-
1
-
-
82355180826
-
P62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS
-
Al-Sarraj S, King A, Troakes C et al (2011) p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72- linked FTLD and MND/ALS. Acta Neuropathol 122:691-702
-
(2011)
Acta Neuropathol
, vol.122
, pp. 691-702
-
-
Al-Sarraj, S.1
King, A.2
Troakes, C.3
-
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 et al (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351:602-611
-
(2006)
Biochem Biophys Res Commun
, vol.351
, pp. 602-611
-
-
Arai, T.1
Hasegawa, M.2
Akiyama, H.3
-
3
-
-
0028353603
-
Neurofilament light and polyadenylated mRNA levels are decreased in amyotrophic lateral sclerosis motor neurons
-
Bergeron C, Beric-Maskarel K, Muntasser S et al (1994) Neurofilament light and polyadenylated mRNA levels are decreased in amyotrophic lateral sclerosis motor neurons. J Neuropathol Exp Neurol 53:221-230
-
(1994)
J Neuropathol Exp Neurol
, vol.53
, pp. 221-230
-
-
Bergeron, C.1
Beric-Maskarel, K.2
Muntasser, S.3
-
4
-
-
84863393788
-
Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72
-
Boeve BF, Boylan KB, Graff-Radford NR et al (2012) Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72. Brain 135:765-783
-
(2012)
Brain
, vol.135
, pp. 765-783
-
-
Boeve, B.F.1
Boylan, K.B.2
Graff-Radford, N.R.3
-
5
-
-
77958519939
-
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
-
Bosco DA, Morfini G, Karabacak NM et al (2010) Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci 13:1396-1403
-
(2010)
Nat Neurosci
, vol.13
, pp. 1396-1403
-
-
Bosco, D.A.1
Morfini, G.2
Karabacak, N.M.3
-
6
-
-
0035943731
-
P190RhoGEF Binds to a destabilizing element in the 30 untranslated region of light neurofilament subunit mRNA and alters the stability of the transcript
-
Canete-Soler R, Wu J, Zhai J, Shamim M, Schlaepfer WW (2001) p190RhoGEF Binds to a destabilizing element in the 30 untranslated region of light neurofilament subunit mRNA and alters the stability of the transcript. J Biol Chem 276:32046-32050
-
(2001)
J Biol Chem
, vol.276
, pp. 32046-32050
-
-
Canete-Soler, R.1
Wu, J.2
Zhai, J.3
Shamim, M.4
Schlaepfer, W.W.5
-
7
-
-
0014336826
-
Proximal axonal enlargement in motor neuron disease
-
Carpenter S (1968) Proximal axonal enlargement in motor neuron disease. Neurology 18:841-851
-
(1968)
Neurology
, vol.18
, pp. 841-851
-
-
Carpenter, S.1
-
8
-
-
2442658908
-
DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)
-
Chen YZ, Bennett CL, Huynh HM et al (2004) DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4). Am J Hum Genet 74:1128-1135
-
(2004)
Am J Hum Genet
, vol.74
, pp. 1128-1135
-
-
Chen, Y.Z.1
Bennett, C.L.2
Huynh, H.M.3
-
9
-
-
84857522741
-
Clinical characteristics of patients with familial amyotrophic lateral sclerosis carrying the pathogenic GGGGCC hexanucleotide repeat expansion of C9ORF72
-
Chio A, Borghero G, Restagno G et al (2012) Clinical characteristics of patients with familial amyotrophic lateral sclerosis carrying the pathogenic GGGGCC hexanucleotide repeat expansion of C9ORF72. Brain 135:784-793
-
(2012)
Brain
, vol.135
, pp. 784-793
-
-
Chio, A.1
Borghero, G.2
Restagno, G.3
-
10
-
-
84857054634
-
Clinicopathological features in amyotrophic lateral sclerosis with expansions in C9ORF72
-
Cooper-Knock J, Hewitt C, Highley JR et al (2012) Clinicopathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain 135:751-764
-
(2012)
Brain
, vol.135
, pp. 751-764
-
-
Cooper-Knock, J.1
Hewitt, C.2
Highley, J.R.3
-
11
-
-
80054832080
-
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS
-
DeJesus-Hernandez M, Mackenzie IR, Boeve BF et al (2011) Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72:245-256
-
(2011)
Neuron
, vol.72
, pp. 245-256
-
-
Dejesus-Hernandez, M.1
MacKenzie, I.R.2
Boeve, B.F.3
-
12
-
-
80051563478
-
Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations
-
Deng HX, Bigio EH, Zhai H et al (2011) Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations. Arch Neurol 68:1057-1061
-
(2011)
Arch Neurol
, vol.68
, pp. 1057-1061
-
-
Deng, H.X.1
Bigio, E.H.2
Zhai, H.3
-
13
-
-
80052580969
-
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/ dementia
-
Deng HX, Chen W, Hong ST et al (2011) Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/ dementia. Nature 477:211-215
-
(2011)
Nature
, vol.477
, pp. 211-215
-
-
Deng, H.X.1
Chen, W.2
Hong, S.T.3
-
14
-
-
77952932485
-
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis
-
Deng HX, Zhai H, Bigio EH et al (2010) FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis. Ann Neurol 67:739-748
-
(2010)
Ann Neurol
, vol.67
, pp. 739-748
-
-
Deng, H.X.1
Zhai, H.2
Bigio, E.H.3
-
15
-
-
84868137686
-
Rho guanine nucleotide exchange factor is a NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis
-
doi: 10/1016/jneurobiolaging.2012.06.021
-
Droppelmann CA, Keller BA, Campos-Melo D, Volkening K, Strong MJ (2012) Rho guanine nucleotide exchange factor is a NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis. Neurobiol Aging. doi: 10/1016/jneurobiolaging.2012.06.021
-
(2012)
Neurobiol Aging
-
-
Droppelmann, C.A.1
Keller, B.A.2
Campos-Melo, D.3
Volkening, K.4
Strong, M.J.5
-
16
-
-
77956155218
-
Ataxin-2 intermediatelength polyglutamine expansions are associated with increased risk for ALS
-
Elden AC, Kim HJ, Hart MP et al (2010) Ataxin-2 intermediatelength polyglutamine expansions are associated with increased risk for ALS. Nature 466:1069-1075
-
(2010)
Nature
, vol.466
, pp. 1069-1075
-
-
Elden, A.C.1
Kim, H.J.2
Hart, M.P.3
-
17
-
-
80052968310
-
TDP-43 and FUS/TLS: Cellular functions and implications for neurodegeneration
-
Fiesel FC, Kahle PJ (2011) TDP-43 and FUS/TLS: cellular functions and implications for neurodegeneration. FEBS J 278: 3550-3568
-
(2011)
FEBS J
, vol.278
, pp. 3550-3568
-
-
Fiesel, F.C.1
Kahle, P.J.2
-
18
-
-
79958135335
-
Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis
-
Forsberg K, Andersen PM, Marklund SL, Brannstrom T (2011) Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis. Acta Neuropathol 121:623-634
-
(2011)
Acta Neuropathol
, vol.121
, pp. 623-634
-
-
Forsberg, K.1
Andersen, P.M.2
Marklund, S.L.3
Brannstrom, T.4
-
19
-
-
77955352066
-
Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients
-
Forsberg K, Jonsson PA, Andersen PM et al (2010) Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients. PLoS ONE 5:e11552
-
(2010)
PLoS ONE
, vol.5
-
-
Forsberg, K.1
Jonsson, P.A.2
Andersen, P.M.3
-
20
-
-
12844269876
-
Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNA
-
Ge WW, Wen W, Strong W, Leystra-Lantz C, Strong MJ (2005) Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNA. J Biol Chem 280:118-124
-
(2005)
J Biol Chem
, vol.280
, pp. 118-124
-
-
Ge, W.W.1
Wen, W.2
Strong, W.3
Leystra-Lantz, C.4
Strong, M.J.5
-
21
-
-
82755189310
-
On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia
-
Geser F, Prvulovic D, O'Dwyer L et al (2011) On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia. Prog Neurobiol 95:649-662
-
(2011)
Prog Neurobiol
, vol.95
, pp. 649-662
-
-
Geser, F.1
Prvulovic, D.2
O'dwyer, L.3
-
22
-
-
33645422711
-
ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis
-
Greenway MJ, Andersen PM, Russ C et al (2006) ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis. Nat Genet 38:411-413
-
(2006)
Nat Genet
, vol.38
, pp. 411-413
-
-
Greenway, M.J.1
Andersen, P.M.2
Russ, C.3
-
23
-
-
79954594082
-
Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders
-
Hortobagyi T, Troakes C, Nishimura AL et al (2011) Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders. Acta Neuropathol 121:519-527
-
(2011)
Acta Neuropathol
, vol.121
, pp. 519-527
-
-
Hortobagyi, T.1
Troakes, C.2
Nishimura, A.L.3
-
24
-
-
82355170711
-
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: An emerging molecular pathway and the significance of glial pathology
-
Ince PG, Highley JR, Kirby J et al (2011) Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Acta Neuropathol 122:657-671
-
(2011)
Acta Neuropathol
, vol.122
, pp. 657-671
-
-
Ince, P.G.1
Highley, J.R.2
Kirby, J.3
-
25
-
-
79954581188
-
Optineurin is colocalized with FUS in basophilic inclusions of ALS with FUS mutation and in basophilic inclusion body disease
-
Ito H, Fujita K, Nakamura M et al (2011) Optineurin is colocalized with FUS in basophilic inclusions of ALS with FUS mutation and in basophilic inclusion body disease. Acta Neuropathol 121:555-557
-
(2011)
Acta Neuropathol
, vol.121
, pp. 555-557
-
-
Ito, H.1
Fujita, K.2
Nakamura, M.3
-
27
-
-
0033055548
-
Recent advances in research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutations: Neuronal Lewy body-like hyaline inclusions and astrocytic hyaline inclusions
-
Kato S, Saito M, Hirano A, Ohama E (1999) Recent advances in research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutations: neuronal Lewy body-like hyaline inclusions and astrocytic hyaline inclusions. Histol Histopathol 14:973-989
-
(1999)
Histol Histopathol
, vol.14
, pp. 973-989
-
-
Kato, S.1
Saito, M.2
Hirano, A.3
Ohama, E.4
-
28
-
-
61349156118
-
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis
-
Kwiatkowski TJ Jr, Bosco DA, Leclerc AL et al (2009) Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 323:1205-1208
-
(2009)
Science
, vol.323
, pp. 1205-1208
-
-
Kwiatkowski Jr., T.J.1
Bosco, D.A.2
Leclerc, A.L.3
-
29
-
-
34447103093
-
TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
-
Kwong LK, Neumann M, Sampathu DM, Lee VM, Trojanowski JQ (2007) TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathol 114:63-70
-
(2007)
Acta Neuropathol
, vol.114
, pp. 63-70
-
-
Kwong, L.K.1
Neumann, M.2
Sampathu, D.M.3
Lee, V.M.4
Trojanowski, J.Q.5
-
30
-
-
77953890823
-
TDP-43 and FUS/TLS: Emerging roles in RNA processing and neurodegeneration
-
Lagier-Tourenne C, Polymenidou M, Cleveland DW (2010) TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet 19:R46-R64
-
(2010)
Hum Mol Genet
, vol.19
-
-
Lagier-Tourenne, C.1
Polymenidou, M.2
Cleveland, D.W.3
-
31
-
-
79953176451
-
Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients
-
Lee T, Li YR, Ingre C et al (2011) Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients. Hum Mol Genet 20:1697-1700
-
(2011)
Hum Mol Genet
, vol.20
, pp. 1697-1700
-
-
Lee, T.1
Li, Y.R.2
Ingre, C.3
-
32
-
-
58549102138
-
Mutant copper-zinc superoxide dismutase associated with amyotrophic lateral sclerosis binds to adenine/uridine-rich stability elements in the vascular endothelial growth factor 30-untranslated region
-
Li X, Lu L, Bush DJ et al (2009) Mutant copper-zinc superoxide dismutase associated with amyotrophic lateral sclerosis binds to adenine/uridine-rich stability elements in the vascular endothelial growth factor 30-untranslated region. J Neurochem 108:1032-1044
-
(2009)
J Neurochem
, vol.108
, pp. 1032-1044
-
-
Li, X.1
Lu, L.2
Bush, D.J.3
-
33
-
-
69249121554
-
Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis
-
Liu HN, Sanelli T, Horne P et al (2009) Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis. Ann Neurol 66:75-80
-
(2009)
Ann Neurol
, vol.66
, pp. 75-80
-
-
Liu, H.N.1
Sanelli, T.2
Horne, P.3
-
34
-
-
71749088420
-
Amyotrophic lateral sclerosis- linked mutant SOD1 sequesters Hu antigen R (HuR) and TIA-1-related protein (TIAR): Implications for impaired posttranscriptional regulation of vascular endothelial growth factor
-
Lu L, Wang S, Zheng L et al (2009) Amyotrophic lateral sclerosis- linked mutant SOD1 sequesters Hu antigen R (HuR) and TIA-1-related protein (TIAR): implications for impaired posttranscriptional regulation of vascular endothelial growth factor. J Biol Chem 284:33989-33998
-
(2009)
J Biol Chem
, vol.284
, pp. 33989-33998
-
-
Lu, L.1
Wang, S.2
Zheng, L.3
-
35
-
-
34547476995
-
Mutant Cu/Zn-superoxide dismutase associated with amyotrophic lateral sclerosis destabilizes vascular endothelial growth factor mRNA and downregulates its expression
-
Lu L, Zheng L, Viera L et al (2007) Mutant Cu/Zn-superoxide dismutase associated with amyotrophic lateral sclerosis destabilizes vascular endothelial growth factor mRNA and downregulates its expression. J Neurosci 27:7929-7938
-
(2007)
J Neurosci
, vol.27
, pp. 7929-7938
-
-
Lu, L.1
Zheng, L.2
Viera, L.3
-
36
-
-
34249946466
-
Pathological TDP- 43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
-
Mackenzie IR, Bigio EH, Ince PG et al (2007) Pathological TDP- 43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol 61:427-434
-
(2007)
Ann Neurol
, vol.61
, pp. 427-434
-
-
MacKenzie, I.R.1
Bigio, E.H.2
Ince, P.G.3
-
37
-
-
84858622829
-
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: A cross-sectional study
-
Majounie E, Renton AE, Mok K et al (2012) Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol 11:323-330
-
(2012)
Lancet Neurol
, vol.11
, pp. 323-330
-
-
Majounie, E.1
Renton, A.E.2
Mok, K.3
-
38
-
-
77952419246
-
Mutations of optineurin in amyotrophic lateral sclerosis
-
Maruyama H, Morino H, Ito H et al (2010) Mutations of optineurin in amyotrophic lateral sclerosis. Nature 465:223-226
-
(2010)
Nature
, vol.465
, pp. 223-226
-
-
Maruyama, H.1
Morino, H.2
Ito, H.3
-
39
-
-
0036723914
-
Selective loss of neurofilament expression in Cu/Zn superoxide dismutase (SOD1) linked amyotrophic lateral sclerosis
-
Menzies FM, Grierson AJ, Cookson MR et al (2002) Selective loss of neurofilament expression in Cu/Zn superoxide dismutase (SOD1) linked amyotrophic lateral sclerosis. J Neurochem 82:1118-1128
-
(2002)
J Neurochem
, vol.82
, pp. 1118-1128
-
-
Menzies, F.M.1
Grierson, A.J.2
Cookson, M.R.3
-
40
-
-
0027478805
-
Peripherin immunoreactive structures in amyotrophic lateral sclerosis
-
Migheli A, Pezzulo T, Attanasio A, Schiffer D (1993) Peripherin immunoreactive structures in amyotrophic lateral sclerosis. Lab Invest 68:185-191
-
(1993)
Lab Invest
, vol.68
, pp. 185-191
-
-
Migheli, A.1
Pezzulo, T.2
Attanasio, A.3
Schiffer, D.4
-
41
-
-
33750998792
-
Innate immunity in amyotrophic lateral sclerosis
-
Moisse K, Strong MJ (2006) Innate immunity in amyotrophic lateral sclerosis. Biochim Biophys Acta 1762:1083-1093
-
(2006)
Biochim Biophys Acta
, vol.1762
, pp. 1083-1093
-
-
Moisse, K.1
Strong, M.J.2
-
42
-
-
58049221032
-
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury
-
Moisse K, Volkening K, Leystra-Lantz C et al (2009) Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury. Brain Res 1249: 202-211
-
(2009)
Brain Res
, vol.1249
, pp. 202-211
-
-
Moisse, K.1
Volkening, K.2
Leystra-Lantz, C.3
-
43
-
-
1842789629
-
Expression of ubiquitin-binding protein p62 in ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis with dementia: Analysis of five autopsy cases with broad clinicopathological spectrum
-
Nakano T, Nakaso K, Nakashima K, Ohama E (2004) Expression of ubiquitin-binding protein p62 in ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis with dementia: analysis of five autopsy cases with broad clinicopathological spectrum. Acta Neuropathol 107:359-364
-
(2004)
Acta Neuropathol
, vol.107
, pp. 359-364
-
-
Nakano, T.1
Nakaso, K.2
Nakashima, K.3
Ohama, E.4
-
44
-
-
33749632259
-
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
-
Neumann M, Sampathu DM, Kwong LK et al (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130-133
-
(2006)
Science
, vol.314
, pp. 130-133
-
-
Neumann, M.1
Sampathu, D.M.2
Kwong, L.K.3
-
45
-
-
80055026924
-
An autopsy case of SOD1-related ALS with TDP-43 positive inclusions
-
Okamoto Y, Ihara M, Urushitani M et al (2011) An autopsy case of SOD1-related ALS with TDP-43 positive inclusions. Neurology 77:1993-1995
-
(2011)
Neurology
, vol.77
, pp. 1993-1995
-
-
Okamoto, Y.1
Ihara, M.2
Urushitani, M.3
-
46
-
-
79960839810
-
Optineurin in neurodegenerative diseases
-
Osawa T, Mizuno Y, Fujita Y et al (2011) Optineurin in neurodegenerative diseases. Neuropathology 31:569-574
-
(2011)
Neuropathology
, vol.31
, pp. 569-574
-
-
Osawa, T.1
Mizuno, Y.2
Fujita, Y.3
-
47
-
-
84864981763
-
Advances in understanding the molecular basis of frontotemporal dementia
-
Rademakers R, Neumann M, Mackenzie IR (2012) Advances in understanding the molecular basis of frontotemporal dementia. Nat Rev Neurol 8(8):423-434
-
(2012)
Nat Rev Neurol
, vol.8
, Issue.8
, pp. 423-434
-
-
Rademakers, R.1
Neumann, M.2
MacKenzie, I.R.3
-
48
-
-
80054837386
-
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21- linked ALS-FTD
-
Renton AE, Majounie E, Waite A et al (2011) A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21- linked ALS-FTD. Neuron 72:257-268
-
(2011)
Neuron
, vol.72
, pp. 257-268
-
-
Renton, A.E.1
Majounie, E.2
Waite, A.3
-
49
-
-
34249313704
-
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS
-
Robertson J, Sanelli T, Xiao S et al (2007) Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS. Neurosci Lett 420:128-132
-
(2007)
Neurosci Lett
, vol.420
, pp. 128-132
-
-
Robertson, J.1
Sanelli, T.2
Xiao, S.3
-
50
-
-
0027401203
-
Mutations in Cu/ Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
-
Rosen DR, Siddique T, Patterson D et al (1993) Mutations in Cu/ Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362:59-62
-
(1993)
Nature
, vol.362
, pp. 59-62
-
-
Rosen, D.R.1
Siddique, T.2
Patterson, D.3
-
51
-
-
0025836354
-
Ubiquitin in motor neuron disease: Study at the light and electron microscope
-
Schiffer D, Autilio-Gambetti L, Chio A et al (1991) Ubiquitin in motor neuron disease: study at the light and electron microscope. J Neuropathol Exp Neurol 50:463-473
-
(1991)
J Neuropathol Exp Neurol
, vol.50
, pp. 463-473
-
-
Schiffer, D.1
Autilio-Gambetti, L.2
Chio, A.3
-
52
-
-
0029842923
-
Immunohistochemical study on superoxide dismutases in spinal cords from autopsied patients with amyotrophic lateral sclerosis
-
Shibata N, Asayama K, Hirano A, Kobayashi M (1996) Immunohistochemical study on superoxide dismutases in spinal cords from autopsied patients with amyotrophic lateral sclerosis. Dev Neurosci 18:492-498
-
(1996)
Dev Neurosci
, vol.18
, pp. 492-498
-
-
Shibata, N.1
Asayama, K.2
Hirano, A.3
Kobayashi, M.4
-
53
-
-
0032253214
-
P62 and the sequestosome, a novel mechanism for protein metabolism
-
Shin J (1998) P62 and the sequestosome, a novel mechanism for protein metabolism. Arch Pharm Res 21:629-633
-
(1998)
Arch Pharm Res
, vol.21
, pp. 629-633
-
-
Shin, J.1
-
54
-
-
42049120518
-
Progranulin genetic variability contributes to amyotrophic lateral sclerosis
-
Sleegers K, Brouwers N, Maurer-Stroh S et al (2008) Progranulin genetic variability contributes to amyotrophic lateral sclerosis. Neurology 71:253-259
-
(2008)
Neurology
, vol.71
, pp. 253-259
-
-
Sleegers, K.1
Brouwers, N.2
Maurer-Stroh, S.3
-
55
-
-
0038693216
-
The basic aspects of therapeutics in amyotrophic lateral sclerosis
-
Strong MJ (2003) The basic aspects of therapeutics in amyotrophic lateral sclerosis. Pharmacol Ther 98:379-414
-
(2003)
Pharmacol Ther
, vol.98
, pp. 379-414
-
-
Strong, M.J.1
-
56
-
-
54049140363
-
The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis
-
Strong MJ (2008) The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 9:323-338
-
(2008)
Amyotroph Lateral Scler
, vol.9
, pp. 323-338
-
-
Strong, M.J.1
-
57
-
-
71049166754
-
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)
-
Strong MJ (2010) The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS). J Neurol Sci 288:1-12
-
(2010)
J Neurol Sci
, vol.288
, pp. 1-12
-
-
Strong, M.J.1
-
58
-
-
67651160559
-
Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis
-
Strong MJ, Grace GM, Freedman M et al (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 10:131-146
-
(2009)
Amyotroph Lateral Scler
, vol.10
, pp. 131-146
-
-
Strong, M.J.1
Grace, G.M.2
Freedman, M.3
-
59
-
-
23844538993
-
The pathobiology of amyotrophic lateral sclerosis: A proteinopathy?
-
Strong MJ, Kesavapany S, Pant HC (2005) The pathobiology of amyotrophic lateral sclerosis: a proteinopathy? J Neuropathol Exp Neurol 64:649-664
-
(2005)
J Neuropathol Exp Neurol
, vol.64
, pp. 649-664
-
-
Strong, M.J.1
Kesavapany, S.2
Pant, H.C.3
-
60
-
-
34249751076
-
TDP43 is a human low molecular weight neurofilament (hNFL) mRNAbinding protein
-
Strong MJ, Volkening K, Hammond R et al (2007) TDP43 is a human low molecular weight neurofilament (hNFL) mRNAbinding protein. Mol Cell Neurosci 35:320-327
-
(2007)
Mol Cell Neurosci
, vol.35
, pp. 320-327
-
-
Strong, M.J.1
Volkening, K.2
Hammond, R.3
-
61
-
-
64049116333
-
Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis
-
Sumi H, Kato S, Mochimaru Y et al (2009) Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 68:37-47
-
(2009)
J Neuropathol Exp Neurol
, vol.68
, pp. 37-47
-
-
Sumi, H.1
Kato, S.2
Mochimaru, Y.3
-
62
-
-
38349082689
-
Creation of engineered human embryonic stem cell lines using phiC31 integrase
-
Thyagarajan B, Liu Y, Shin S et al (2008) Creation of engineered human embryonic stem cell lines using phiC31 integrase. Stem Cells 26:119-126
-
(2008)
Stem Cells
, vol.26
, pp. 119-126
-
-
Thyagarajan, B.1
Liu, Y.2
Shin, S.3
-
63
-
-
0035008830
-
Site-specific genomic integration in mammalian cells mediated by phage phiC31 integrase
-
Thyagarajan B, Olivares EC, Hollis RP, Ginsburg DS, Calos MP (2001) Site-specific genomic integration in mammalian cells mediated by phage phiC31 integrase. Mol Cell Biol 21:3926-3934
-
(2001)
Mol Cell Biol
, vol.21
, pp. 3926-3934
-
-
Thyagarajan, B.1
Olivares, E.C.2
Hollis, R.P.3
Ginsburg, D.S.4
Calos, M.P.5
-
64
-
-
0029809134
-
P62, a phosphotyrosine-independent ligand of the SH2 domain of p56lck, belongs to a new class of ubiquitin-binding proteins
-
Vadlamudi RK, Joung I, Strominger JL, Shin J (1996) p62, a phosphotyrosine-independent ligand of the SH2 domain of p56lck, belongs to a new class of ubiquitin-binding proteins. J Biol Chem 271:20235-20237
-
(1996)
J Biol Chem
, vol.271
, pp. 20235-20237
-
-
Vadlamudi, R.K.1
Joung, I.2
Strominger, J.L.3
Shin, J.4
-
65
-
-
79959653680
-
Expanded ATXN2 CAG repeat size in ALS identifies genetic overlap between ALS and SCA2
-
Van Damme P, Veldink JH, van Blitterswijk M et al (2011) Expanded ATXN2 CAG repeat size in ALS identifies genetic overlap between ALS and SCA2. Neurology 76:2066-2072
-
(2011)
Neurology
, vol.76
, pp. 2066-2072
-
-
Van Damme, P.1
Veldink, J.H.2
Van Blitterswijk, M.3
-
66
-
-
61349162349
-
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6
-
Vance C, Rogelj B, Hortobagyi T et al (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 323:1208-1211
-
(2009)
Science
, vol.323
, pp. 1208-1211
-
-
Vance, C.1
Rogelj, B.2
Hortobagyi, T.3
-
67
-
-
77649317237
-
Human low molecular weight neurofilament (NFL) mRNA interacts with a predicted p190RhoGEF homologue (RGNEF) in humans
-
Volkening K, Leystra-Lantz C, Strong MJ (2010) Human low molecular weight neurofilament (NFL) mRNA interacts with a predicted p190RhoGEF homologue (RGNEF) in humans. Amyotroph Lateral Scler 11:97-103
-
(2010)
Amyotroph Lateral Scler
, vol.11
, pp. 97-103
-
-
Volkening, K.1
Leystra-Lantz, C.2
Strong, M.J.3
-
68
-
-
0033763630
-
Characterization of neuronal intermediate filament protein expression in cervical spinal motor neurons in sporadic amyotrophic lateral sclerosis (ALS)
-
Wong NK, He BP, Strong MJ (2000) Characterization of neuronal intermediate filament protein expression in cervical spinal motor neurons in sporadic amyotrophic lateral sclerosis (ALS). J Neuropathol Exp Neurol 59:972-982
-
(2000)
J Neuropathol Exp Neurol
, vol.59
, pp. 972-982
-
-
Wong, N.K.1
He, B.P.2
Strong, M.J.3
-
69
-
-
31944452549
-
In vivo identification of ribonucleoprotein-RNA interactions
-
Zielinski J, Kilk K, Peritz T et al (2006) In vivo identification of ribonucleoprotein-RNA interactions. Proc Natl Acad Sci USA 103:1557-1562
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 1557-1562
-
-
Zielinski, J.1
Kilk, K.2
Peritz, T.3
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