-
1
-
-
84954535655
-
A missense mutation in PPP1R15B causes a syndrome including diabetes, short stature, and microcephaly
-
Abdulkarim, B., et al. A missense mutation in PPP1R15B causes a syndrome including diabetes, short stature, and microcephaly. Diabetes 64 (2015), 3951–3962.
-
(2015)
Diabetes
, vol.64
, pp. 3951-3962
-
-
Abdulkarim, B.1
-
2
-
-
84878333056
-
Tau accumulation activates the unfolded protein response by impairing endoplasmic reticulum-associated degradation
-
Abisambra, J.F., et al. Tau accumulation activates the unfolded protein response by impairing endoplasmic reticulum-associated degradation. J. Neurosci. 33 (2013), 9498–9507.
-
(2013)
J. Neurosci.
, vol.33
, pp. 9498-9507
-
-
Abisambra, J.F.1
-
3
-
-
84941954604
-
A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits
-
Aharony, I., et al. A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits. Hum. Mol. Genet. 24 (2015), 2604–2614.
-
(2015)
Hum. Mol. Genet.
, vol.24
, pp. 2604-2614
-
-
Aharony, I.1
-
4
-
-
84155163741
-
A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis
-
Al-Saif, A., Al-Mohanna, F., Bohlega, S., A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis. Ann. Neurol. 70 (2011), 913–919.
-
(2011)
Ann. Neurol.
, vol.70
, pp. 913-919
-
-
Al-Saif, A.1
Al-Mohanna, F.2
Bohlega, S.3
-
5
-
-
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., et al. 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
-
6
-
-
85056070270
-
Rapamycin improves motor function, reduces 4-hydroxynonenal adducted protein in brain, and attenuates synaptic injury in a mouse model of synucleinopathy
-
Bai, X., et al. Rapamycin improves motor function, reduces 4-hydroxynonenal adducted protein in brain, and attenuates synaptic injury in a mouse model of synucleinopathy. Pathobiol. Aging Age Relat. Dis., 5, 2015, 28743.
-
(2015)
Pathobiol. Aging Age Relat. Dis.
, vol.5
, pp. 28743
-
-
Bai, X.1
-
7
-
-
39349083915
-
Adapting proteostasis for disease intervention
-
Balch, W.E., et al. Adapting proteostasis for disease intervention. Science 319 (2008), 916–919.
-
(2008)
Science
, vol.319
, pp. 916-919
-
-
Balch, W.E.1
-
8
-
-
33748561495
-
Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers
-
Behrends, C., et al. Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. Mol. Cell 23 (2006), 887–897.
-
(2006)
Mol. Cell
, vol.23
, pp. 887-897
-
-
Behrends, C.1
-
9
-
-
79551647443
-
Induction of the unfolded protein response by alpha-synuclein in experimental models of Parkinson's disease
-
Bellucci, A., et al. Induction of the unfolded protein response by alpha-synuclein in experimental models of Parkinson's disease. J. Neurochem. 116 (2011), 588–605.
-
(2011)
J. Neurochem.
, vol.116
, pp. 588-605
-
-
Bellucci, A.1
-
10
-
-
34547807613
-
Global changes to the ubiquitin system in Huntington's disease
-
Bennett, E.J., et al. Global changes to the ubiquitin system in Huntington's disease. Nature 448 (2007), 704–708.
-
(2007)
Nature
, vol.448
, pp. 704-708
-
-
Bennett, E.J.1
-
11
-
-
84920946243
-
Mammalian ER mannosidase I resides in quality control vesicles, where it encounters its glycoprotein substrates
-
Benyair, R., et al. Mammalian ER mannosidase I resides in quality control vesicles, where it encounters its glycoprotein substrates. Mol. Biol. Cell 26 (2015), 172–184.
-
(2015)
Mol. Biol. Cell
, vol.26
, pp. 172-184
-
-
Benyair, R.1
-
12
-
-
80855139444
-
Protein quality control, retention, and degradation at the endoplasmic reticulum
-
Benyair, R., Ron, E., Lederkremer, G.Z., Protein quality control, retention, and degradation at the endoplasmic reticulum. Int. Rev. Cell Mol. Biol. 292 (2011), 197–280.
-
(2011)
Int. Rev. Cell Mol. Biol.
, vol.292
, pp. 197-280
-
-
Benyair, R.1
Ron, E.2
Lederkremer, G.Z.3
-
13
-
-
84931571933
-
Glycan regulation of ER-associated degradation through compartmentalization
-
Benyair, R., Ogen-Shtern, N., Lederkremer, G.Z., Glycan regulation of ER-associated degradation through compartmentalization. Semin. Cell Dev. Biol. 41 (2015), 99–109.
-
(2015)
Semin. Cell Dev. Biol.
, vol.41
, pp. 99-109
-
-
Benyair, R.1
Ogen-Shtern, N.2
Lederkremer, G.Z.3
-
14
-
-
84890954073
-
Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial
-
Berk, J.L., et al. Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial. JAMA 310 (2013), 2658–2667.
-
(2013)
JAMA
, vol.310
, pp. 2658-2667
-
-
Berk, J.L.1
-
15
-
-
84929069085
-
Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration
-
Bernard-Marissal, N., et al. Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration. Brain 138 (2015), 875–890.
-
(2015)
Brain
, vol.138
, pp. 875-890
-
-
Bernard-Marissal, N.1
-
16
-
-
65249181587
-
The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease
-
Bett, J.S., et al. The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS One, 4, 2009, e5128.
-
(2009)
PLoS One
, vol.4
, pp. e5128
-
-
Bett, J.S.1
-
17
-
-
0036307333
-
Loss of kinase activity in a patient with Wolcott-Rallison syndrome caused by a novel mutation in the EIF2AK3 gene
-
Biason-Lauber, A., et al. Loss of kinase activity in a patient with Wolcott-Rallison syndrome caused by a novel mutation in the EIF2AK3 gene. Diabetes 51 (2002), 2301–2305.
-
(2002)
Diabetes
, vol.51
, pp. 2301-2305
-
-
Biason-Lauber, A.1
-
18
-
-
84864742403
-
Proteostasis and movement disorders: Parkinson's disease and amyotrophic lateral sclerosis
-
Bosco, D.A., et al. Proteostasis and movement disorders: Parkinson's disease and amyotrophic lateral sclerosis. Cold Spring Harb. Perspect. Biol., 3, 2011, a007500.
-
(2011)
Cold Spring Harb. Perspect. Biol.
, vol.3
, pp. a007500
-
-
Bosco, D.A.1
-
19
-
-
84870733168
-
Protein folding in the endoplasmic reticulum
-
Braakman, I., Hebert, D.N., Protein folding in the endoplasmic reticulum. Cold Spring Harb. Perspect. Biol., 5, 2013, a013201.
-
(2013)
Cold Spring Harb. Perspect. Biol.
, vol.5
, pp. a013201
-
-
Braakman, I.1
Hebert, D.N.2
-
20
-
-
83455202793
-
Alpha-synuclein misfolding and Parkinson's disease
-
Breydo, L., Wu, J.W., Uversky, V.N., Alpha-synuclein misfolding and Parkinson's disease. Biochim. Biophys. Acta 1822 (2012), 261–285.
-
(2012)
Biochim. Biophys. Acta
, vol.1822
, pp. 261-285
-
-
Breydo, L.1
Wu, J.W.2
Uversky, V.N.3
-
21
-
-
84870907436
-
Cleaning Up: ER-Associated Degradation to the Rescue
-
Brodsky, J.L., Cleaning Up: ER-Associated Degradation to the Rescue. Cell 151 (2012), 1163–1167.
-
(2012)
Cell
, vol.151
, pp. 1163-1167
-
-
Brodsky, J.L.1
-
22
-
-
0034652248
-
Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency
-
Burrows, J.A., Willis, L.K., Perlmutter, D.H., Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency. Proc. Natl. Acad. Sci. USA 97 (2000), 1796–1801.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 1796-1801
-
-
Burrows, J.A.1
Willis, L.K.2
Perlmutter, D.H.3
-
23
-
-
44849102178
-
Getting in and out from calnexin/calreticulin cycles
-
Caramelo, J.J., Parodi, A.J., Getting in and out from calnexin/calreticulin cycles. J. Biol. Chem. 283 (2008), 10221–10225.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 10221-10225
-
-
Caramelo, J.J.1
Parodi, A.J.2
-
24
-
-
84946491193
-
A sweet code for glycoprotein folding
-
Caramelo, J.J., Parodi, A.J., A sweet code for glycoprotein folding. FEBS Lett. 589 (2015), 3379–3387.
-
(2015)
FEBS Lett.
, vol.589
, pp. 3379-3387
-
-
Caramelo, J.J.1
Parodi, A.J.2
-
25
-
-
67650566385
-
Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease
-
Carnemolla, A., et al. Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease. J. Biol. Chem. 284 (2009), 18167–18173.
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 18167-18173
-
-
Carnemolla, A.1
-
26
-
-
79956036398
-
The ER stress factor XBP1s prevents amyloid-β neurotoxicity
-
Casas-Tinto, S., et al. The ER stress factor XBP1s prevents amyloid-β neurotoxicity. Hum. Mol. Genet. 20 (2011), 2144–2160.
-
(2011)
Hum. Mol. Genet.
, vol.20
, pp. 2144-2160
-
-
Casas-Tinto, S.1
-
27
-
-
84871870648
-
Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation
-
Chiang, W.C., et al. Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation. Investig. Ophthalmol. Vis. Sci. 53 (2012), 7159–7166.
-
(2012)
Investig. Ophthalmol. Vis. Sci.
, vol.53
, pp. 7159-7166
-
-
Chiang, W.C.1
-
28
-
-
76249091683
-
Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease
-
Cho, K.J., et al. Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease. Neuroscience 163 (2009), 1128–1134.
-
(2009)
Neuroscience
, vol.163
, pp. 1128-1134
-
-
Cho, K.J.1
-
29
-
-
16644379264
-
Natural oligomers of the amyloid-beta protein specifically disrupt cognitive function
-
Cleary, J.P., et al. Natural oligomers of the amyloid-beta protein specifically disrupt cognitive function. Nat. Neurosci. 8 (2005), 79–84.
-
(2005)
Nat. Neurosci.
, vol.8
, pp. 79-84
-
-
Cleary, J.P.1
-
30
-
-
84863230467
-
Endoplasmic reticulum stress is important for the manifestations of alpha-synucleinopathy in vivo
-
Colla, E., et al. Endoplasmic reticulum stress is important for the manifestations of alpha-synucleinopathy in vivo. J. Neurosci. 32 (2012), 3306–3320.
-
(2012)
J. Neurosci.
, vol.32
, pp. 3306-3320
-
-
Colla, E.1
-
31
-
-
84863229691
-
Accumulation of toxic alpha-synuclein oligomer within endoplasmic reticulum occurs in alpha-synucleinopathy in vivo
-
Colla, E., et al. Accumulation of toxic alpha-synuclein oligomer within endoplasmic reticulum occurs in alpha-synucleinopathy in vivo. J. Neurosci. 32 (2012), 3301–3305.
-
(2012)
J. Neurosci.
, vol.32
, pp. 3301-3305
-
-
Colla, E.1
-
32
-
-
0030866540
-
Human prion diseases and bovine spongiform encephalopathy (BSE)
-
Collinge, J., Human prion diseases and bovine spongiform encephalopathy (BSE). Hum. Mol. Genet. 6 (1997), 1699–1705.
-
(1997)
Hum. Mol. Genet.
, vol.6
, pp. 1699-1705
-
-
Collinge, J.1
-
33
-
-
84906993311
-
Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain
-
Cooley, C.B., et al. Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain. Proc. Natl. Acad. Sci. USA 111 (2014), 13046–13051.
-
(2014)
Proc. Natl. Acad. Sci. USA
, vol.111
, pp. 13046-13051
-
-
Cooley, C.B.1
-
34
-
-
33947719749
-
eIF2alpha phosphorylation bidirectionally regulates the switch from short- to long-term synaptic plasticity and memory
-
Costa-Mattioli, M., et al. eIF2alpha phosphorylation bidirectionally regulates the switch from short- to long-term synaptic plasticity and memory. Cell 129 (2007), 195–206.
-
(2007)
Cell
, vol.129
, pp. 195-206
-
-
Costa-Mattioli, M.1
-
35
-
-
84923871028
-
alpha-Synuclein-mediated inhibition of ATF6 processing into COPII vesicles disrupts UPR signaling in Parkinson's disease
-
Credle, J.J., et al. alpha-Synuclein-mediated inhibition of ATF6 processing into COPII vesicles disrupts UPR signaling in Parkinson's disease. Neurobiol. Dis. 76 (2015), 112–125.
-
(2015)
Neurobiol. Dis.
, vol.76
, pp. 112-125
-
-
Credle, J.J.1
-
36
-
-
84927619395
-
Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit
-
Das, I., et al. Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science 348 (2015), 239–242.
-
(2015)
Science
, vol.348
, pp. 239-242
-
-
Das, I.1
-
37
-
-
0034425698
-
EIF2AK3, encoding translation initiation factor 2-alpha kinase 3, is mutated in patients with Wolcott-Rallison syndrome
-
Delepine, M., et al. EIF2AK3, encoding translation initiation factor 2-alpha kinase 3, is mutated in patients with Wolcott-Rallison syndrome. Nat. Genet. 25 (2000), 406–409.
-
(2000)
Nat. Genet.
, vol.25
, pp. 406-409
-
-
Delepine, M.1
-
38
-
-
0037457966
-
Chaperones increase association of tau protein with microtubules
-
Dou, F., et al. Chaperones increase association of tau protein with microtubules. Proc. Natl. Acad. Sci. 100 (2003), 721–726.
-
(2003)
Proc. Natl. Acad. Sci.
, vol.100
, pp. 721-726
-
-
Dou, F.1
-
39
-
-
57749116408
-
Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity
-
Duennwald, M.L., Lindquist, S., Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity. Genes. Dev. 22 (2008), 3308–3319.
-
(2008)
Genes. Dev.
, vol.22
, pp. 3308-3319
-
-
Duennwald, M.L.1
Lindquist, S.2
-
40
-
-
84858374665
-
The amyloid state of proteins in human diseases
-
Eisenberg, D., Jucker, M., The amyloid state of proteins in human diseases. Cell 148 (2012), 1188–1203.
-
(2012)
Cell
, vol.148
, pp. 1188-1203
-
-
Eisenberg, D.1
Jucker, M.2
-
41
-
-
0037336295
-
Quality control in the endoplasmic reticulum
-
Ellgaard, L., Helenius, A., Quality control in the endoplasmic reticulum. Nat. Rev. Mol. Cell Biol. 4 (2003), 181–191.
-
(2003)
Nat. Rev. Mol. Cell Biol.
, vol.4
, pp. 181-191
-
-
Ellgaard, L.1
Helenius, A.2
-
42
-
-
84923233765
-
Legal but lethal: functional protein aggregation at the verge of toxicity
-
Falsone, A., Falsone, S.F., Legal but lethal: functional protein aggregation at the verge of toxicity. Front. Cell. Neurosci., 9, 2015, 45.
-
(2015)
Front. Cell. Neurosci.
, vol.9
, pp. 45
-
-
Falsone, A.1
Falsone, S.F.2
-
43
-
-
84961288468
-
Mitochondrial function in neuronal cells depends on p97/VCP/Cdc48-mediated quality control
-
Fang, L., et al. Mitochondrial function in neuronal cells depends on p97/VCP/Cdc48-mediated quality control. Front. Cell. Neurosci., 9, 2015, 16.
-
(2015)
Front. Cell. Neurosci.
, vol.9
, pp. 16
-
-
Fang, L.1
-
44
-
-
0035807839
-
The tumor autocrine motility factor receptor, gp78, is a ubiquitin protein ligase implicated in degradation from the endoplasmic reticulum
-
Fang, S., et al. The tumor autocrine motility factor receptor, gp78, is a ubiquitin protein ligase implicated in degradation from the endoplasmic reticulum. Proc. Natl. Acad. Sci. USA 98 (2001), 14422–14427.
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 14422-14427
-
-
Fang, S.1
-
45
-
-
84940185085
-
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions
-
Farrawell, N.E., et al. Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions. Sci. Rep., 5, 2015, 13416.
-
(2015)
Sci. Rep.
, vol.5
, pp. 13416
-
-
Farrawell, N.E.1
-
46
-
-
0142157600
-
Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice
-
Ferrante, R.J., et al. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. J. Neurosci. 23 (2003), 9418–9427.
-
(2003)
J. Neurosci.
, vol.23
, pp. 9418-9427
-
-
Ferrante, R.J.1
-
47
-
-
33747195017
-
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity
-
Ferreiro, E., et al. An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity. Neurobiol. Dis. 23 (2006), 669–678.
-
(2006)
Neurobiol. Dis.
, vol.23
, pp. 669-678
-
-
Ferreiro, E.1
-
49
-
-
43449129732
-
The ubiquitin-proteasome pathway in Huntington's disease
-
Finkbeiner, S., Mitra, S., The ubiquitin-proteasome pathway in Huntington's disease. Sci. World J. 8 (2008), 421–433.
-
(2008)
Sci. World J.
, vol.8
, pp. 421-433
-
-
Finkbeiner, S.1
Mitra, S.2
-
50
-
-
0041315902
-
Polyubiquitin serves as a recognition signal, rather than a ratcheting molecule, during retrotranslocation of proteins across the endoplasmic reticulum membrane
-
Flierman, D., et al. Polyubiquitin serves as a recognition signal, rather than a ratcheting molecule, during retrotranslocation of proteins across the endoplasmic reticulum membrane. J. Biol. Chem. 278 (2003), 34774–34782.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 34774-34782
-
-
Flierman, D.1
-
51
-
-
84896111540
-
Loss of proteostasis induced by amyloid beta peptide in brain endothelial cells
-
Fonseca, A.C., et al. Loss of proteostasis induced by amyloid beta peptide in brain endothelial cells. Biochim. Biophys. Acta 1843 (2014), 1150–1161.
-
(2014)
Biochim. Biophys. Acta
, vol.1843
, pp. 1150-1161
-
-
Fonseca, A.C.1
-
52
-
-
84939989763
-
Cellular factors modulating the mechanism of tau protein aggregation
-
Fontaine, S.N., et al. Cellular factors modulating the mechanism of tau protein aggregation. Cell. Mol. Life Sci. 72 (2015), 1863–1879.
-
(2015)
Cell. Mol. Life Sci.
, vol.72
, pp. 1863-1879
-
-
Fontaine, S.N.1
-
53
-
-
84862303631
-
ER stress inhibits neuronal death by promoting autophagy
-
Fouillet, A., et al. ER stress inhibits neuronal death by promoting autophagy. Autophagy 8 (2012), 915–926.
-
(2012)
Autophagy
, vol.8
, pp. 915-926
-
-
Fouillet, A.1
-
54
-
-
84903529679
-
Pharmacological stimulation of sigma-1 receptors has neurorestorative effects in experimental parkinsonism
-
Francardo, V., et al. Pharmacological stimulation of sigma-1 receptors has neurorestorative effects in experimental parkinsonism. Brain 137 (2014), 1998–2014.
-
(2014)
Brain
, vol.137
, pp. 1998-2014
-
-
Francardo, V.1
-
55
-
-
84870735655
-
Endoplasmic reticulum stress sensing in the unfolded protein response
-
Gardner, B.M., et al. Endoplasmic reticulum stress sensing in the unfolded protein response. Cold Spring Harb. Perspect. Biol., 5, 2013, a013169.
-
(2013)
Cold Spring Harb. Perspect. Biol.
, vol.5
, pp. a013169
-
-
Gardner, B.M.1
-
56
-
-
84954204422
-
Regulating extracellular proteostasis capacity through the unfolded protein response
-
Genereux, J.C., Wiseman, R.L., Regulating extracellular proteostasis capacity through the unfolded protein response. Prion 9 (2015), 10–21.
-
(2015)
Prion
, vol.9
, pp. 10-21
-
-
Genereux, J.C.1
Wiseman, R.L.2
-
57
-
-
84922460332
-
Delivering a disease-modifying treatment for Huntington's disease
-
Godinho, B.M.D.C., et al. Delivering a disease-modifying treatment for Huntington's disease. Drug. Discov. Today 20 (2015), 50–64.
-
(2015)
Drug. Discov. Today
, vol.20
, pp. 50-64
-
-
Godinho, B.M.D.C.1
-
58
-
-
84927566116
-
A novel mutation in VCP causes Charcot-Marie-Tooth Type 2 disease
-
Gonzalez, M.A., et al. A novel mutation in VCP causes Charcot-Marie-Tooth Type 2 disease. Brain 137 (2014), 2897–2902.
-
(2014)
Brain
, vol.137
, pp. 2897-2902
-
-
Gonzalez, M.A.1
-
59
-
-
84863498486
-
Glucose regulated protein 78 diminishes alpha-synuclein neurotoxicity in a rat model of Parkinson disease
-
Gorbatyuk, M.S., et al. Glucose regulated protein 78 diminishes alpha-synuclein neurotoxicity in a rat model of Parkinson disease. Mol. Ther. 20 (2012), 1327–1337.
-
(2012)
Mol. Ther.
, vol.20
, pp. 1327-1337
-
-
Gorbatyuk, M.S.1
-
60
-
-
0037073748
-
Golgi fragmentation occurs in the cells with prefibrillar alpha-synuclein aggregates and precedes the formation of fibrillar inclusion
-
Gosavi, N., et al. Golgi fragmentation occurs in the cells with prefibrillar alpha-synuclein aggregates and precedes the formation of fibrillar inclusion. J. Biol. Chem. 277 (2002), 48984–48992.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 48984-48992
-
-
Gosavi, N.1
-
61
-
-
84945156205
-
The need for thorough phase II
-
Gray, J.A., Fleet, D., Winblad, B., The need for thorough phase II. Stud. Med. Dev. Alzheimer's Dis. Alzheimers Res. Ther., 7, 2015, 67.
-
(2015)
Stud. Med. Dev. Alzheimer's Dis. Alzheimers Res. Ther.
, vol.7
, pp. 67
-
-
Gray, J.A.1
Fleet, D.2
Winblad, B.3
-
62
-
-
0028979993
-
Amyloid beta-protein inhibits ubiquitin-dependent protein degradation in vitro
-
Gregori, L., et al. Amyloid beta-protein inhibits ubiquitin-dependent protein degradation in vitro. J. Biol. Chem. 270 (1995), 19702–19708.
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 19702-19708
-
-
Gregori, L.1
-
63
-
-
78651394090
-
Mannose trimming is required for delivery of a glycoprotein from EDEM1 to XTP3-B and to late endoplasmic reticulum-associated degradation steps
-
Groisman, B., et al. Mannose trimming is required for delivery of a glycoprotein from EDEM1 to XTP3-B and to late endoplasmic reticulum-associated degradation steps. J. Biol. Chem. 286 (2011), 1292–1300.
-
(2011)
J. Biol. Chem.
, vol.286
, pp. 1292-1300
-
-
Groisman, B.1
-
64
-
-
84941139665
-
HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons
-
Guedes-Dias, P., et al. HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons. Biochim. Biophys. Acta 1852 (2015), 2484–2493.
-
(2015)
Biochim. Biophys. Acta
, vol.1852
, pp. 2484-2493
-
-
Guedes-Dias, P.1
-
65
-
-
0034329159
-
Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease
-
Gusella, J.F., MacDonald, M.E., Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat. Rev. Neurosci. 1 (2000), 109–115.
-
(2000)
Nat. Rev. Neurosci.
, vol.1
, pp. 109-115
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
66
-
-
66249137770
-
Sigma receptors suppress multiple aspects of microglial activation
-
Hall, A.A., et al. Sigma receptors suppress multiple aspects of microglial activation. Glia 57 (2009), 744–754.
-
(2009)
Glia
, vol.57
, pp. 744-754
-
-
Hall, A.A.1
-
67
-
-
84988811921
-
Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity
-
Halliday, M., et al. Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity. Cell Death Dis., 6, 2015, e1672.
-
(2015)
Cell Death Dis.
, vol.6
, pp. e1672
-
-
Halliday, M.1
-
68
-
-
0028076031
-
Folding of VSV G protein: sequential interaction with BiP and calnexin
-
Hammond, C., Helenius, A., Folding of VSV G protein: sequential interaction with BiP and calnexin. Science 266 (1994), 456–458.
-
(1994)
Science
, vol.266
, pp. 456-458
-
-
Hammond, C.1
Helenius, A.2
-
69
-
-
36849026845
-
A kinase inhibitor activates the IRE1alpha RNase to confer cytoprotection against ER stress
-
Han, D., et al. A kinase inhibitor activates the IRE1alpha RNase to confer cytoprotection against ER stress. Biochem. Biophys. Res. Commun. 365 (2008), 777–783.
-
(2008)
Biochem. Biophys. Res. Commun.
, vol.365
, pp. 777-783
-
-
Han, D.1
-
70
-
-
0036437307
-
Transcriptional and translational control in the Mammalian unfolded protein response
-
Harding, H.P., et al. Transcriptional and translational control in the Mammalian unfolded protein response. Annu. Rev. Cell Dev. Biol. 18 (2002), 575–599.
-
(2002)
Annu. Rev. Cell Dev. Biol.
, vol.18
, pp. 575-599
-
-
Harding, H.P.1
-
71
-
-
33749177143
-
A hundred years of Alzheimer's disease research
-
Hardy, J., A hundred years of Alzheimer's disease research. Neuron 52 (2006), 3–13.
-
(2006)
Neuron
, vol.52
, pp. 3-13
-
-
Hardy, J.1
-
72
-
-
84870937818
-
Sigma-1 receptor chaperone and brain-derived neurotrophic factor: emerging links between cardiovascular disease and depression
-
Hashimoto, K., Sigma-1 receptor chaperone and brain-derived neurotrophic factor: emerging links between cardiovascular disease and depression. Prog. Neurobiol. 100 (2013), 15–29.
-
(2013)
Prog. Neurobiol.
, vol.100
, pp. 15-29
-
-
Hashimoto, K.1
-
73
-
-
20544440605
-
TEB4 is a C4HC3 RING finger-containing ubiquitin ligase of the endoplasmic reticulum
-
Hassink, G., et al. TEB4 is a C4HC3 RING finger-containing ubiquitin ligase of the endoplasmic reticulum. Biochem. J. 388 (2005), 647–655.
-
(2005)
Biochem. J.
, vol.388
, pp. 647-655
-
-
Hassink, G.1
-
74
-
-
84874872486
-
Putting huntingtin “aggregation” in view with windows into the cellular milieu
-
Hatters, D.M., Putting huntingtin “aggregation” in view with windows into the cellular milieu. Curr. Top. Med. Chem. 12 (2012), 2611–2622.
-
(2012)
Curr. Top. Med. Chem.
, vol.12
, pp. 2611-2622
-
-
Hatters, D.M.1
-
75
-
-
35549006797
-
Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survival
-
Hayashi, T., Su, T.P., Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survival. Cell 131 (2007), 596–610.
-
(2007)
Cell
, vol.131
, pp. 596-610
-
-
Hayashi, T.1
Su, T.P.2
-
76
-
-
35748948975
-
In and out of the ER: protein folding, quality control, degradation, and related human diseases
-
Hebert, D.N., Molinari, M., In and out of the ER: protein folding, quality control, degradation, and related human diseases. Physiol. Rev. 87 (2007), 1377–1408.
-
(2007)
Physiol. Rev.
, vol.87
, pp. 1377-1408
-
-
Hebert, D.N.1
Molinari, M.2
-
77
-
-
84866322000
-
Visualization of cell-to-cell transmission of mutant huntingtin oligomers
-
Herrera, F., et al. Visualization of cell-to-cell transmission of mutant huntingtin oligomers. PLoS Curr., 3, 2011, RRN1210.
-
(2011)
PLoS Curr.
, vol.3
, pp. RRN1210
-
-
Herrera, F.1
-
78
-
-
70349627027
-
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
-
Hetz, C., et al. 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
-
79
-
-
84897094564
-
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases
-
Hetz, C., Mollereau, B., Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases. Nat. Rev. Neurosci. 15 (2014), 233–249.
-
(2014)
Nat. Rev. Neurosci.
, vol.15
, pp. 233-249
-
-
Hetz, C.1
Mollereau, B.2
-
80
-
-
84883387793
-
Targeting the unfolded protein response in disease
-
Hetz, C., Chevet, E., Harding, H.P., Targeting the unfolded protein response in disease. Nat. Rev. Drug. Discov. 12 (2013), 703–719.
-
(2013)
Nat. Rev. Drug. Discov.
, vol.12
, pp. 703-719
-
-
Hetz, C.1
Chevet, E.2
Harding, H.P.3
-
81
-
-
28244499949
-
Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response
-
Hidvegi, T., et al. Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response. J. Biol. Chem. 280 (2005), 39002–39015.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 39002-39015
-
-
Hidvegi, T.1
-
82
-
-
84859983420
-
Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease
-
Hipp, M.S., et al. Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease. J. Cell Biol. 196 (2012), 573–587.
-
(2012)
J. Cell Biol.
, vol.196
, pp. 573-587
-
-
Hipp, M.S.1
-
83
-
-
84906794886
-
Proteostasis impairment in protein-misfolding and -aggregation diseases
-
Hipp, M.S., Park, S.H., Hartl, F.U., Proteostasis impairment in protein-misfolding and -aggregation diseases. Trends Cell Biol. 24 (2014), 506–514.
-
(2014)
Trends Cell Biol.
, vol.24
, pp. 506-514
-
-
Hipp, M.S.1
Park, S.H.2
Hartl, F.U.3
-
84
-
-
63649161943
-
The ubiquitylation machinery of the endoplasmic reticulum
-
Hirsch, C., et al. The ubiquitylation machinery of the endoplasmic reticulum. Nature 458 (2009), 453–460.
-
(2009)
Nature
, vol.458
, pp. 453-460
-
-
Hirsch, C.1
-
85
-
-
9144223729
-
Inefficient degradation of truncated polyglutamine proteins by the proteasome
-
Holmberg, C.I., et al. Inefficient degradation of truncated polyglutamine proteins by the proteasome. EMBO J. 23 (2004), 4307–4318.
-
(2004)
EMBO J.
, vol.23
, pp. 4307-4318
-
-
Holmberg, C.I.1
-
86
-
-
33646188838
-
The unfolded protein response affects neuronal cell cycle protein expression: implications for Alzheimer's disease pathogenesis
-
Hoozemans, J.J., et al. The unfolded protein response affects neuronal cell cycle protein expression: implications for Alzheimer's disease pathogenesis. Exp. Gerontol. 41 (2006), 380–386.
-
(2006)
Exp. Gerontol.
, vol.41
, pp. 380-386
-
-
Hoozemans, J.J.1
-
87
-
-
33846605587
-
Activation of the unfolded protein response in Parkinson's disease
-
Hoozemans, J.J., et al. Activation of the unfolded protein response in Parkinson's disease. Biochem. Biophys. Res. Commun. 354 (2007), 707–711.
-
(2007)
Biochem. Biophys. Res. Commun.
, vol.354
, pp. 707-711
-
-
Hoozemans, J.J.1
-
88
-
-
65349093893
-
The unfolded protein response is activated in pretangle neurons in Alzheimer's disease hippocampus
-
Hoozemans, J.J., et al. The unfolded protein response is activated in pretangle neurons in Alzheimer's disease hippocampus. Am. J. Pathol. 174 (2009), 1241–1251.
-
(2009)
Am. J. Pathol.
, vol.174
, pp. 1241-1251
-
-
Hoozemans, J.J.1
-
89
-
-
77952811195
-
EDEM1 accelerates the trimming of alpha1,2-linked mannose on the C branch of N-glycans
-
Hosokawa, N., et al. EDEM1 accelerates the trimming of alpha1,2-linked mannose on the C branch of N-glycans. Glycobiology 20 (2010), 567–575.
-
(2010)
Glycobiology
, vol.20
, pp. 567-575
-
-
Hosokawa, N.1
-
90
-
-
84929709547
-
Quantitative interaction proteomics of neurodegenerative disease proteins
-
Hosp, F., et al. Quantitative interaction proteomics of neurodegenerative disease proteins. Cell Rep. 11 (2015), 1134–1146.
-
(2015)
Cell Rep.
, vol.11
, pp. 1134-1146
-
-
Hosp, F.1
-
91
-
-
84879392732
-
Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-kappaB pathway
-
Hyrskyluoto, A., et al. Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-kappaB pathway. Cell Death Dis., 4, 2013, e646.
-
(2013)
Cell Death Dis.
, vol.4
, pp. e646
-
-
Hyrskyluoto, A.1
-
92
-
-
2542448088
-
Infiltration of the brain by pathogens causes Alzheimer's disease
-
Itzhaki, R.F., et al. Infiltration of the brain by pathogens causes Alzheimer's disease. Neurobiol. Aging 25 (2004), 619–627.
-
(2004)
Neurobiol. Aging
, vol.25
, pp. 619-627
-
-
Itzhaki, R.F.1
-
93
-
-
28244437028
-
The Yin and Yang of protein folding
-
Jahn, T.R., Radford, S.E., The Yin and Yang of protein folding. FEBS J. 272 (2005), 5962–5970.
-
(2005)
FEBS J.
, vol.272
, pp. 5962-5970
-
-
Jahn, T.R.1
Radford, S.E.2
-
94
-
-
84941198850
-
Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy
-
Jaunmuktane, Z., et al. Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy. Nature 525 (2015), 247–250.
-
(2015)
Nature
, vol.525
, pp. 247-250
-
-
Jaunmuktane, Z.1
-
95
-
-
84938212067
-
Targeting the IRE1alpha-XBP1 branch of the unfolded protein response in human diseases
-
Jiang, D., Niwa, M., Koong, A.C., Targeting the IRE1alpha-XBP1 branch of the unfolded protein response in human diseases. Semin. Cancer Biol. 33 (2015), 48–56.
-
(2015)
Semin. Cancer Biol.
, vol.33
, pp. 48-56
-
-
Jiang, D.1
Niwa, M.2
Koong, A.C.3
-
96
-
-
78049402866
-
Wolcott-Rallison syndrome
-
Julier, C., Nicolino, M., Wolcott-Rallison syndrome. Orphanet J. Rare Dis., 5, 2010, 29.
-
(2010)
Orphanet J. Rare Dis.
, vol.5
, pp. 29
-
-
Julier, C.1
Nicolino, M.2
-
97
-
-
0035162691
-
A novel quality control compartment derived from the endoplasmic reticulum
-
Kamhi-Nesher, S., et al. A novel quality control compartment derived from the endoplasmic reticulum. Mol. Biol. Cell 12 (2001), 1711–1723.
-
(2001)
Mol. Biol. Cell
, vol.12
, pp. 1711-1723
-
-
Kamhi-Nesher, S.1
-
98
-
-
77949764687
-
Loss of HRD1-mediated protein degradation causes amyloid precursor protein accumulation and amyloid-beta generation
-
Kaneko, M., et al. Loss of HRD1-mediated protein degradation causes amyloid precursor protein accumulation and amyloid-beta generation. J. Neurosci. 30 (2010), 3924–3932.
-
(2010)
J. Neurosci.
, vol.30
, pp. 3924-3932
-
-
Kaneko, M.1
-
99
-
-
84896837095
-
Hsp90-tau complex reveals molecular basis for specificity in chaperone action
-
Karagöz, G.E., et al. Hsp90-tau complex reveals molecular basis for specificity in chaperone action. Cell 156 (2014), 963–974.
-
(2014)
Cell
, vol.156
, pp. 963-974
-
-
Karagöz, G.E.1
-
100
-
-
84948717105
-
Huntingtin haplotypes provide prioritized target panels for allele-specific silencing in huntington disease patients of european ancestry
-
Kay, C., et al. Huntingtin haplotypes provide prioritized target panels for allele-specific silencing in huntington disease patients of european ancestry. Mol. Ther. 23 (2015), 1759–1771.
-
(2015)
Mol. Ther.
, vol.23
, pp. 1759-1771
-
-
Kay, C.1
-
101
-
-
0242668337
-
Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis
-
Kayed, R., et al. Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis. Science 300 (2003), 486–489.
-
(2003)
Science
, vol.300
, pp. 486-489
-
-
Kayed, R.1
-
102
-
-
0036677435
-
Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease
-
Keene, C.D., et al. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc. Natl. Acad. Sci. USA 99 (2002), 10671–10676.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, pp. 10671-10676
-
-
Keene, C.D.1
-
103
-
-
84927626719
-
PINK1-induced mitophagy promotes neuroprotection in Huntington's disease
-
Khalil, B., et al. PINK1-induced mitophagy promotes neuroprotection in Huntington's disease. Cell Death Dis., 6, 2015, e1617.
-
(2015)
Cell Death Dis.
, vol.6
, pp. e1617
-
-
Khalil, B.1
-
104
-
-
78049383942
-
Protein homeostasis in models of aging and age-related conformational disease
-
Kikis, E.A., Gidalevitz, T., Morimoto, R.I., Protein homeostasis in models of aging and age-related conformational disease. Adv. Exp. Med. Biol. 694 (2010), 138–159.
-
(2010)
Adv. Exp. Med. Biol.
, vol.694
, pp. 138-159
-
-
Kikis, E.A.1
Gidalevitz, T.2
Morimoto, R.I.3
-
105
-
-
9144239817
-
Human HRD1 is an E3 ubiquitin ligase involved in degradation of proteins from the endoplasmic reticulum
-
Kikkert, M., et al. Human HRD1 is an E3 ubiquitin ligase involved in degradation of proteins from the endoplasmic reticulum. J. Biol. Chem. 279 (2004), 3525–3534.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 3525-3534
-
-
Kikkert, M.1
-
106
-
-
84906777179
-
Granulovacuolar degeneration and unfolded protein response in mouse models of tauopathy and Abeta amyloidosis
-
Kohler, C., Dinekov, M., Gotz, J., Granulovacuolar degeneration and unfolded protein response in mouse models of tauopathy and Abeta amyloidosis. Neurobiol. Dis. 71 (2014), 169–179.
-
(2014)
Neurobiol. Dis.
, vol.71
, pp. 169-179
-
-
Kohler, C.1
Dinekov, M.2
Gotz, J.3
-
107
-
-
0034693217
-
Herp, a new ubiquitin-like membrane protein induced by endoplasmic reticulum stress
-
Kokame, K., et al. Herp, a new ubiquitin-like membrane protein induced by endoplasmic reticulum stress. J Biol. Chem. 275 (2000), 32846–32853.
-
(2000)
J Biol. Chem.
, vol.275
, pp. 32846-32853
-
-
Kokame, K.1
-
108
-
-
34548651757
-
PERK-dependent compartmentalization of ERAD and unfolded protein response machineries during ER stress
-
Kondratyev, M., et al. PERK-dependent compartmentalization of ERAD and unfolded protein response machineries during ER stress. Exp. Cell Res. 313 (2007), 3395–3407.
-
(2007)
Exp. Cell Res.
, vol.313
, pp. 3395-3407
-
-
Kondratyev, M.1
-
109
-
-
0033773935
-
Conformational disease
-
Kopito, R.R., Ron, D., Conformational disease. Nat. Cell Biol. 2 (2000), E207–E209.
-
(2000)
Nat. Cell Biol.
, vol.2
, pp. E207-E209
-
-
Kopito, R.R.1
Ron, D.2
-
110
-
-
84856958110
-
VCP mutations in familial and sporadic amyotrophic lateral sclerosis
-
837
-
Koppers, M., et al. VCP mutations in familial and sporadic amyotrophic lateral sclerosis. Neurobiol. Aging 33 (2012), e7–13 837.
-
(2012)
Neurobiol. Aging
, vol.33
, pp. e7-13
-
-
Koppers, M.1
-
111
-
-
33646185061
-
Membrane topology of the yeast endoplasmic reticulum-localized ubiquitin ligase Doa10 and comparison with its human ortholog TEB4 (MARCH-VI)
-
Kreft, S.G., Wang, L., Hochstrasser, M., Membrane topology of the yeast endoplasmic reticulum-localized ubiquitin ligase Doa10 and comparison with its human ortholog TEB4 (MARCH-VI). J. Biol. Chem. 281 (2006), 4646–4653.
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 4646-4653
-
-
Kreft, S.G.1
Wang, L.2
Hochstrasser, M.3
-
112
-
-
79961013560
-
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease
-
Labbadia, J., et al. Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. J. Clin. Investig. 121 (2011), 3306–3319.
-
(2011)
J. Clin. Investig.
, vol.121
, pp. 3306-3319
-
-
Labbadia, J.1
-
113
-
-
84930746830
-
The biology of proteostasis in aging and disease
-
Labbadia, J., Morimoto, R.I., The biology of proteostasis in aging and disease. Annu. Rev. Biochem. 84 (2015), 435–464.
-
(2015)
Annu. Rev. Biochem.
, vol.84
, pp. 435-464
-
-
Labbadia, J.1
Morimoto, R.I.2
-
114
-
-
78650811716
-
Formation and toxicity of soluble polyglutamine oligomers in living cells
-
Lajoie, P., Snapp, E.L., Formation and toxicity of soluble polyglutamine oligomers in living cells. PLoS One, 5, 2010, e15245.
-
(2010)
PLoS One
, vol.5
, pp. e15245
-
-
Lajoie, P.1
Snapp, E.L.2
-
115
-
-
0034730172
-
Inhibition of the ubiquitin-proteasome system in Alzheimer's disease
-
Lam, Y.A., et al. Inhibition of the ubiquitin-proteasome system in Alzheimer's disease. Proc. Natl. Acad. Sci. USA 97 (2000), 9902–9906.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, pp. 9902-9906
-
-
Lam, Y.A.1
-
116
-
-
84962570989
-
N-Glycan-based ER molecular chaperone and protein quality control system: the calnexin binding cycle
-
Lamriben, L., et al. N-Glycan-based ER molecular chaperone and protein quality control system: the calnexin binding cycle. Traffic 17 (2016), 308–326.
-
(2016)
Traffic
, vol.17
, pp. 308-326
-
-
Lamriben, L.1
-
117
-
-
34948838383
-
Riluzole in Huntington's disease: a 3-year, randomized controlled study
-
Landwehrmeyer, G.B., et al. Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann. Neurol. 62 (2007), 262–272.
-
(2007)
Ann. Neurol.
, vol.62
, pp. 262-272
-
-
Landwehrmeyer, G.B.1
-
118
-
-
70349855203
-
Glycoprotein folding, quality control and ER-associated degradation
-
Lederkremer, G.Z., Glycoprotein folding, quality control and ER-associated degradation. Curr. Opin. Struct. Biol. 19 (2009), 515–523.
-
(2009)
Curr. Opin. Struct. Biol.
, vol.19
, pp. 515-523
-
-
Lederkremer, G.Z.1
-
119
-
-
83455169115
-
IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux
-
Lee, H., et al. IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux. Hum. Mol. Genet. 21 (2012), 101–114.
-
(2012)
Hum. Mol. Genet.
, vol.21
, pp. 101-114
-
-
Lee, H.1
-
120
-
-
77956288847
-
Activation of PERK signaling attenuates Abeta-mediated ER stress
-
Lee do, Y., et al. Activation of PERK signaling attenuates Abeta-mediated ER stress. PLoS One, 5, 2010, e10489.
-
(2010)
PLoS One
, vol.5
, pp. e10489
-
-
Lee do, Y.1
-
121
-
-
84873027092
-
Compartmentalization of endoplasmic reticulum quality control and ER-associated degradation factors
-
Leitman, J., et al. Compartmentalization of endoplasmic reticulum quality control and ER-associated degradation factors. DNA Cell Biol. 32 (2013), 2–7.
-
(2013)
DNA Cell Biol.
, vol.32
, pp. 2-7
-
-
Leitman, J.1
-
122
-
-
84897002606
-
ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin
-
Leitman, J., et al. ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin. PLoS One, 9, 2014, e90803.
-
(2014)
PLoS One
, vol.9
, pp. e90803
-
-
Leitman, J.1
-
123
-
-
84898754350
-
Herp coordinates compartmentalization and recruitment of HRD1 and misfolded proteins for ERAD
-
Leitman, J., et al. Herp coordinates compartmentalization and recruitment of HRD1 and misfolded proteins for ERAD. Mol. Biol. Cell 25 (2014), 1050–1060.
-
(2014)
Mol. Biol. Cell
, vol.25
, pp. 1050-1060
-
-
Leitman, J.1
-
124
-
-
84887792874
-
Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress
-
Leitman, J., Ulrich Hartl, F., Lederkremer, G.Z., Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress. Nat. Commun., 4, 2013, 2753.
-
(2013)
Nat. Commun.
, vol.4
, pp. 2753
-
-
Leitman, J.1
Ulrich Hartl, F.2
Lederkremer, G.Z.3
-
125
-
-
36049049392
-
IRE1 signaling affects cell fate during the unfolded protein response
-
Lin, J.H., et al. 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
-
126
-
-
84876032962
-
New directions in ER stress-induced cell death
-
Logue, S.E., et al. New directions in ER stress-induced cell death. Apoptosis 18 (2013), 537–546.
-
(2013)
Apoptosis
, vol.18
, pp. 537-546
-
-
Logue, S.E.1
-
127
-
-
84946925426
-
Long term aggresome accumulation leads to dna damage, p53-dependent cell cycle arrest, and steric interference in mitosis
-
Lu, M., Boschetti, C., Tunnacliffe, A., Long term aggresome accumulation leads to dna damage, p53-dependent cell cycle arrest, and steric interference in mitosis. J. Biol. Chem. 290 (2015), 27986–28000.
-
(2015)
J. Biol. Chem.
, vol.290
, pp. 27986-28000
-
-
Lu, M.1
Boschetti, C.2
Tunnacliffe, A.3
-
128
-
-
84883453343
-
Suppression of eIF2alpha kinases alleviates Alzheimer's disease-related plasticity and memory deficits
-
Ma, T., et al. Suppression of eIF2alpha kinases alleviates Alzheimer's disease-related plasticity and memory deficits. Nat. Neurosci. 16 (2013), 1299–1305.
-
(2013)
Nat. Neurosci.
, vol.16
, pp. 1299-1305
-
-
Ma, T.1
-
129
-
-
77952583297
-
Protein quality control in the ER: the recognition of misfolded proteins
-
Maattanen, P., et al. Protein quality control in the ER: the recognition of misfolded proteins. Semin. Cell Dev. Biol. 21 (2010), 500–511.
-
(2010)
Semin. Cell Dev. Biol.
, vol.21
, pp. 500-511
-
-
Maattanen, P.1
-
130
-
-
85018400910
-
Neurodegenerative disease: VCP mutations lead to defects in mitochondrial dynamics
-
Malpass, K., Neurodegenerative disease: VCP mutations lead to defects in mitochondrial dynamics. Nat. Rev. Neurol., 9, 2013, 239.
-
(2013)
Nat. Rev. Neurol.
, vol.9
, pp. 239
-
-
Malpass, K.1
-
131
-
-
23044437134
-
Neuroprotective effects of sigma-1 receptor agonists against beta-amyloid-induced toxicity
-
Marrazzo, A., et al. Neuroprotective effects of sigma-1 receptor agonists against beta-amyloid-induced toxicity. Neuroreport 16 (2005), 1223–1226.
-
(2005)
Neuroreport
, vol.16
, pp. 1223-1226
-
-
Marrazzo, A.1
-
132
-
-
26444471905
-
Structural properties and neuronal toxicity of amyotrophic lateral sclerosis-associated Cu/Zn superoxide dismutase 1 aggregates
-
Matsumoto, G., et al. Structural properties and neuronal toxicity of amyotrophic lateral sclerosis-associated Cu/Zn superoxide dismutase 1 aggregates. J. Cell Biol. 171 (2005), 75–85.
-
(2005)
J. Cell Biol.
, vol.171
, pp. 75-85
-
-
Matsumoto, G.1
-
133
-
-
0035660075
-
The interaction between neuroactive steroids and the sigma1 receptor function: behavioral consequences and therapeutic opportunities
-
Maurice, T., et al. The interaction between neuroactive steroids and the sigma1 receptor function: behavioral consequences and therapeutic opportunities. Brain Res. Brain Res. Rev. 37 (2001), 116–132.
-
(2001)
Brain Res. Brain Res. Rev.
, vol.37
, pp. 116-132
-
-
Maurice, T.1
-
134
-
-
84885191322
-
Respiratory infection promotes T cell infiltration and amyloid-beta deposition in APP/PS1 mice
-
McManus, R.M., et al. Respiratory infection promotes T cell infiltration and amyloid-beta deposition in APP/PS1 mice. Neurobiol. Aging 35 (2014), 109–121.
-
(2014)
Neurobiol. Aging
, vol.35
, pp. 109-121
-
-
McManus, R.M.1
-
135
-
-
0035910634
-
Proteasomal function is impaired in substantia nigra in Parkinson's disease
-
McNaught, K.S., Jenner, P., Proteasomal function is impaired in substantia nigra in Parkinson's disease. Neurosci. Lett. 297 (2001), 191–194.
-
(2001)
Neurosci. Lett.
, vol.297
, pp. 191-194
-
-
McNaught, K.S.1
Jenner, P.2
-
136
-
-
67349104211
-
Molecular mechanisms underlying polyalanine diseases
-
Messaed, C., Rouleau, G.A., Molecular mechanisms underlying polyalanine diseases. Neurobiol. Dis. 34 (2009), 397–405.
-
(2009)
Neurobiol. Dis.
, vol.34
, pp. 397-405
-
-
Messaed, C.1
Rouleau, G.A.2
-
137
-
-
84911862220
-
Sigma-1 receptor is involved in degradation of intranuclear inclusions in a cellular model of Huntington's disease
-
Miki, Y., et al. Sigma-1 receptor is involved in degradation of intranuclear inclusions in a cellular model of Huntington's disease. Neurobiol. Dis. 74 (2015), 25–31.
-
(2015)
Neurobiol. Dis.
, vol.74
, pp. 25-31
-
-
Miki, Y.1
-
138
-
-
3142582012
-
Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum
-
Miranda, E., Romisch, K., Lomas, D.A., Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum. J. Biol. Chem. 279 (2004), 28283–28291.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 28283-28291
-
-
Miranda, E.1
Romisch, K.2
Lomas, D.A.3
-
139
-
-
63249135140
-
Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease
-
Mitra, S., Tsvetkov, A.S., Finkbeiner, S., Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease. J. Biol. Chem. 284 (2009), 4398–4403.
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 4398-4403
-
-
Mitra, S.1
Tsvetkov, A.S.2
Finkbeiner, S.3
-
140
-
-
84861450392
-
Sustained translational repression by eIF2alpha-P mediates prion neurodegeneration
-
Moreno, J.A., et al. Sustained translational repression by eIF2alpha-P mediates prion neurodegeneration. Nature 485 (2012), 507–511.
-
(2012)
Nature
, vol.485
, pp. 507-511
-
-
Moreno, J.A.1
-
141
-
-
84901317040
-
Recent technical developments in the study of ER-associated degradation
-
Nakatsukasa, K., Kamura, T., Brodsky, J.L., Recent technical developments in the study of ER-associated degradation. Curr. Opin. Cell Biol. 29c (2014), 82–91.
-
(2014)
Curr. Opin. Cell Biol.
, vol.29c
, pp. 82-91
-
-
Nakatsukasa, K.1
Kamura, T.2
Brodsky, J.L.3
-
142
-
-
84923260646
-
Targeted excision of VCP R155H mutation by Cre-LoxP technology as a promising therapeutic strategy for valosin-containing protein disease
-
Nalbandian, A., et al. Targeted excision of VCP R155H mutation by Cre-LoxP technology as a promising therapeutic strategy for valosin-containing protein disease. Hum. Gene. Ther. Methods 26 (2015), 13–24.
-
(2015)
Hum. Gene. Ther. Methods
, vol.26
, pp. 13-24
-
-
Nalbandian, A.1
-
143
-
-
84929121456
-
Sigma-1 receptor directly interacts with Rac1-GTPase in the brain mitochondria
-
Natsvlishvili, N., et al. Sigma-1 receptor directly interacts with Rac1-GTPase in the brain mitochondria. BMC Biochem., 16, 2015, 11.
-
(2015)
BMC Biochem.
, vol.16
, pp. 11
-
-
Natsvlishvili, N.1
-
144
-
-
75649105019
-
Current treatment and recent clinical research in Alzheimer's disease
-
Neugroschl, J., Sano, M., Current treatment and recent clinical research in Alzheimer's disease. Mt. Sinai J. Med. 77 (2010), 3–16.
-
(2010)
Mt. Sinai J. Med.
, vol.77
, pp. 3-16
-
-
Neugroschl, J.1
Sano, M.2
-
145
-
-
84924047943
-
Role of sigma-1 receptors in neurodegenerative diseases
-
Nguyen, L., et al. Role of sigma-1 receptors in neurodegenerative diseases. J. Pharmacol. Sci. 127 (2015), 17–29.
-
(2015)
J. Pharmacol. Sci.
, vol.127
, pp. 17-29
-
-
Nguyen, L.1
-
146
-
-
84905987689
-
EDEM2 initiates mammalian glycoprotein ERAD by catalyzing the first mannose trimming step
-
Ninagawa, S., et al. EDEM2 initiates mammalian glycoprotein ERAD by catalyzing the first mannose trimming step. J. Cell Biol. 206 (2014), 347–356.
-
(2014)
J. Cell Biol.
, vol.206
, pp. 347-356
-
-
Ninagawa, S.1
-
147
-
-
44849124411
-
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
-
Nishitoh, H., et al. 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
-
148
-
-
84867485621
-
TUDCA, a bile acid, attenuates amyloid precursor protein processing and amyloid-beta deposition in APP/PS1 mice
-
Nunes, A.F., et al. TUDCA, a bile acid, attenuates amyloid precursor protein processing and amyloid-beta deposition in APP/PS1 mice. Mol. Neurobiol. 45 (2012), 440–454.
-
(2012)
Mol. Neurobiol.
, vol.45
, pp. 440-454
-
-
Nunes, A.F.1
-
149
-
-
57649245230
-
Phosphorylation of the translation initiation factor eIF2alpha increases BACE1 levels and promotes amyloidogenesis
-
O'Connor, T., et al. Phosphorylation of the translation initiation factor eIF2alpha increases BACE1 levels and promotes amyloidogenesis. Neuron 60 (2008), 988–1009.
-
(2008)
Neuron
, vol.60
, pp. 988-1009
-
-
O'Connor, T.1
-
150
-
-
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
-
151
-
-
33748795800
-
EDEM1 regulates ER-associated degradation by accelerating de-mannosylation of folding-defective polypeptides and by inhibiting their covalent aggregation
-
Olivari, S., et al. EDEM1 regulates ER-associated degradation by accelerating de-mannosylation of folding-defective polypeptides and by inhibiting their covalent aggregation. Biochem. Biophys. Res. Commun. 349 (2006), 1278–1284.
-
(2006)
Biochem. Biophys. Res. Commun.
, vol.349
, pp. 1278-1284
-
-
Olivari, S.1
-
152
-
-
78650963274
-
Amyloid-like aggregates sequester numerous metastable proteins with essential cellular functions
-
Olzscha, H., et al. Amyloid-like aggregates sequester numerous metastable proteins with essential cellular functions. Cell 144 (2011), 67–78.
-
(2011)
Cell
, vol.144
, pp. 67-78
-
-
Olzscha, H.1
-
153
-
-
34548331451
-
Is the ubiquitin-proteasome system impaired in Huntington's disease?
-
Ortega, Z., Diaz-Hernandez, M., Lucas, J.J., Is the ubiquitin-proteasome system impaired in Huntington's disease?. Cell. Mol. Life Sci. 64 (2007), 2245–2257.
-
(2007)
Cell. Mol. Life Sci.
, vol.64
, pp. 2245-2257
-
-
Ortega, Z.1
Diaz-Hernandez, M.2
Lucas, J.J.3
-
154
-
-
84881478531
-
Potential for therapeutic manipulation of the UPR in disease
-
Park, S.W., Ozcan, U., Potential for therapeutic manipulation of the UPR in disease. Semin. Immunopathol. 35 (2013), 351–373.
-
(2013)
Semin. Immunopathol.
, vol.35
, pp. 351-373
-
-
Park, S.W.1
Ozcan, U.2
-
155
-
-
0020957538
-
Transient glucosylation of protein-bound Man9GlcNAc2, Man8GlcNAc2, and Man7GlcNAc2 in calf thyroid cells. A possible recognition signal in the processing of glycoproteins
-
Parodi, A.J., Mendelzon, D.H., Lederkremer, G.Z., Transient glucosylation of protein-bound Man9GlcNAc2, Man8GlcNAc2, and Man7GlcNAc2 in calf thyroid cells. A possible recognition signal in the processing of glycoproteins. J. Biol. Chem. 258 (1983), 8260–8265.
-
(1983)
J. Biol. Chem.
, vol.258
, pp. 8260-8265
-
-
Parodi, A.J.1
Mendelzon, D.H.2
Lederkremer, G.Z.3
-
156
-
-
84927949614
-
Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain
-
Pearce, M.M., et al. Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain. Nat. Commun., 6, 2015, 6768.
-
(2015)
Nat. Commun.
, vol.6
, pp. 6768
-
-
Pearce, M.M.1
-
157
-
-
84947474720
-
Comparative incidence of conformational, neurodegenerative disorders
-
de Pedro-Cuesta, J., et al. Comparative incidence of conformational, neurodegenerative disorders. PLoS One, 10, 2015, e0137342.
-
(2015)
PLoS One
, vol.10
, pp. e0137342
-
-
de Pedro-Cuesta, J.1
-
158
-
-
79551604651
-
Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates
-
Perlmutter, D.H., Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates. Annu. Rev. Med. 62 (2011), 333–345.
-
(2011)
Annu. Rev. Med.
, vol.62
, pp. 333-345
-
-
Perlmutter, D.H.1
-
159
-
-
11144356089
-
CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation
-
Petrucelli, L., et al. CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation. Hum. Mol. Genet. 13 (2004), 703–714.
-
(2004)
Hum. Mol. Genet.
, vol.13
, pp. 703-714
-
-
Petrucelli, L.1
-
160
-
-
84949511959
-
Polyalanine expansions drive a shift into alpha-helical clusters without amyloid-fibril formation
-
Polling, S., et al. Polyalanine expansions drive a shift into alpha-helical clusters without amyloid-fibril formation. Nat. Struct. Mol. Biol. 22 (2015), 1008–1015.
-
(2015)
Nat. Struct. Mol. Biol.
, vol.22
, pp. 1008-1015
-
-
Polling, S.1
-
161
-
-
84857953079
-
The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis
-
Prell, T., et al. The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis. Eur. J. Neurosci. 35 (2012), 652–660.
-
(2012)
Eur. J. Neurosci.
, vol.35
, pp. 652-660
-
-
Prell, T.1
-
162
-
-
0036136901
-
AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation
-
Rabinovich, E., et al. AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation. Mol. Cell Biol. 22 (2002), 626–634.
-
(2002)
Mol. Cell Biol.
, vol.22
, pp. 626-634
-
-
Rabinovich, E.1
-
163
-
-
84942082866
-
Neurobiology of Alzheimer's disease: integrated molecular, physiological, anatomical, biomarker, and cognitive dimensions
-
Raskin, J., et al. Neurobiology of Alzheimer's disease: integrated molecular, physiological, anatomical, biomarker, and cognitive dimensions. Curr. Alzheimer Res. 12 (2015), 712–722.
-
(2015)
Curr. Alzheimer Res.
, vol.12
, pp. 712-722
-
-
Raskin, J.1
-
164
-
-
80655144729
-
Bypass of glycan-dependent glycoprotein delivery to ERAD by up-regulated EDEM1
-
Ron, E., et al. Bypass of glycan-dependent glycoprotein delivery to ERAD by up-regulated EDEM1. Mol. Biol. Cell 22 (2011), 3945–3954.
-
(2011)
Mol. Biol. Cell
, vol.22
, pp. 3945-3954
-
-
Ron, E.1
-
165
-
-
3142514201
-
Protein aggregation and neurodegenerative disease
-
Suppl, S10-7
-
Ross, C.A., Poirier, M.A., Protein aggregation and neurodegenerative disease. Nat. Med., 10, 2004 Suppl, S10-7.
-
(2004)
Nat. Med.
, vol.10
-
-
Ross, C.A.1
Poirier, M.A.2
-
166
-
-
84912572110
-
Modulation of the maladaptive stress response to manage diseases of protein folding
-
Roth, D.M., et al. Modulation of the maladaptive stress response to manage diseases of protein folding. PLoS Biol., 12, 2014, e1001998.
-
(2014)
PLoS Biol.
, vol.12
, pp. e1001998
-
-
Roth, D.M.1
-
167
-
-
60849093466
-
Current hypotheses for the underlying biology of amyotrophic lateral sclerosis
-
Rothstein, J.D., Current hypotheses for the underlying biology of amyotrophic lateral sclerosis. Ann. Neurol. 65:Suppl 1 (2009), S3–S9.
-
(2009)
Ann. Neurol.
, vol.65
, pp. S3-S9
-
-
Rothstein, J.D.1
-
168
-
-
80053603907
-
Unravelling the twists and turns of the serpinopathies
-
Roussel, B.D., et al. Unravelling the twists and turns of the serpinopathies. FEBS J. 278 (2011), 3859–3867.
-
(2011)
FEBS J.
, vol.278
, pp. 3859-3867
-
-
Roussel, B.D.1
-
169
-
-
84870838478
-
Endoplasmic reticulum dysfunction in neurological disease
-
Roussel, B.D., et al. Endoplasmic reticulum dysfunction in neurological disease. Lancet Neurol. 12 (2013), 105–118.
-
(2013)
Lancet Neurol.
, vol.12
, pp. 105-118
-
-
Roussel, B.D.1
-
170
-
-
63349104307
-
Protective effect against Parkinson's disease-related insults through the activation of XBP1
-
Sado, M., et al. Protective effect against Parkinson's disease-related insults through the activation of XBP1. Brain Res. 1257 (2009), 16–24.
-
(2009)
Brain Res.
, vol.1257
, pp. 16-24
-
-
Sado, M.1
-
171
-
-
77950853379
-
Endoplasmic reticulum stress in motor neurons of the spinal cord in sporadic amyotrophic lateral sclerosis
-
Sasaki, S., Endoplasmic reticulum stress in motor neurons of the spinal cord in sporadic amyotrophic lateral sclerosis. J. Neuropathol. Exp. Neurol. 69 (2010), 346–355.
-
(2010)
J. Neuropathol. Exp. Neurol.
, vol.69
, pp. 346-355
-
-
Sasaki, S.1
-
172
-
-
3042717240
-
Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation
-
Schaffar, G., et al. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol. Cell 15 (2004), 95–105.
-
(2004)
Mol. Cell
, vol.15
, pp. 95-105
-
-
Schaffar, G.1
-
173
-
-
84939562716
-
The unfolded protein response in neurodegenerative diseases: a neuropathological perspective
-
Scheper, W., Hoozemans, J.J., The unfolded protein response in neurodegenerative diseases: a neuropathological perspective. Acta Neuropathol. 130 (2015), 315–331.
-
(2015)
Acta Neuropathol.
, vol.130
, pp. 315-331
-
-
Scheper, W.1
Hoozemans, J.J.2
-
174
-
-
22244446505
-
The mammalian unfolded protein response
-
Schroder, M., Kaufman, R.J., The mammalian unfolded protein response. Annu. Rev. Biochem. 74 (2005), 739–789.
-
(2005)
Annu. Rev. Biochem.
, vol.74
, pp. 739-789
-
-
Schroder, M.1
Kaufman, R.J.2
-
175
-
-
28144436887
-
The ubiquitin-domain protein HERP forms a complex with components of the endoplasmic reticulum associated degradation pathway
-
Schulze, A., et al. The ubiquitin-domain protein HERP forms a complex with components of the endoplasmic reticulum associated degradation pathway. J. Mol. Biol. 354 (2005), 1021–1027.
-
(2005)
J. Mol. Biol.
, vol.354
, pp. 1021-1027
-
-
Schulze, A.1
-
176
-
-
84990896014
-
Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation
-
Seidel, K., et al. Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation. Neuropathol. Appl. Neurobiol., 2015.
-
(2015)
Neuropathol. Appl. Neurobiol.
-
-
Seidel, K.1
-
177
-
-
37849030901
-
Polyglutamine diseases: emerging concepts in pathogenesis and therapy
-
Spec No. 2
-
Shao, J., Diamond, M.I., Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum. Mol. Genet. 16 (2007), R115–R123 Spec No. 2.
-
(2007)
Hum. Mol. Genet.
, vol.16
, pp. R115-R123
-
-
Shao, J.1
Diamond, M.I.2
-
178
-
-
84991007871
-
Genesis of ER stress in huntington's disease
-
Shenkman, M., Eiger, H., Lederkremer, G.Z., Genesis of ER stress in huntington's disease. ER Stress Dis. 2 (2015), 94–106.
-
(2015)
ER Stress Dis.
, vol.2
, pp. 94-106
-
-
Shenkman, M.1
Eiger, H.2
Lederkremer, G.Z.3
-
179
-
-
84876991539
-
Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments
-
Shoulders, M.D., et al. Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments. Cell Rep. 3 (2013), 1279–1292.
-
(2013)
Cell Rep.
, vol.3
, pp. 1279-1292
-
-
Shoulders, M.D.1
-
180
-
-
84881530677
-
Pharmacological brake-release of mRNA translation enhances cognitive memory
-
Sidrauski, C., et al. Pharmacological brake-release of mRNA translation enhances cognitive memory. Elife, 2, 2013, e00498.
-
(2013)
Elife
, vol.2
, pp. e00498
-
-
Sidrauski, C.1
-
181
-
-
28244466782
-
CHOP/GADD153 is a mediator of apoptotic death in substantia nigra dopamine neurons in an in vivo neurotoxin model of parkinsonism
-
Silva, R.M., et al. CHOP/GADD153 is a mediator of apoptotic death in substantia nigra dopamine neurons in an in vivo neurotoxin model of parkinsonism. J. Neurochem. 95 (2005), 974–986.
-
(2005)
J. Neurochem.
, vol.95
, pp. 974-986
-
-
Silva, R.M.1
-
182
-
-
84880606810
-
Disorders of protein misfolding: alpha-1-antitrypsin deficiency as prototype
-
Silverman, G.A., Pak, S.C., Perlmutter, D.H., Disorders of protein misfolding: alpha-1-antitrypsin deficiency as prototype. J. Pediatr. 163 (2013), 320–326.
-
(2013)
J. Pediatr.
, vol.163
, pp. 320-326
-
-
Silverman, G.A.1
Pak, S.C.2
Perlmutter, D.H.3
-
183
-
-
20144385980
-
PACS-2 controls endoplasmic reticulum-mitochondria communication and Bid-mediated apoptosis
-
Simmen, T., et al. PACS-2 controls endoplasmic reticulum-mitochondria communication and Bid-mediated apoptosis. EMBO J. 24 (2005), 717–729.
-
(2005)
EMBO J.
, vol.24
, pp. 717-729
-
-
Simmen, T.1
-
184
-
-
84929274276
-
Molecular chaperones and neuronal proteostasis
-
Smith, H.L., Li, W., Cheetham, M.E., Molecular chaperones and neuronal proteostasis. Semin. Cell Dev. Biol. 40 (2015), 142–152.
-
(2015)
Semin. Cell Dev. Biol.
, vol.40
, pp. 142-152
-
-
Smith, H.L.1
Li, W.2
Cheetham, M.E.3
-
185
-
-
82355184462
-
Activating transcription factor 6 limits intracellular accumulation of mutant alpha(1)-antitrypsin Z and mitochondrial damage in hepatoma cells
-
Smith, S.E., et al. Activating transcription factor 6 limits intracellular accumulation of mutant alpha(1)-antitrypsin Z and mitochondrial damage in hepatoma cells. J. Biol. Chem. 286 (2011), 41563–41577.
-
(2011)
J. Biol. Chem.
, vol.286
, pp. 41563-41577
-
-
Smith, S.E.1
-
186
-
-
84881426112
-
Intracellular accumulation of toxic turn amyloid-beta is associated with endoplasmic reticulum stress in Alzheimer's disease
-
Soejima, N., et al. Intracellular accumulation of toxic turn amyloid-beta is associated with endoplasmic reticulum stress in Alzheimer's disease. Curr. Alzheimer Res. 10 (2013), 11–20.
-
(2013)
Curr. Alzheimer Res.
, vol.10
, pp. 11-20
-
-
Soejima, N.1
-
187
-
-
84890239048
-
The ubiquitin-proteasome-system
-
Sommer, T., Wolf, D.H., The ubiquitin-proteasome-system. Biochim. Biophys. Acta, 1843, 2014, 1.
-
(2014)
Biochim. Biophys. Acta
, vol.1843
, pp. 1
-
-
Sommer, T.1
Wolf, D.H.2
-
188
-
-
0037264120
-
Unfolding the role of protein misfolding in neurodegenerative diseases
-
Soto, C., Unfolding the role of protein misfolding in neurodegenerative diseases. Nat. Rev. Neurosci. 4 (2003), 49–60.
-
(2003)
Nat. Rev. Neurosci.
, vol.4
, pp. 49-60
-
-
Soto, C.1
-
189
-
-
84944750508
-
beta-Amyloid: the key peptide in the pathogenesis of Alzheimer's disease
-
Sun, X., Chen, W.D., Wang, Y.D., beta-Amyloid: the key peptide in the pathogenesis of Alzheimer's disease. Front. Pharmacol., 6, 2015, 221.
-
(2015)
Front. Pharmacol.
, vol.6
, pp. 221
-
-
Sun, X.1
Chen, W.D.2
Wang, Y.D.3
-
190
-
-
79952264011
-
Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress
-
Tabas, I., Ron, D., Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress. Nat. Cell Biol. 13 (2011), 184–190.
-
(2011)
Nat. Cell Biol.
, vol.13
, pp. 184-190
-
-
Tabas, I.1
Ron, D.2
-
191
-
-
38349158062
-
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
-
Takahashi, T., et al. Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum. Mol. Genet. 17 (2008), 345–356.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 345-356
-
-
Takahashi, T.1
-
192
-
-
84931565973
-
N-linked sugar-regulated protein folding and quality control in the ER
-
Tannous, A., et al. N-linked sugar-regulated protein folding and quality control in the ER. Semin. Cell Dev. Biol. 41 (2015), 79–89.
-
(2015)
Semin. Cell Dev. Biol.
, vol.41
, pp. 79-89
-
-
Tannous, A.1
-
193
-
-
0037077040
-
Toxic proteins in neurodegenerative disease
-
Taylor, J.P., Hardy, J., Fischbeck, K.H., Toxic proteins in neurodegenerative disease. Science 296 (2002), 1991–1995.
-
(2002)
Science
, vol.296
, pp. 1991-1995
-
-
Taylor, J.P.1
Hardy, J.2
Fischbeck, K.H.3
-
194
-
-
79953288480
-
Selective inhibition of a regulatory subunit of protein phosphatase 1 restores proteostasis
-
Tsaytler, P., et al. Selective inhibition of a regulatory subunit of protein phosphatase 1 restores proteostasis. Science 332 (2011), 91–94.
-
(2011)
Science
, vol.332
, pp. 91-94
-
-
Tsaytler, P.1
-
195
-
-
77956254963
-
Parkin directly modulates 26S proteasome activity
-
Um, J.W., et al. Parkin directly modulates 26S proteasome activity. J. Neurosci. 30 (2010), 11805–11814.
-
(2010)
J. Neurosci.
, vol.30
, pp. 11805-11814
-
-
Um, J.W.1
-
196
-
-
0034723235
-
Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1
-
Urano, F., et al. Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1. Science 287 (2000), 664–666.
-
(2000)
Science
, vol.287
, pp. 664-666
-
-
Urano, F.1
-
197
-
-
84860471873
-
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy
-
Vidal, R.L., et al. Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy. Hum. Mol. Genet. 21 (2012), 2245–2262.
-
(2012)
Hum. Mol. Genet.
, vol.21
, pp. 2245-2262
-
-
Vidal, R.L.1
-
198
-
-
84942615942
-
Guanabenz treatment accelerates disease in a mutant SOD1 mouse model of ALS
-
Vieira, F.G., et al. Guanabenz treatment accelerates disease in a mutant SOD1 mouse model of ALS. PLoS One, 10, 2015, e0135570.
-
(2015)
PLoS One
, vol.10
, pp. e0135570
-
-
Vieira, F.G.1
-
199
-
-
84937392013
-
Neurodegenerative diseases: expanding the prion concept
-
Walker, L.C., Jucker, M., Neurodegenerative diseases: expanding the prion concept. Annu. Rev. Neurosci. 38 (2015), 87–103.
-
(2015)
Annu. Rev. Neurosci.
, vol.38
, pp. 87-103
-
-
Walker, L.C.1
Jucker, M.2
-
200
-
-
0037041426
-
Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo
-
Walsh, D.M., et al. Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo. Nature 416 (2002), 535–539.
-
(2002)
Nature
, vol.416
, pp. 535-539
-
-
Walsh, D.M.1
-
201
-
-
84928963751
-
Widespread Proteome Remodeling and Aggregation in Aging C. elegans
-
Walther, D.M., et al. Widespread Proteome Remodeling and Aggregation in Aging C. elegans. Cell 161 (2015), 919–932.
-
(2015)
Cell
, vol.161
, pp. 919-932
-
-
Walther, D.M.1
-
202
-
-
84943339574
-
Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates
-
Wear, M.P., et al. Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates. PLS One, 10, 2015, e0136362.
-
(2015)
PLS One
, vol.10
, pp. e0136362
-
-
Wear, M.P.1
-
203
-
-
38849146956
-
ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones
-
Wei, H., et al. ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones. Hum. Mol. Genet. 17 (2008), 469–477.
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 469-477
-
-
Wei, H.1
-
204
-
-
31144470450
-
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation
-
Weihl, C.C., et al. Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation. Hum. Mol. Genet. 15 (2006), 189–199.
-
(2006)
Hum. Mol. Genet.
, vol.15
, pp. 189-199
-
-
Weihl, C.C.1
-
205
-
-
79955979595
-
Phenylbutyric acid reduces amyloid plaques and rescues cognitive behavior in AD transgenic mice
-
Wiley, J.C., Pettan-Brewer, C., Ladiges, W.C., Phenylbutyric acid reduces amyloid plaques and rescues cognitive behavior in AD transgenic mice. Aging Cell 10 (2011), 418–428.
-
(2011)
Aging Cell
, vol.10
, pp. 418-428
-
-
Wiley, J.C.1
Pettan-Brewer, C.2
Ladiges, W.C.3
-
206
-
-
84955098544
-
Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA
-
Woerner, A.C., et al. Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA. Science 351 (2016), 173–176.
-
(2016)
Science
, vol.351
, pp. 173-176
-
-
Woerner, A.C.1
-
207
-
-
80053564708
-
Amyloid in neurodegenerative diseases: friend or foe?
-
Wolfe, K.J., Cyr, D.M., Amyloid in neurodegenerative diseases: friend or foe?. Semin. Cell Dev. Biol. 22 (2011), 476–481.
-
(2011)
Semin. Cell Dev. Biol.
, vol.22
, pp. 476-481
-
-
Wolfe, K.J.1
Cyr, D.M.2
-
208
-
-
77749270634
-
Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP
-
Yang, H., et al. Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP. PLoS One, 5, 2010, e8905.
-
(2010)
PLoS One
, vol.5
, pp. e8905
-
-
Yang, H.1
-
209
-
-
0038487228
-
Function of the p97-Ufd1-Npl4 complex in retrotranslocation from the ER to the cytosol: dual recognition of nonubiquitinated polypeptide segments and polyubiquitin chains
-
Ye, Y., Meyer, H.H., Rapoport, T.A., Function of the p97-Ufd1-Npl4 complex in retrotranslocation from the ER to the cytosol: dual recognition of nonubiquitinated polypeptide segments and polyubiquitin chains. J. Cell Biol. 162 (2003), 71–84.
-
(2003)
J. Cell Biol.
, vol.162
, pp. 71-84
-
-
Ye, Y.1
Meyer, H.H.2
Rapoport, T.A.3
-
210
-
-
33745019916
-
Beta-amyloid peptides induces neuronal apoptosis via a mechanism independent of unfolded protein responses
-
Yu, M.S., et al. Beta-amyloid peptides induces neuronal apoptosis via a mechanism independent of unfolded protein responses. Apoptosis 11 (2006), 687–700.
-
(2006)
Apoptosis
, vol.11
, pp. 687-700
-
-
Yu, M.S.1
-
211
-
-
84862209309
-
A BAX/BAK and cyclophilin D-independent intrinsic apoptosis pathway
-
Zamorano, S., et al. A BAX/BAK and cyclophilin D-independent intrinsic apoptosis pathway. PLoS One, 7, 2012, e37782.
-
(2012)
PLoS One
, vol.7
, pp. e37782
-
-
Zamorano, S.1
-
212
-
-
0030933129
-
Conformation-independent binding of monoglucosylated ribonuclease B to calnexin
-
Zapun, A., et al. Conformation-independent binding of monoglucosylated ribonuclease B to calnexin. Cell 88 (1997), 29–38.
-
(1997)
Cell
, vol.88
, pp. 29-38
-
-
Zapun, A.1
-
213
-
-
84859609686
-
Huntington disease and the huntingtin protein
-
Zheng, Z., Diamond, M.I., Huntington disease and the huntingtin protein. Prog. Mol. Biol. Transl. Sci. 107 (2012), 189–214.
-
(2012)
Prog. Mol. Biol. Transl. Sci.
, vol.107
, pp. 189-214
-
-
Zheng, Z.1
Diamond, M.I.2
-
214
-
-
84923225433
-
Update on Huntington's disease: advances in care and emerging therapeutic options
-
Zielonka, D., Mielcarek, M., Landwehrmeyer, G.B., Update on Huntington's disease: advances in care and emerging therapeutic options. Park. Relat. Disord. 21 (2015), 169–178.
-
(2015)
Park. Relat. Disord.
, vol.21
, pp. 169-178
-
-
Zielonka, D.1
Mielcarek, M.2
Landwehrmeyer, G.B.3
-
215
-
-
0032054744
-
CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum
-
Zinszner, H., et al. CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum. Genes. Dev. 12 (1998), 982–995.
-
(1998)
Genes. Dev.
, vol.12
, pp. 982-995
-
-
Zinszner, H.1
-
216
-
-
84859577308
-
AAV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington's disease
-
Zuleta, A., et al. AAV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington's disease. Biochem. Biophys. Res. Commun. 420 (2012), 558–563.
-
(2012)
Biochem. Biophys. Res. Commun.
, vol.420
, pp. 558-563
-
-
Zuleta, A.1
|