atkin, J.D., Farg, M.A., Turner, B.J., Tomas, D., Lysaght, J.A., Nunan, J., Rembach, A., Nagley, P., Beart, P.M., Cheema, S.S., Horne, M.K., Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1. J. Biol. Chem. 281 (2006), 30152–30165.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 30152-30165
-
-
Atkin, J.D.1
Farg, M.A.2
Turner, B.J.3
Tomas, D.4
Lysaght, J.A.5
Nunan, J.6
Rembach, A.7
Nagley, P.8
Beart, P.M.9
Cheema, S.S.10
Horne, M.K.11
-
5
-
-
43649100018
-
Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
-
Atkin, J.D., Farg, M.A., Walker, A.K., McLean, C., Tomas, D., Horne, M.K., Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol. Dis. 30 (2008), 400–407.
-
(2008)
Neurobiol. Dis.
, vol.30
, pp. 400-407
-
-
Atkin, J.D.1
Farg, M.A.2
Walker, A.K.3
McLean, C.4
Tomas, D.5
Horne, M.K.6
-
6
-
-
33749056809
-
ALS: a disease of motor neurons and their nonneuronal neighbors
-
Boillée, S., Vande Velde, C., Cleveland, D.W., ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 52 (2006), 39–59.
-
(2006)
Neuron
, vol.52
, pp. 39-59
-
-
Boillée, S.1
Vande Velde, C.2
Cleveland, D.W.3
-
7
-
-
77958519939
-
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
-
Bosco, D.A., Morfini, G., Karabacak, N.M., Song, Y., Gros-Louis, F., Pasinelli, P., Goolsby, H., Fontaine, B.A., Lemay, N., McKenna-Yasek, D., et al. Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat. Neurosci. 13 (2010), 1396–1403.
-
(2010)
Nat. Neurosci.
, vol.13
, pp. 1396-1403
-
-
Bosco, D.A.1
Morfini, G.2
Karabacak, N.M.3
Song, Y.4
Gros-Louis, F.5
Pasinelli, P.6
Goolsby, H.7
Fontaine, B.A.8
Lemay, N.9
McKenna-Yasek, D.10
-
8
-
-
84906314307
-
TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord
-
Brettschneider, J., Arai, K., Del Tredici, K., Toledo, J.B., Robinson, J.L., Lee, E.B., Kuwabara, S., Shibuya, K., Irwin, D.J., Fang, L., et al. TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord. Acta Neuropathol. 128 (2014), 423–437.
-
(2014)
Acta Neuropathol.
, vol.128
, pp. 423-437
-
-
Brettschneider, J.1
Arai, K.2
Del Tredici, K.3
Toledo, J.B.4
Robinson, J.L.5
Lee, E.B.6
Kuwabara, S.7
Shibuya, K.8
Irwin, D.J.9
Fang, L.10
-
9
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
-
Bruijn, L.I., Houseweart, M.K., Kato, S., Anderson, K.L., Anderson, S.D., Ohama, E., Reaume, A.G., Scott, R.W., Cleveland, D.W., Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281 (1998), 1851–1854.
-
(1998)
Science
, vol.281
, pp. 1851-1854
-
-
Bruijn, L.I.1
Houseweart, M.K.2
Kato, S.3
Anderson, K.L.4
Anderson, S.D.5
Ohama, E.6
Reaume, A.G.7
Scott, R.W.8
Cleveland, D.W.9
-
10
-
-
0037011917
-
IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA
-
Calfon, M., Zeng, H., Urano, F., Till, J.H., Hubbard, S.R., Harding, H.P., Clark, S.G., Ron, D., IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA. Nature 415 (2002), 92–96.
-
(2002)
Nature
, vol.415
, pp. 92-96
-
-
Calfon, M.1
Zeng, H.2
Urano, F.3
Till, J.H.4
Hubbard, S.R.5
Harding, H.P.6
Clark, S.G.7
Ron, D.8
-
11
-
-
84930637080
-
Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits
-
Chew, J., Gendron, T.F., Prudencio, M., Sasaguri, H., Zhang, Y.J., Castanedes-Casey, M., Lee, C.W., Jansen-West, K., Kurti, A., Murray, M.E., et al. Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits. Science 348 (2015), 1151–1154.
-
(2015)
Science
, vol.348
, pp. 1151-1154
-
-
Chew, J.1
Gendron, T.F.2
Prudencio, M.3
Sasaguri, H.4
Zhang, Y.J.5
Castanedes-Casey, M.6
Lee, C.W.7
Jansen-West, K.8
Kurti, A.9
Murray, M.E.10
-
12
-
-
84885808774
-
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention
-
Donnelly, C.J., Zhang, P.W., Pham, J.T., Haeusler, A.R., Mistry, N.A., Vidensky, S., Daley, E.L., Poth, E.M., Hoover, B., Fines, D.M., et al. RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron 80 (2013), 415–428.
-
(2013)
Neuron
, vol.80
, pp. 415-428
-
-
Donnelly, C.J.1
Zhang, P.W.2
Pham, J.T.3
Haeusler, A.R.4
Mistry, N.A.5
Vidensky, S.6
Daley, E.L.7
Poth, E.M.8
Hoover, B.9
Fines, D.M.10
-
13
-
-
84940925534
-
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
-
Freibaum, B.D., Lu, Y., Lopez-Gonzalez, R., Kim, N.C., Almeida, S., Lee, K.H., Badders, N., Valentine, M., Miller, B.L., Wong, P.C., et al. GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport. Nature 525 (2015), 129–133.
-
(2015)
Nature
, vol.525
, pp. 129-133
-
-
Freibaum, B.D.1
Lu, Y.2
Lopez-Gonzalez, R.3
Kim, N.C.4
Almeida, S.5
Lee, K.H.6
Badders, N.7
Valentine, M.8
Miller, B.L.9
Wong, P.C.10
-
14
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
-
Gurney, M.E., Pu, H., Chiu, A.Y., Dal Canto, M.C., Polchow, C.Y., Alexander, D.D., Caliendo, J., Hentati, A., Kwon, Y.W., Deng, H.X., et al. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264 (1994), 1772–1775.
-
(1994)
Science
, vol.264
, pp. 1772-1775
-
-
Gurney, M.E.1
Pu, H.2
Chiu, A.Y.3
Dal Canto, M.C.4
Polchow, C.Y.5
Alexander, D.D.6
Caliendo, J.7
Hentati, A.8
Kwon, Y.W.9
Deng, H.X.10
-
15
-
-
84896259966
-
C9orf72 nucleotide repeat structures initiate molecular cascades of disease
-
Haeusler, A.R., Donnelly, C.J., Periz, G., Simko, E.A., Shaw, P.G., Kim, M.S., Maragakis, N.J., Troncoso, J.C., Pandey, A., Sattler, R., et al. C9orf72 nucleotide repeat structures initiate molecular cascades of disease. Nature 507 (2014), 195–200.
-
(2014)
Nature
, vol.507
, pp. 195-200
-
-
Haeusler, A.R.1
Donnelly, C.J.2
Periz, G.3
Simko, E.A.4
Shaw, P.G.5
Kim, M.S.6
Maragakis, N.J.7
Troncoso, J.C.8
Pandey, A.9
Sattler, R.10
-
16
-
-
68049110633
-
IRE1alpha kinase activation modes control alternate endoribonuclease outputs to determine divergent cell fates
-
Han, D., Lerner, A.G., Vande Walle, L., Upton, J.P., Xu, W., Hagen, A., Backes, B.J., Oakes, S.A., Papa, F.R., IRE1alpha kinase activation modes control alternate endoribonuclease outputs to determine divergent cell fates. Cell 138 (2009), 562–575.
-
(2009)
Cell
, vol.138
, pp. 562-575
-
-
Han, D.1
Lerner, A.G.2
Vande Walle, L.3
Upton, J.P.4
Xu, W.5
Hagen, A.6
Backes, B.J.7
Oakes, S.A.8
Papa, F.R.9
-
17
-
-
70349627027
-
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
-
Hetz, C., Thielen, P., Matus, S., Nassif, M., Court, F., Kiffin, R., Martinez, G., Cuervo, A.M., Brown, R.H., Glimcher, L.H., XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. Genes Dev. 23 (2009), 2294–2306.
-
(2009)
Genes Dev.
, vol.23
, pp. 2294-2306
-
-
Hetz, C.1
Thielen, P.2
Matus, S.3
Nassif, M.4
Court, F.5
Kiffin, R.6
Martinez, G.7
Cuervo, A.M.8
Brown, R.H.9
Glimcher, L.H.10
-
18
-
-
0345734277
-
HIPK2 regulates transforming growth factor-beta-induced c-Jun NH(2)-terminal kinase activation and apoptosis in human hepatoma cells
-
Hofmann, T.G., Stollberg, N., Schmitz, M.L., Will, H., HIPK2 regulates transforming growth factor-beta-induced c-Jun NH(2)-terminal kinase activation and apoptosis in human hepatoma cells. Cancer Res. 63 (2003), 8271–8277.
-
(2003)
Cancer Res.
, vol.63
, pp. 8271-8277
-
-
Hofmann, T.G.1
Stollberg, N.2
Schmitz, M.L.3
Will, H.4
-
19
-
-
77955961922
-
Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS
-
Israelson, A., Arbel, N., Da Cruz, S., Ilieva, H., Yamanaka, K., Shoshan-Barmatz, V., Cleveland, D.W., Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron 67 (2010), 575–587.
-
(2010)
Neuron
, vol.67
, pp. 575-587
-
-
Israelson, A.1
Arbel, N.2
Da Cruz, S.3
Ilieva, H.4
Yamanaka, K.5
Shoshan-Barmatz, V.6
Cleveland, D.W.7
-
20
-
-
63849246525
-
Protein structure prediction on the Web: a case study using the Phyre server
-
Kelley, L.A., Sternberg, M.J., Protein structure prediction on the Web: a case study using the Phyre server. Nat. Protoc. 4 (2009), 363–371.
-
(2009)
Nat. Protoc.
, vol.4
, pp. 363-371
-
-
Kelley, L.A.1
Sternberg, M.J.2
-
21
-
-
33645798615
-
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
-
Kikuchi, H., Almer, G., Yamashita, S., Guégan, C., Nagai, M., Xu, Z., Sosunov, A.A., McKhann, G.M. 2nd, Przedborski, S., Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc. Natl. Acad. Sci. USA 103 (2006), 6025–6030.
-
(2006)
Proc. Natl. Acad. Sci. USA
, vol.103
, pp. 6025-6030
-
-
Kikuchi, H.1
Almer, G.2
Yamashita, S.3
Guégan, C.4
Nagai, M.5
Xu, Z.6
Sosunov, A.A.7
McKhann, G.M.8
Przedborski, S.9
-
22
-
-
57049117856
-
Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities
-
Kim, I., Xu, W., Reed, J.C., Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities. Nat. Rev. Drug Discov. 7 (2008), 1013–1030.
-
(2008)
Nat. Rev. Drug Discov.
, vol.7
, pp. 1013-1030
-
-
Kim, I.1
Xu, W.2
Reed, J.C.3
-
23
-
-
84902191691
-
Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1
-
Kiskinis, E., Sandoe, J., Williams, L.A., Boulting, G.L., Moccia, R., Wainger, B.J., Han, S., Peng, T., Thams, S., Mikkilineni, S., et al. Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell 14 (2014), 781–795.
-
(2014)
Cell Stem Cell
, vol.14
, pp. 781-795
-
-
Kiskinis, E.1
Sandoe, J.2
Williams, L.A.3
Boulting, G.L.4
Moccia, R.5
Wainger, B.J.6
Han, S.7
Peng, T.8
Thams, S.9
Mikkilineni, S.10
-
24
-
-
84155167265
-
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
-
Lee, E.B., Lee, V.M., Trojanowski, J.Q., Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration. Nat. Rev. Neurosci. 13 (2012), 38–50.
-
(2012)
Nat. Rev. Neurosci.
, vol.13
, pp. 38-50
-
-
Lee, E.B.1
Lee, V.M.2
Trojanowski, J.Q.3
-
25
-
-
36049049392
-
IRE1 signaling affects cell fate during the unfolded protein response
-
Lin, J.H., Li, H., Yasumura, D., Cohen, H.R., Zhang, C., Panning, B., Shokat, K.M., Lavail, M.M., Walter, P., IRE1 signaling affects cell fate during the unfolded protein response. Science 318 (2007), 944–949.
-
(2007)
Science
, vol.318
, pp. 944-949
-
-
Lin, J.H.1
Li, H.2
Yasumura, D.3
Cohen, H.R.4
Zhang, C.5
Panning, B.6
Shokat, K.M.7
Lavail, M.M.8
Walter, P.9
-
26
-
-
84881490873
-
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
-
Ling, S.C., Polymenidou, M., Cleveland, D.W., Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron 79 (2013), 416–438.
-
(2013)
Neuron
, vol.79
, pp. 416-438
-
-
Ling, S.C.1
Polymenidou, M.2
Cleveland, D.W.3
-
27
-
-
84896697357
-
The neuropathology associated with repeat expansions in the C9ORF72 gene
-
Mackenzie, I.R., Frick, P., Neumann, M., The neuropathology associated with repeat expansions in the C9ORF72 gene. Acta Neuropathol. 127 (2014), 347–357.
-
(2014)
Acta Neuropathol.
, vol.127
, pp. 347-357
-
-
Mackenzie, I.R.1
Frick, P.2
Neumann, M.3
-
28
-
-
79960936435
-
High-throughput kinase profiling: a more efficient approach toward the discovery of new kinase inhibitors
-
Miduturu, C.V., Deng, X., Kwiatkowski, N., Yang, W., Brault, L., Filippakopoulos, P., Chung, E., Yang, Q., Schwaller, J., Knapp, S., et al. High-throughput kinase profiling: a more efficient approach toward the discovery of new kinase inhibitors. Chem. Biol. 18 (2011), 868–879.
-
(2011)
Chem. Biol.
, vol.18
, pp. 868-879
-
-
Miduturu, C.V.1
Deng, X.2
Kwiatkowski, N.3
Yang, W.4
Brault, L.5
Filippakopoulos, P.6
Chung, E.7
Yang, Q.8
Schwaller, J.9
Knapp, S.10
-
29
-
-
84874962380
-
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS
-
Mori, K., Weng, S.M., Arzberger, T., May, S., Rentzsch, K., Kremmer, E., Schmid, B., Kretzschmar, H.A., Cruts, M., Van Broeckhoven, C., et al. The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science 339 (2013), 1335–1338.
-
(2013)
Science
, vol.339
, pp. 1335-1338
-
-
Mori, K.1
Weng, S.M.2
Arzberger, T.3
May, S.4
Rentzsch, K.5
Kremmer, E.6
Schmid, B.7
Kretzschmar, H.A.8
Cruts, M.9
Van Broeckhoven, C.10
-
30
-
-
33749632259
-
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
-
Neumann, M., Sampathu, D.M., Kwong, L.K., Truax, A.C., Micsenyi, M.C., Chou, T.T., Bruce, J., Schuck, T., Grossman, M., Clark, C.M., et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314 (2006), 130–133.
-
(2006)
Science
, vol.314
, pp. 130-133
-
-
Neumann, M.1
Sampathu, D.M.2
Kwong, L.K.3
Truax, A.C.4
Micsenyi, M.C.5
Chou, T.T.6
Bruce, J.7
Schuck, T.8
Grossman, M.9
Clark, C.M.10
-
31
-
-
44849124411
-
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
-
Nishitoh, H., Kadowaki, H., Nagai, A., Maruyama, T., Yokota, T., Fukutomi, H., Noguchi, T., Matsuzawa, A., Takeda, K., Ichijo, H., ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev. 22 (2008), 1451–1464.
-
(2008)
Genes Dev.
, vol.22
, pp. 1451-1464
-
-
Nishitoh, H.1
Kadowaki, H.2
Nagai, A.3
Maruyama, T.4
Yokota, T.5
Fukutomi, H.6
Noguchi, T.7
Matsuzawa, A.8
Takeda, K.9
Ichijo, H.10
-
32
-
-
84921901605
-
The role of endoplasmic reticulum stress in human pathology
-
Oakes, S.A., Papa, F.R., The role of endoplasmic reticulum stress in human pathology. Annu. Rev. Pathol. 10 (2015), 173–194.
-
(2015)
Annu. Rev. Pathol.
, vol.10
, pp. 173-194
-
-
Oakes, S.A.1
Papa, F.R.2
-
33
-
-
33747605320
-
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
-
Pasinelli, P., Brown, R.H., Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat. Rev. Neurosci. 7 (2006), 710–723.
-
(2006)
Nat. Rev. Neurosci.
, vol.7
, pp. 710-723
-
-
Pasinelli, P.1
Brown, R.H.2
-
34
-
-
84896799718
-
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects
-
Qiu, H., Lee, S., Shang, Y., Wang, W.Y., Au, K.F., Kamiya, S., Barmada, S.J., Finkbeiner, S., Lui, H., Carlton, C.E., et al. ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects. J. Clin. Invest. 124 (2014), 981–999.
-
(2014)
J. Clin. Invest.
, vol.124
, pp. 981-999
-
-
Qiu, H.1
Lee, S.2
Shang, Y.3
Wang, W.Y.4
Au, K.F.5
Kamiya, S.6
Barmada, S.J.7
Finkbeiner, S.8
Lui, H.9
Carlton, C.E.10
-
35
-
-
54749148734
-
Transient inhibition of ATM kinase is sufficient to enhance cellular sensitivity to ionizing radiation
-
Rainey, M.D., Charlton, M.E., Stanton, R.V., Kastan, M.B., Transient inhibition of ATM kinase is sufficient to enhance cellular sensitivity to ionizing radiation. Cancer Res. 68 (2008), 7466–7474.
-
(2008)
Cancer Res.
, vol.68
, pp. 7466-7474
-
-
Rainey, M.D.1
Charlton, M.E.2
Stanton, R.V.3
Kastan, M.B.4
-
36
-
-
34250899722
-
Signal integration in the endoplasmic reticulum unfolded protein response
-
Ron, D., Walter, P., Signal integration in the endoplasmic reticulum unfolded protein response. Nat. Rev. Mol. Cell Biol. 8 (2007), 519–529.
-
(2007)
Nat. Rev. Mol. Cell Biol.
, vol.8
, pp. 519-529
-
-
Ron, D.1
Walter, P.2
-
37
-
-
67349164383
-
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
-
Saxena, S., Cabuy, E., Caroni, P., A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat. Neurosci. 12 (2009), 627–636.
-
(2009)
Nat. Neurosci.
, vol.12
, pp. 627-636
-
-
Saxena, S.1
Cabuy, E.2
Caroni, P.3
-
38
-
-
84876961787
-
Transcriptional corepressors HIPK1 and HIPK2 control angiogenesis via TGF-β-TAK1-dependent mechanism
-
Shang, Y., Doan, C.N., Arnold, T.D., Lee, S., Tang, A.A., Reichardt, L.F., Huang, E.J., Transcriptional corepressors HIPK1 and HIPK2 control angiogenesis via TGF-β-TAK1-dependent mechanism. PLoS Biol., 11, 2013, e1001527.
-
(2013)
PLoS Biol.
, vol.11
, pp. e1001527
-
-
Shang, Y.1
Doan, C.N.2
Arnold, T.D.3
Lee, S.4
Tang, A.A.5
Reichardt, L.F.6
Huang, E.J.7
-
39
-
-
58549088349
-
ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB
-
Suzuki, H., Kanekura, K., Levine, T.P., Kohno, K., Olkkonen, V.M., Aiso, S., Matsuoka, M., ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB. J. Neurochem. 108 (2009), 973–985.
-
(2009)
J. Neurochem.
, vol.108
, pp. 973-985
-
-
Suzuki, H.1
Kanekura, K.2
Levine, T.P.3
Kohno, K.4
Olkkonen, V.M.5
Aiso, S.6
Matsuoka, M.7
-
40
-
-
80052081196
-
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis
-
Walker, A.K., Atkin, J.D., Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis. IUBMB Life 63 (2011), 754–763.
-
(2011)
IUBMB Life
, vol.63
, pp. 754-763
-
-
Walker, A.K.1
Atkin, J.D.2
-
41
-
-
84896710448
-
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation
-
Walker, A.K., Soo, K.Y., Sundaramoorthy, V., Parakh, S., Ma, Y., Farg, M.A., Wallace, R.H., Crouch, P.J., Turner, B.J., Horne, M.K., Atkin, J.D., ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation. PLoS ONE, 8, 2013, e81170.
-
(2013)
PLoS ONE
, vol.8
, pp. e81170
-
-
Walker, A.K.1
Soo, K.Y.2
Sundaramoorthy, V.3
Parakh, S.4
Ma, Y.5
Farg, M.A.6
Wallace, R.H.7
Crouch, P.J.8
Turner, B.J.9
Horne, M.K.10
Atkin, J.D.11
-
42
-
-
84945450480
-
Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43
-
Walker, A.K., Spiller, K.J., Ge, G., Zheng, A., Xu, Y., Zhou, M., Tripathy, K., Kwong, L.K., Trojanowski, J.Q., Lee, V.M., Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43. Acta Neuropathol. 130 (2015), 643–660.
-
(2015)
Acta Neuropathol.
, vol.130
, pp. 643-660
-
-
Walker, A.K.1
Spiller, K.J.2
Ge, G.3
Zheng, A.4
Xu, Y.5
Zhou, M.6
Tripathy, K.7
Kwong, L.K.8
Trojanowski, J.Q.9
Lee, V.M.10
-
43
-
-
79551584057
-
The unfolded protein response in familial amyotrophic lateral sclerosis
-
Wang, L., Popko, B., Roos, R.P., The unfolded protein response in familial amyotrophic lateral sclerosis. Hum. Mol. Genet. 20 (2011), 1008–1015.
-
(2011)
Hum. Mol. Genet.
, vol.20
, pp. 1008-1015
-
-
Wang, L.1
Popko, B.2
Roos, R.P.3
-
44
-
-
7244223245
-
Interaction of Brn3a and HIPK2 mediates transcriptional repression of sensory neuron survival
-
Wiggins, A.K., Wei, G., Doxakis, E., Wong, C., Tang, A.A., Zang, K., Luo, E.J., Neve, R.L., Reichardt, L.F., Huang, E.J., Interaction of Brn3a and HIPK2 mediates transcriptional repression of sensory neuron survival. J. Cell Biol. 167 (2004), 257–267.
-
(2004)
J. Cell Biol.
, vol.167
, pp. 257-267
-
-
Wiggins, A.K.1
Wei, G.2
Doxakis, E.3
Wong, C.4
Tang, A.A.5
Zang, K.6
Luo, E.J.7
Neve, R.L.8
Reichardt, L.F.9
Huang, E.J.10
-
45
-
-
0035966269
-
XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor
-
Yoshida, H., Matsui, T., Yamamoto, A., Okada, T., Mori, K., XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor. Cell 107 (2001), 881–891.
-
(2001)
Cell
, vol.107
, pp. 881-891
-
-
Yoshida, H.1
Matsui, T.2
Yamamoto, A.3
Okada, T.4
Mori, K.5
-
46
-
-
33845889727
-
Essential function of HIPK2 in TGFbeta-dependent survival of midbrain dopamine neurons
-
Zhang, J., Pho, V., Bonasera, S.J., Holtzman, J., Tang, A.T., Hellmuth, J., Tang, S., Janak, P.H., Tecott, L.H., Huang, E.J., Essential function of HIPK2 in TGFbeta-dependent survival of midbrain dopamine neurons. Nat. Neurosci. 10 (2007), 77–86.
-
(2007)
Nat. Neurosci.
, vol.10
, pp. 77-86
-
-
Zhang, J.1
Pho, V.2
Bonasera, S.J.3
Holtzman, J.4
Tang, A.T.5
Hellmuth, J.6
Tang, S.7
Janak, P.H.8
Tecott, L.H.9
Huang, E.J.10
-
47
-
-
84919912448
-
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress
-
Zhang, Y.J., Jansen-West, K., Xu, Y.F., Gendron, T.F., Bieniek, K.F., Lin, W.L., Sasaguri, H., Caulfield, T., Hubbard, J., Daughrity, L., et al. Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol. 128 (2014), 505–524.
-
(2014)
Acta Neuropathol.
, vol.128
, pp. 505-524
-
-
Zhang, Y.J.1
Jansen-West, K.2
Xu, Y.F.3
Gendron, T.F.4
Bieniek, K.F.5
Lin, W.L.6
Sasaguri, H.7
Caulfield, T.8
Hubbard, J.9
Daughrity, L.10
-
48
-
-
84940923271
-
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport
-
Zhang, K., Donnelly, C.J., Haeusler, A.R., Grima, J.C., Machamer, J.B., Steinwald, P., Daley, E.L., Miller, S.J., Cunningham, K.M., Vidensky, S., et al. The C9orf72 repeat expansion disrupts nucleocytoplasmic transport. Nature 525 (2015), 56–61.
-
(2015)
Nature
, vol.525
, pp. 56-61
-
-
Zhang, K.1
Donnelly, C.J.2
Haeusler, A.R.3
Grima, J.C.4
Machamer, J.B.5
Steinwald, P.6
Daley, E.L.7
Miller, S.J.8
Cunningham, K.M.9
Vidensky, S.10
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