-
1
-
-
33751250197
-
Genetics of familial and sporadic amyotrophic lateral sclerosis
-
DOI 10.1016/j.bbadis.2006.01.004, PII S0925443906000068
-
Gros-Louis F., Gaspar C., Rouleau G. A., Genetics of familial and sporadic amyotrophic lateral sclerosis Biochimica et (Pubitemid 44781174)
-
(2006)
Biochimica et Biophysica Acta - Molecular Basis of Disease
, vol.1762
, Issue.11-12
, pp. 956-972
-
-
Gros-Louis, F.1
Gaspar, C.2
Rouleau, G.A.3
-
2
-
-
70350075024
-
Genetics of motor neuron disorders: New insights into pathogenic mechanisms
-
Dion P. A., Daoud H., Rouleau G. A., Genetics of motor neuron disorders: new insights into pathogenic mechanisms Nature Reviews Genetics 2009 10 11 769 782
-
(2009)
Nature Reviews Genetics
, vol.10
, Issue.11
, pp. 769-782
-
-
Dion, P.A.1
Daoud, H.2
Rouleau, G.A.3
-
4
-
-
0027401203
-
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
-
DOI 10.1038/362059a0
-
Rosen D. R., Siddique T., Patterson D., Figlewicz D. A., Sapp P., Hentati A., Donaldson D., Goto J., O'Regan J. P., Deng H. X., Rahmani Z., Krizus A., McKenna-Yasek D., Cayabyab A., Gaston S. M., Berger R., Tanzi R. E., Halperin J. J., Herzfeldt B., Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis Nature 1993 362 6415 59 62 (Pubitemid 23087289)
-
(1993)
Nature
, vol.362
, Issue.6415
, pp. 59-62
-
-
Rosen, D.R.1
Siddique, T.2
Patterson, D.3
Figlewicz, D.A.4
Sapp, P.5
Hentati, A.6
Donaldson, D.7
Goto, J.8
O'Regan, J.P.9
Deng, H.-X.10
Rahmani, Z.11
Krizus, A.12
McKenna-Yasek, D.13
Cayabyab, A.14
Gaston, S.M.15
Berger, R.16
Tanzi, R.E.17
Halperin, J.J.18
Herzfeldt, B.19
-
5
-
-
41149180753
-
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
-
DOI 10.1126/science.1154584
-
Sreedharan J., Blair I. P., Tripathi V. B., Hu X., Vance C., Rogelj B., Ackerley S., Durnall J. C., Williams K. L., Buratti E., Baralle F., De Belleroche J., Mitchell J. D., Leigh P. N., Al-Chalabi A., Miller C. C., Nicholson G., Shaw C. E., TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis Science 2008 319 5870 1668 1672 (Pubitemid 351432505)
-
(2008)
Science
, vol.319
, Issue.5870
, pp. 1668-1672
-
-
Sreedharan, J.1
Blair, I.P.2
Tripathi, V.B.3
Hu, X.4
Vance, C.5
Rogelj, B.6
Ackerley, S.7
Durnall, J.C.8
Williams, K.L.9
Buratti, E.10
Baralle, F.11
De Belleroche, J.12
Mitchell, J.D.13
Leigh, P.N.14
Al-Chalabi, A.15
Miller, C.C.16
Nicholson, G.17
Shaw, C.E.18
-
6
-
-
42649120983
-
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
-
DOI 10.1038/ng.132, PII NG132
-
Kabashi E., Valdmanis P. N., Dion P., Spiegelman D., McConkey B. J., Velde C. V., Bouchard J. P., Lacomblez L., Pochigaeva K., Salachas F., Pradat P. F., Camu W., Meininger V., Dupre N., Rouleau G. A., TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis Nature Genetics 2008 40 5 572 574 (Pubitemid 351601218)
-
(2008)
Nature Genetics
, vol.40
, Issue.5
, pp. 572-574
-
-
Kabashi, E.1
Valdmanis, P.N.2
Dion, P.3
Spiegelman, D.4
McConkey, B.J.5
Velde, C.V.6
Bouchard, J.-P.7
Lacomblez, L.8
Pochigaeva, K.9
Salachas, F.10
Pradat, P.-F.11
Camu, W.12
Meininger, V.13
Dupre, N.14
Rouleau, G.A.15
-
7
-
-
41949100148
-
TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: A genetic and histopathological analysis
-
Van Deerlin V. M., Leverenz J. B., Bekris L. M., Bird T. D., Yuan W., Elman L. B., Clay D., Wood E. M., Chen-Plotkin A. S., Martinez-Lage M., Steinbart E., McCluskey L., Grossman M., Neumann M., Wu I. L., Yang W. S., Kalb R., Galasko D. R., Montine T. J., Trojanowski J. Q., Lee V. M. Y., Schellenberg G. D., Yu C. E., TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis The Lancet Neurology 2008 7 5 409 416
-
(2008)
The Lancet Neurology
, vol.7
, Issue.5
, pp. 409-416
-
-
Van Deerlin, V.M.1
Leverenz, J.B.2
Bekris, L.M.3
Bird, T.D.4
Yuan, W.5
Elman, L.B.6
Clay, D.7
Wood, E.M.8
Chen-Plotkin, A.S.9
Martinez-Lage, M.10
Steinbart, E.11
McCluskey, L.12
Grossman, M.13
Neumann, M.14
Wu, I.L.15
Yang, W.S.16
Kalb, R.17
Galasko, D.R.18
Montine, T.J.19
Trojanowski, J.Q.20
Lee, V.M.Y.21
Schellenberg, G.D.22
Yu, C.E.23
more..
-
8
-
-
41949119043
-
TDP-43 A315T mutation in familial motor neuron disease
-
DOI 10.1002/ana.21344
-
Gitcho M. A., Baloh R. H., Chakraverty S., Mayo K., Norton J. B., Levitch D., Hatanpaa K. J., White C. L., Bigio E. H., Caselli R., Baker M., Al-Lozi M. T., Morris J. C., Pestronk A., Rademakers R., Goate A. M., Cairns N. J., TDP-43 A315T mutation in familial motor neuron disease Annals of Neurology 2008 63 4 535 538 (Pubitemid 351614720)
-
(2008)
Annals of Neurology
, vol.63
, Issue.4
, pp. 535-538
-
-
Gitcho, M.A.1
Baloh, R.H.2
Chakraverty, S.3
Mayo, K.4
Norton, J.B.5
Levitch, D.6
Hatanpaa, K.J.7
White III, C.L.8
Bigio, E.H.9
Caselli, R.10
Baker, M.11
Al-Lozi, M.T.12
Morris, J.C.13
Pestronk, A.14
Rademakers, R.15
Goate, A.M.16
Cairns, N.J.17
-
9
-
-
42949094584
-
TDP-43 mutation in familial amyotrophic lateral sclerosis
-
DOI 10.1002/ana.21392
-
Yokoseki A., Shiga A., Tan C. F., Tagawa A., Kaneko H., Koyama A., Eguchi H., Tsujino A., Ikeuchi T., Kakita A., Okamoto K., Nishizawa M., Takahashi H., Onodera O., TDP-43 mutation in familial amyotrophic lateral sclerosis Annals of Neurology 2008 63 4 538 542 (Pubitemid 351614721)
-
(2008)
Annals of Neurology
, vol.63
, Issue.4
, pp. 538-542
-
-
Yokoseki, A.1
Shiga, A.2
Tan, C.-F.3
Tagawa, A.4
Kaneko, H.5
Koyama, A.6
Eguchi, H.7
Tsujino, A.8
Ikeuchi, T.9
Kakita, A.10
Okamoto, K.11
Nishizawa, M.12
Takahashi, H.13
Onodera, O.14
-
10
-
-
61349156118
-
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis
-
Kwiatkowski T. J., Bosco D. A., LeClerc A. L., Tamrazian E., Vanderburg C. R., Russ C., Davis A., Gilchrist J., Kasarskis E. J., Munsat T., Valdmanis P., Rouleau G. A., Hosler B. A., Cortelli P., De Jong P. J., Yoshinaga Y., Haines J. L., Pericak-Vance M. A., Yan J., Ticozzi N., Siddique T., McKenna-Yasek D., Sapp P. C., Horvitz H. R., Landers J. E., Brown R. H., Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis Science 2009 323 5918 1205 1208
-
(2009)
Science
, vol.323
, Issue.5918
, pp. 1205-1208
-
-
Kwiatkowski, T.J.1
Bosco, D.A.2
Leclerc, A.L.3
Tamrazian, E.4
Vanderburg, C.R.5
Russ, C.6
Davis, A.7
Gilchrist, J.8
Kasarskis, E.J.9
Munsat, T.10
Valdmanis, P.11
Rouleau, G.A.12
Hosler, B.A.13
Cortelli, P.14
De Jong, P.J.15
Yoshinaga, Y.16
Haines, J.L.17
Pericak-Vance, M.A.18
Yan, J.19
Ticozzi, N.20
Siddique, T.21
McKenna-Yasek, D.22
Sapp, P.C.23
Horvitz, H.R.24
Landers, J.E.25
Brown, R.H.26
more..
-
11
-
-
61349162349
-
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6
-
Vance C., Rogelj B., Hortobgyi T., De Vos K. J., Nishimura A. L., Sreedharan J., Hu X., Smith B., Ruddy D., Wright P., Ganesalingam J., Williams K. L., Tripathi V., Al-Saraj S., Al-Chalabi A., Leigh P. N., Blair I. P., Nicholson G., De Belleroche J., Gallo J. M., Miller C. C., Shaw C. E., Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6 Science 2009 323 5918 1208 1211
-
(2009)
Science
, vol.323
, Issue.5918
, pp. 1208-1211
-
-
Vance, C.1
Rogelj, B.2
Hortobgyi, T.3
De Vos, K.J.4
Nishimura, A.L.5
Sreedharan, J.6
Hu, X.7
Smith, B.8
Ruddy, D.9
Wright, P.10
Ganesalingam, J.11
Williams, K.L.12
Tripathi, V.13
Al-Saraj, S.14
Al-Chalabi, A.15
Leigh, P.N.16
Blair, I.P.17
Nicholson, G.18
De Belleroche, J.19
Gallo, J.M.20
Miller, C.C.21
Shaw, C.E.22
more..
-
12
-
-
0034785483
-
A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2
-
DOI 10.1038/ng1001-166
-
Hadano S., Hand C. K., Osuga H., Yanagisawa Y., Otomo A., Devon R. S., Miyamoto N., Showguchi-Miyata J., Okada Y., Singaraja R., Figlewicz D. A., Kwiatkowski T., Hosler B. A., Sagie T., Skaug J., Nasir J., Brown R. H., Scherer S. W., Rouleau G. A., Hayden M. R., Ikeda J. E., A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2 Nature Genetics 2001 29 2 166 173 (Pubitemid 32952653)
-
(2001)
Nature Genetics
, vol.29
, Issue.2
, pp. 166-173
-
-
Hadano, S.1
Hand, C.K.2
Osuga, H.3
Yanagisawa, Y.4
Otomo, A.5
Devon, R.S.6
Miyamoto, N.7
Showguchi-Miyata, J.8
Okada, Y.9
Singaraja, R.10
Figlewicz, D.A.11
Kwiatkowski, T.12
Hosler, B.A.13
Sagie, T.14
Skaug, J.15
Nasir, J.16
Brown, R.H.17
Scherer, S.W.18
Rouleau, G.A.19
Hayden, M.R.20
Ikeda, J.-E.21
more..
-
13
-
-
2442658908
-
DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)
-
DOI 10.1086/421054
-
Chen Y. Z., Bennett C. L., Huynh H. M., Blair I. P., Puls I., Irobi J., Dierick I., Abel A., Kennerson M. L., Rabin B. A., Nicholson G. A., Auer-Grumbach M., Wagner K., De Jonghe P., Griffin J. W., Fischbeck K. H., Timmerman V., Cornblath D. R., Chance P. F., DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4) American Journal of Human Genetics 2004 74 6 1128 1135 (Pubitemid 38669312)
-
(2004)
American Journal of Human Genetics
, vol.74
, Issue.6
, pp. 1128-1135
-
-
Chen, Y.-Z.1
Bennett, C.L.2
Huynh, H.M.3
Blair, I.P.4
Puls, I.5
Irobi, J.6
Dierick, I.7
Abel, A.8
Kennerson, M.L.9
Rabin, B.A.10
Nicholson, G.A.11
Auer-Grumbach, M.12
Wagner, K.13
De Jonghe, P.14
Griffin, J.W.15
Fischbeck, K.H.16
Timmerman, V.17
Cornblath, D.R.18
Chance, P.F.19
-
14
-
-
77249126425
-
SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis
-
Orlacchio A., Babalini C., Borreca A., Patrono C., Massa R., Basaran S., Munhoz R. P., Rogaeva E. A., St George-Hyslop P. H., Bernardi G., Kawarai T., SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis Brain 2010 133 2 591 598
-
(2010)
Brain
, vol.133
, Issue.2
, pp. 591-598
-
-
Orlacchio, A.1
Babalini, C.2
Borreca, A.3
Patrono, C.4
Massa, R.5
Basaran, S.6
Munhoz, R.P.7
Rogaeva, E.A.8
St George-Hyslop, P.H.9
Bernardi, G.10
Kawarai, T.11
-
15
-
-
6344257200
-
A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis
-
DOI 10.1086/425287
-
Nishimura A. L., Mitne-Neto M., Silva H. C. A., Richieri-Costa A., Middleton S., Cascio D., Kok F., Oliveira J. R. M., Gillingwater T., Webb J., Skehel P., Zatz M., A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis American Journal of Human Genetics 2004 75 5 822 831 (Pubitemid 39390489)
-
(2004)
American Journal of Human Genetics
, vol.75
, Issue.5
, pp. 822-831
-
-
Nishimura, A.L.1
Mitne-Neto, M.2
Silva, H.C.A.3
Richieri-Costa, A.4
Middleton, S.5
Cascio, D.6
Kok, F.7
Oliveira, J.R.M.8
Gillingwater, T.9
Webb, J.10
Skehel, P.11
Zatz, M.12
-
16
-
-
33645422711
-
ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis
-
Greenway M. J., Andersen P. M., Russ G., Ennis S., Cashman S., Donaghy C., Patterson V., Swingler R., Kieran D., Prehn J., Morrison K. E., Green A., Acharya K. R., Brown R. H., Hardiman O., ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis Nature Genetics 2006 38 4 411 413
-
(2006)
Nature Genetics
, vol.38
, Issue.4
, pp. 411-413
-
-
Greenway, M.J.1
Andersen, P.M.2
Russ, G.3
Ennis, S.4
Cashman, S.5
Donaghy, C.6
Patterson, V.7
Swingler, R.8
Kieran, D.9
Prehn, J.10
Morrison, K.E.11
Green, A.12
Acharya, K.R.13
Brown, R.H.14
Hardiman, O.15
-
17
-
-
77952419246
-
Mutations of optineurin in amyotrophic lateral sclerosis
-
Maruyama H., Morino H., Ito H., Izumi Y., Kato H., Watanabe Y., Kinoshita Y., Kamada M., Nodera H., Suzuki H., Komure O., Matsuura S., Kobatake K., Morimoto N., Abe K., Suzuki N., Aoki M., Kawata A., Hirai T., Kato T., Ogasawara K., Hirano A., Takumi T., Kusaka H., Hagiwara K., Kaji R., Kawakami H., Mutations of optineurin in amyotrophic lateral sclerosis Nature 2010 465 7295 223 226
-
(2010)
Nature
, vol.465
, Issue.7295
, pp. 223-226
-
-
Maruyama, H.1
Morino, H.2
Ito, H.3
Izumi, Y.4
Kato, H.5
Watanabe, Y.6
Kinoshita, Y.7
Kamada, M.8
Nodera, H.9
Suzuki, H.10
Komure, O.11
Matsuura, S.12
Kobatake, K.13
Morimoto, N.14
Abe, K.15
Suzuki, N.16
Aoki, M.17
Kawata, A.18
Hirai, T.19
Kato, T.20
Ogasawara, K.21
Hirano, A.22
Takumi, T.23
Kusaka, H.24
Hagiwara, K.25
Kaji, R.26
Kawakami, H.27
more..
-
18
-
-
0037382240
-
Mutant dynactin in motor neuron disease
-
DOI 10.1038/ng1123
-
Puls I., Jonnakuty C., LaMonte B. H., Holzbaur E. L. F., Tokito M., Mann E., Floeter M. K., Bidus K., Drayna D., Oh S. J., Brown R. H., Ludlow C. L., Fischbeck K. H., Mutant dynactin in motor neuron disease Nature Genetics 2003 33 4 455 456 (Pubitemid 36390002)
-
(2003)
Nature Genetics
, vol.33
, Issue.4
, pp. 455-456
-
-
Puls, I.1
Jonnakuty, C.2
LaMonte, B.H.3
Holzbaur, E.L.F.4
Tokito, M.5
Mann, E.6
Floeter, M.K.7
Bidus, K.8
Drayna, D.9
Oh, S.J.10
Brown Jr., R.H.11
Ludlow, C.L.12
Fischbeck, K.H.13
-
19
-
-
0014368955
-
An hereditary motor neurone disease with progressive denervation of muscle in the mouse: The mutant 'wobbler'
-
Duchen L. W., Strich S. J., An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler' Journal of Neurology Neurosurgery and Psychiatry 1968 31 6 535 542
-
(1968)
Journal of Neurology Neurosurgery and Psychiatry
, vol.31
, Issue.6
, pp. 535-542
-
-
Duchen, L.W.1
Strich, S.J.2
-
20
-
-
0029257498
-
Neuromuscular degeneration (nmd): A mutation on mouse chromosome 19 that causes motor neuron degeneration
-
Cook S. A., Johnson K. R., Bronson R. T., Davisson M. T., Neuromuscular degeneration (nmd): a mutation on mouse chromosome 19 that causes motor neuron degeneration Mammalian Genome 1995 6 3 187 191
-
(1995)
Mammalian Genome
, vol.6
, Issue.3
, pp. 187-191
-
-
Cook, S.A.1
Johnson, K.R.2
Bronson, R.T.3
Davisson, M.T.4
-
21
-
-
0025777705
-
A new mouse mutant with progressive motor neuronopathy
-
Schmalbruch H., Jensen H. J. S., Bjaerg M., Kamieniecka Z., Kurland L., A new mouse mutant with progressive motor neuronopathy Journal of Neuropathology and Experimental Neurology 1991 50 3 192 204
-
(1991)
Journal of Neuropathology and Experimental Neurology
, vol.50
, Issue.3
, pp. 192-204
-
-
Schmalbruch, H.1
Jensen, H.J.S.2
Bjaerg, M.3
Kamieniecka, Z.4
Kurland, L.5
-
22
-
-
0035933649
-
SHIRPA, a protocol for behavioral assessment: Validation for longitudinal study of neurological dysfunction in mice
-
DOI 10.1016/S0304-3940(01)01885-7, PII S0304394001018857
-
Rogers D. C., Peters JO., Martin J. E., Ball S., Nicholson S. J., Witherden A. S., Hafezparast M., Latcham J., Robinson T. L., Quilter C. A., Fisher E. M. C., SHIRPA, a protocol for behavioral assessment: validation for longitudinal study of neurological dysfunction in mice Neuroscience Letters 2001 306 1-2 89 92 (Pubitemid 32524305)
-
(2001)
Neuroscience Letters
, vol.306
, Issue.1-2
, pp. 89-92
-
-
Rogers, D.C.1
Peters, J.2
Martin, J.E.3
Ball, S.4
Nicholson, S.J.5
Witherden, A.S.6
Hafezparast, M.7
Latcham, J.8
Robinson, T.L.9
Quilter, C.A.10
Fisher, E.M.C.11
-
23
-
-
0034425715
-
Genome-wide, large-scale production of mutant mice by ENU mutagenesis
-
DOI 10.1038/78146
-
De Angelis M. H., Flaswinkel H., Fuchs H., Rathkolb B., Soewarto D., Marschall S., Heffner S., Pargent W., Wuensch K., Jung M., Reis A., Richter T., Alessandrini F., Jakob T., Fuchs E., Kolb H., Kremmer E., Schaeble K., Rollinski B., Roscher A., Peters C., Meitinger T., Strom T., Steckler T., Holsboer F., Klopstock T., Gekeler F., Schindewolf C., Jung T., Avraham K., Behrendt H., Ring J., Zimmer A., Schughart K., Pfeffer K., Wolf E., Balling R., Genome-wide, large-scale production of mutant mice by ENU mutagenesis Nature Genetics 2000 25 4 444 447 (Pubitemid 32983440)
-
(2000)
Nature Genetics
, vol.25
, Issue.4
, pp. 444-447
-
-
De Angelis, M.H.1
Flaswinkel, H.2
Fuchs, H.3
Rathkolb, B.4
Soewarto, D.5
Marschall, S.6
Heffner, S.7
Pargent, W.8
Wuensch, K.9
Jung, M.10
Reis, A.11
Richter, T.12
Alessandrini, F.13
Jakob, T.14
Fuchs, E.15
Kolb, H.16
Kremmer, E.17
Schaeble, K.18
Rollinski, B.19
Roscher, A.20
Peters, C.21
Meitinger, T.22
Strom, T.23
Steckler, T.24
Holsboer, F.25
Klopstock, T.26
Gekeler, F.27
Schindewolf, C.28
Jung, T.29
Avraham, K.30
Behrendt, H.31
Ring, J.32
Zimmer, A.33
Schughart, K.34
Pfeffer, K.35
Wolf, E.36
Balling, R.37
more..
-
24
-
-
0037171031
-
Genetic modifiers that aggravate the neurological phenotype of the wobbler mouse
-
Ulbrich M., Schmidt V. C., Ronsiek M., Mussmann A., Bartsch J. W., Augustin M., Jockusch H., Schmitt-John T., Genetic modifiers that aggravate the neurological phenotype of the wobbler mouse NeuroReport 2002 13 4 535 539 (Pubitemid 34293205)
-
(2002)
NeuroReport
, vol.13
, Issue.4
, pp. 535-539
-
-
Ulbrich, M.1
Schmidt, V.C.2
Ronsiek, M.3
Mussmann, A.4
Bartsch, J.W.5
Augustin, M.6
Jockusch, H.7
Schmitt-John, T.8
-
25
-
-
0026594583
-
Wobbler, a mutation affecting motoneuron survival and gonadal functions in the mouse, maps to proximal chromosome 11
-
Kaupmann K., Simon-Chazottes D., Guenet J. L., Jockusch H., Wobbler, a mutation affecting motoneuron survival and gonadal functions in the mouse, maps to proximal chromosome 11 Genomics 1992 13 1 39 43
-
(1992)
Genomics
, vol.13
, Issue.1
, pp. 39-43
-
-
Kaupmann, K.1
Simon-Chazottes, D.2
Guenet, J.L.3
Jockusch, H.4
-
26
-
-
0032744796
-
Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse
-
Rathke-Hartlieb S., Schmidt V. C., Jockusch H., Schmitt-John T., Bartsch J. W., Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse NeuroReport 1999 10 16 3411 3416
-
(1999)
NeuroReport
, vol.10
, Issue.16
, pp. 3411-3416
-
-
Rathke-Hartlieb, S.1
Schmidt, V.C.2
Jockusch, H.3
Schmitt-John, T.4
Bartsch, J.W.5
-
27
-
-
0025873873
-
Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR)
-
Heimann P., Laage S., Jockusch H., Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR) Differentiation 1991 47 2 77 83
-
(1991)
Differentiation
, vol.47
, Issue.2
, pp. 77-83
-
-
Heimann, P.1
Laage, S.2
Jockusch, H.3
-
28
-
-
27644469489
-
Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse
-
DOI 10.1038/ng1661, PII N1661
-
Schmitt-John T., Drepper C., Mumann A., Hahn P., Kuhlmann M., Thiel C., Hafner M., Lengeling A., Heimann P., Jones J. M., Meisler M. H., Jockusch H., Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse Nature Genetics 2005 37 11 1213 1215 (Pubitemid 41568703)
-
(2005)
Nature Genetics
, vol.37
, Issue.11
, pp. 1213-1215
-
-
Schmitt-John, T.1
Drepper, C.2
Mussmann, A.3
Hahn, P.4
Kuhlmann, M.5
Thiel, C.6
Hafner, M.7
Lengeling, A.8
Heimann, P.9
Jones, J.M.10
Meisler, M.H.11
Jockusch, H.12
-
29
-
-
0029053881
-
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
-
Wong P. C., Pardo C. A., Borchelt D. R., Lee M. K., Copeland N. G., Jenkins N. A., Sisodia S. S., Cleveland D. W., Price D. L., An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria Neuron 1995 14 6 1105 1116
-
(1995)
Neuron
, vol.14
, Issue.6
, pp. 1105-1116
-
-
Wong, P.C.1
Pardo, C.A.2
Borchelt, D.R.3
Lee, M.K.4
Copeland, N.G.5
Jenkins, N.A.6
Sisodia, S.S.7
Cleveland, D.W.8
Price, D.L.9
-
30
-
-
0031051485
-
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
-
DOI 10.1016/S0896-6273(00)80272-X
-
Bruijn L. I., Becher M. W., Lee M. K., Anderson K. L., Jenkins N. A., Copeland N. G., Sisodia S. S., Rothstein J. D., Borchelt D. R., Price D. L., Cleveland D. W., ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions Neuron 1997 18 2 327 338 (Pubitemid 27111114)
-
(1997)
Neuron
, vol.18
, Issue.2
, pp. 327-338
-
-
Bruijn, L.I.1
Becher, M.W.2
Lee, M.K.3
Anderson, K.L.4
Jenkins, N.A.5
Copeland, N.G.6
Sisodia, S.S.7
Rothstein, J.D.8
Borchelt, D.R.9
Price, D.L.10
Cleveland, D.W.11
-
31
-
-
0028888945
-
Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis
-
Ripps M. E., Huntley G. W., Hof P. R., Morrison J. H., Gordon J. W., Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis Proceedings of the National Academy of Sciences of the United States of America 1995 92 3 689 693
-
(1995)
Proceedings of the National Academy of Sciences of the United States of America
, vol.92
, Issue.3
, pp. 689-693
-
-
Ripps, M.E.1
Huntley, G.W.2
Hof, P.R.3
Morrison, J.H.4
Gordon, J.W.5
-
32
-
-
0028284779
-
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
-
Gurney M. E., Pu H., Chiu A. Y., Dal Canto M. C., Polchow C. Y., Alexander D. D., Caliendo J., Hentati A., Kwon Y. W., Deng H. X., Chen W., Zhai P., Sufit R. L., Siddique T., Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation Science 1994 264 5166 1772 1775 (Pubitemid 24227760)
-
(1994)
Science
, vol.264
, Issue.5166
, pp. 1772-1775
-
-
Gurney, M.E.1
Pu, H.2
Chiu, A.Y.3
Dal Canto, M.C.4
Polchow, C.Y.5
Alexander, D.D.6
Caliendo, J.7
Hentati, A.8
Kwon, Y.W.9
Deng, H.-X.10
Chen, W.11
Zhai, P.12
Sufit, R.L.13
Siddique, T.14
-
33
-
-
26444542945
-
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: αB-crystallin modulates aggregation
-
DOI 10.1093/hmg/ddi236
-
Wang J., Xu G., Li H., Gonzales V., Fromholt D., Karch C., Copeland N. G., Jenkins N. A., Borchelt D. R., Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: B-crystallin modulates aggregation Human Molecular Genetics 2005 14 16 2335 2347 (Pubitemid 41417996)
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.16
, pp. 2335-2347
-
-
Wang, J.1
Xu, G.2
Li, H.3
Gonzales, V.4
Fromholt, D.5
Karch, C.6
Copeland, N.G.7
Jenkins, N.A.8
Borchelt, D.R.9
-
34
-
-
18044371459
-
Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification
-
DOI 10.1016/j.molbrainres.2004.11.019
-
Watanabe Y., Yasui K., Nakano T., Doi K., Fukada Y., Kitayama M., Ishimoto M., Kurihara S., Kawashima M., Fukuda H., Adachi Y., Inoue T., Nakashima K., Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification Molecular Brain Research 2005 135 1-2 12 20 (Pubitemid 40602394)
-
(2005)
Molecular Brain Research
, vol.135
, Issue.1-2
, pp. 12-20
-
-
Watanabe, Y.1
Yasui, K.2
Nakano, T.3
Doi, K.4
Fukada, Y.5
Kitayama, M.6
Ishimoto, M.7
Kurihara, S.8
Kawashima, M.9
Fukuda, H.10
Adachi, Y.11
Inoue, T.12
Nakashima, K.13
-
35
-
-
0242524455
-
Copper-binding-site-null SOD1 causes ALS in transgenic mice: Aggregates of non-native SOD1 delineate a common feature
-
DOI 10.1093/hmg/ddg312
-
Wang J., Slunt H., Gonzales V., Fromholt D., Coonfield M., Copeland N. G., Jenkins N. A., Borchelt D. R., Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature Human Molecular Genetics 2003 12 21 2753 2764 (Pubitemid 37407112)
-
(2003)
Human Molecular Genetics
, vol.12
, Issue.21
, pp. 2753-2764
-
-
Wang, J.1
Slunt, H.2
Gonzales, V.3
Fromholt, D.4
Coonfield, M.5
Copeland, N.G.6
Jenkins, N.A.7
Borchelt, D.R.8
-
36
-
-
39849103473
-
Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice
-
DOI 10.1523/JNEUROSCI.5258-07.2008
-
Jaarsma D., Teuling E., Haasdijk E. D., De Zeeuw C. I., Hoogenraad C. C., Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice Journal of Neuroscience 2008 28 9 2075 2088 (Pubitemid 351317636)
-
(2008)
Journal of Neuroscience
, vol.28
, Issue.9
, pp. 2075-2088
-
-
Jaarsma, D.1
Teuling, E.2
Haasdijk, E.D.3
De Zeeuw, C.I.4
Hoogenraad, C.C.5
-
37
-
-
0035575761
-
Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: Associated mutations develop motor neuron disease
-
Nagai M., Aoki M., Miyoshi I., Kato M., Pasinelli P., Kasai N., Brown R. H., Itoyama Y., Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease Journal of Neuroscience 2001 21 23 9246 9254 (Pubitemid 33096868)
-
(2001)
Journal of Neuroscience
, vol.21
, Issue.23
, pp. 9246-9254
-
-
Nagai, M.1
Aoki, M.2
Miyoshi, I.3
Kato, M.4
Pasinelli, P.5
Kasai, N.6
Brown Jr., R.H.7
Itoyama, Y.8
-
38
-
-
0037022339
-
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
-
DOI 10.1073/pnas.032539299
-
Howland D. S., Liu J., She Y., Goad B., Maragakis N. J., Kim B., Erickson J., Kulik J., DeVito L., Psaltis G., DeGennaro L. J., Cleveland D. W., Rothstein J. D., Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS) Proceedings of the National Academy of Sciences of the United States of America 2002 99 3 1604 1609 (Pubitemid 34136539)
-
(2002)
Proceedings of the National Academy of Sciences of the United States of America
, vol.99
, Issue.3
, pp. 1604-1609
-
-
Howland, D.S.1
Liu, J.2
She, Y.3
Goad, B.4
Maragakis, N.J.5
Kim, B.6
Erickson, J.7
Kulik, J.8
DeVito, L.9
Psaltis, G.10
DeGennaro, L.J.11
Cleveland, D.W.12
Rothstein, J.D.13
-
39
-
-
33845361242
-
Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1
-
DOI 10.1097/01.jnen.0000248545.36046.3c, PII 0000507220061200000004
-
Jonsson P. A., Graffmo K. S., Brnnstrm T., Nilsson P., Andersen P. M., Marklund S. L., Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1 Journal of Neuropathology and Experimental Neurology 2006 65 12 1126 1136 (Pubitemid 44885937)
-
(2006)
Journal of Neuropathology and Experimental Neurology
, vol.65
, Issue.12
, pp. 1126-1136
-
-
Jonsson, P.A.1
Graffmo, K.S.2
Brannstrom, T.3
Nilsson, P.4
Andersen, P.M.5
Marklund, S.L.6
-
40
-
-
0033968408
-
Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death
-
DOI 10.1038/72842
-
Hicks G. G., Singh N., Nashabi A., Mai S., Bozek G., Klewes L., Arapovic D., White E. K., Koury M. J., Oltz E. M., Van Kaer L., Ruley H. E., Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death Nature Genetics 2000 24 2 175 179 (Pubitemid 30094720)
-
(2000)
Nature Genetics
, vol.24
, Issue.2
, pp. 175-179
-
-
Hicks, G.G.1
Singh, N.2
Nashabi, A.3
Mai, S.4
Bozek, G.5
Klewes, L.6
Arapovic, D.7
White, E.K.8
Koury, M.J.9
Oltz, E.M.10
Van Kaer, L.11
Ruley, H.E.12
-
41
-
-
0034142209
-
Male sterility and enhanced radiation sensitivity in TLS(-/-) mice
-
Kuroda M., Sok J., Webb L., Baechtold H., Urano F., Yin Y., Chung P., De Rooij D. G., Akhmedov A., Ashley T., Ron D., Male sterility and enhanced radiation sensitivity in TLS(-/-) mice EMBO Journal 2000 19 3 453 462 (Pubitemid 30072615)
-
(2000)
EMBO Journal
, vol.19
, Issue.3
, pp. 453-462
-
-
Kuroda, M.1
Sok, S.2
Webb, L.3
Baechtold, H.4
Urano, F.5
Yin, Y.6
Chung, P.7
De Rooij, D.G.8
Akhmedov, A.9
Ashley, T.10
Ron, D.11
-
42
-
-
73249152831
-
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
-
Wegorzewska I., Bell S., Cairns N. J., Miller T. M., Baloh R. H., TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration Proceedings of the National Academy of Sciences of the United States of America 2009 106 44 18809 18814
-
(2009)
Proceedings of the National Academy of Sciences of the United States of America
, vol.106
, Issue.44
, pp. 18809-18814
-
-
Wegorzewska, I.1
Bell, S.2
Cairns, N.J.3
Miller, T.M.4
Baloh, R.H.5
-
43
-
-
77649269011
-
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
-
Wils H., Kleinberger G., Janssens J., Pereson S., Joris G., Cuijt I., Smits V., Ceuterick-De Groote C., Van Broeckhoven C., Kumar-Singh S., TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration Proceedings of the National Academy of Sciences of the United States of America 2010 107 8 3858 3863
-
(2010)
Proceedings of the National Academy of Sciences of the United States of America
, vol.107
, Issue.8
, pp. 3858-3863
-
-
Wils, H.1
Kleinberger, G.2
Janssens, J.3
Pereson, S.4
Joris, G.5
Cuijt, I.6
Smits, V.7
Ceuterick-De Groote, C.8
Van Broeckhoven, C.9
Kumar-Singh, S.10
-
44
-
-
79251518539
-
Progressive motor weakness in transgenic mice expressing human TDP-43
-
Stallings N. R., Puttaparthi K., Luther C. M., Burns D. K., Elliott J. L., Progressive motor weakness in transgenic mice expressing human TDP-43 Neurobiology of Disease 2010 40 2 404 414
-
(2010)
Neurobiology of Disease
, vol.40
, Issue.2
, pp. 404-414
-
-
Stallings, N.R.1
Puttaparthi, K.2
Luther, C.M.3
Burns, D.K.4
Elliott, J.L.5
-
45
-
-
77956199371
-
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice
-
Xu Y.-F., Gendron T. F., Zhang Y.-J., Lin W.-L., D'Alton S., Sheng H., Casey M. C., Tong J., Knight J., Yu X., Rademakers R., Boylan K., Hutton M., McGowan E., Dickson D. W., Lewis J., Petrucelli L., Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice Journal of Neuroscience 2010 30 32 10851 10859
-
(2010)
Journal of Neuroscience
, vol.30
, Issue.32
, pp. 10851-10859
-
-
Xu, Y.-F.1
Gendron, T.F.2
Zhang, Y.-J.3
Lin, W.-L.4
D'Alton, S.5
Sheng, H.6
Casey, M.C.7
Tong, J.8
Knight, J.9
Yu, X.10
Rademakers, R.11
Boylan, K.12
Hutton, M.13
McGowan, E.14
Dickson, D.W.15
Lewis, J.16
Petrucelli, L.17
-
46
-
-
77950421249
-
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene
-
Zhou H., Huang C., Chen H., Wang D., Landel C. P., Xia P. Y., Bowser R., Liu Y. J., Xia XU. G., Transgenic rat model of neurodegeneration caused by mutation in the TDP gene PLoS Genetics 2010 6 3
-
(2010)
PLoS Genetics
, vol.6
, Issue.3
-
-
Zhou, H.1
Huang, C.2
Chen, H.3
Wang, D.4
Landel, C.P.5
Xia, P.Y.6
Bowser, R.7
Liu, Y.J.8
Xia, Xu.G.9
-
47
-
-
0032408306
-
Identification of the mouse neuromuscular degeneration gene and mapping of a second site suppressor allele
-
DOI 10.1016/S0896-6273(00)80652-2
-
Cox G. A., Mahaffey C. L., Frankel W. N., Identification of the mouse neuromuscular degeneration gene and mapping of a second site suppressor allele Neuron 1998 21 6 1327 1337 (Pubitemid 29022535)
-
(1998)
Neuron
, vol.21
, Issue.6
, pp. 1327-1337
-
-
Cox, G.A.1
Mahaffey, C.L.2
Frankel, W.N.3
-
48
-
-
17944374029
-
Mutations in the gene encoding immunoglobulin μ-binding protein 2 cause spinal muscular atrophy with respiratory distress type 1
-
DOI 10.1038/ng703
-
Grohmann K., Schuelke M., Diers A., Hoffmann K., Lucke B., Adams C., Bertini E., Leonhardt-Horti H., Muntoni F., Ouvrier R., Pfeufer A., Rossi R., Van Maldergem L., Wilmshurst JO. M., Wienker T. F., Sendtner M., Rudnik-Schneborn S., Zerres K., Hbner C., Mutations in the gene encoding immunoglobulin -binding protein 2 cause spinal muscular atrophy with respiratory distress type 1 Nature Genetics 2001 29 1 75 77 (Pubitemid 32801813)
-
(2001)
Nature Genetics
, vol.29
, Issue.1
, pp. 75-77
-
-
Grohmann, K.1
Schuelke, M.2
Diers, A.3
Hoffmann, K.4
Lucke, B.5
Adams, C.6
Bertini, E.7
Leonhardt-Horti, H.8
Muntoni, F.9
Ouvrier, R.10
Pfeufer, A.11
Rossi, R.12
Van Maldergem, L.13
Wilmshurst, J.M.14
Wienker, T.F.15
Sendtner, M.16
Rudnik-Schoneborn, S.17
Zerres, K.18
Hubner, C.19
-
49
-
-
0036842251
-
A missense mutation in Tbce causes progressive motor neuronopathy in mice
-
DOI 10.1038/ng1016
-
Martin N., Jaubert J., Gounon P., Salido E., Haase G., Szatanik M., Gunet J. L., A missense mutation in Tbce causes progressive motor neuronopathy in mice Nature Genetics 2002 32 3 443 447 (Pubitemid 35266123)
-
(2002)
Nature Genetics
, vol.32
, Issue.3
, pp. 443-447
-
-
Martin, N.1
Jaubert, J.2
Gounon, P.3
Salido, E.4
Haase, G.5
Szatanik, M.6
Guenet, J.-L.7
-
50
-
-
23844448652
-
Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress
-
DOI 10.1523/JNEUROSCI.1645-05.2005
-
Cai H., Lin X., Xie C., Laird F. M., Lai C., Wen H., Chiang H. C., Shim H., Farah M. H., Hoke A., Price D. L., Wong P. C., Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress Journal of Neuroscience 2005 25 33 7567 7574 (Pubitemid 41170614)
-
(2005)
Journal of Neuroscience
, vol.25
, Issue.33
, pp. 7567-7574
-
-
Cai, H.1
Lin, X.2
Xie, C.3
Laird, F.M.4
Lai, C.5
Wen, H.6
Chiang, H.-C.7
Shim, H.8
Farah, M.H.9
Hoke, A.10
Price, D.L.11
Wong, P.C.12
-
51
-
-
31144448704
-
Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking
-
DOI 10.1093/hmg/ddi440
-
Hadano S., Benn S. C., Kakuta S., Otomo A., Sudo K., Kunita R., Suzuki-Utsunomiya K., Mizumura H., Shefner J. M., Cox G. A., Iwakura Y., Brown R. H., Ikeda J. E., Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking Human Molecular Genetics 2006 15 2 233 250 (Pubitemid 43125978)
-
(2006)
Human Molecular Genetics
, vol.15
, Issue.2
, pp. 233-250
-
-
Hadano, S.1
Benn, S.C.2
Kakuta, S.3
Otomo, A.4
Sudo, K.5
Kunita, R.6
Suzuki-Utsunomiya, K.7
Mizumura, H.8
Shefner, J.M.9
Cox, G.A.10
Iwakura, Y.11
Brown Jr., R.H.12
Ikeda, J.-E.13
-
52
-
-
33745473350
-
Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities
-
DOI 10.1073/pnas.0510197103
-
Devon R. S., Orban P. C., Gerrow K., Barbieri M. A., Schwab C., Cao L. P., Helm J. R., Bissada M., Cruz-Aguado R., Davidson T. L., Witmer J., Metzler M., Lam C. K., Tetzlaff W., Simpson E. M., McCaffery J. M., El-Husseini A. E., Leavitt B. R., Hayden M. R., Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities Proceedings of the National Academy of Sciences of the United States of America 2006 103 25 9595 9600 (Pubitemid 43955870)
-
(2006)
Proceedings of the National Academy of Sciences of the United States of America
, vol.103
, Issue.25
, pp. 9595-9600
-
-
Devon, R.S.1
Orban, P.C.2
Gerrow, K.3
Barbieri, M.A.4
Schwab, C.5
Cao, L.P.6
Helm, J.R.7
Bissada, M.8
Cruz-Aguado, R.9
Davidson, T.-L.10
Witmer, J.11
Metzler, M.12
Lam, C.K.13
Tetzlaff, W.14
Simpson, E.M.15
McCaffery, J.M.16
El-Husseini, A.E.17
Leavitt, B.R.18
Hayden, M.R.19
-
53
-
-
33745728401
-
Progressive spinal axonal degeneration and slowness in ALS2-deficient mice
-
Yamanaka K., Miller T. M., McAlonis-Downes M., Chun S. J., Cleveland D. W., Progressive spinal axonal degeneration and slowness in ALS2-deficient mice Annals of Neurology 2006 60 1 95 104
-
(2006)
Annals of Neurology
, vol.60
, Issue.1
, pp. 95-104
-
-
Yamanaka, K.1
Miller, T.M.2
McAlonis-Downes, M.3
Chun, S.J.4
Cleveland, D.W.5
-
54
-
-
77951298381
-
Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology
-
Tudor E. L., Galtrey C. M., Perkinton M. S., Lau K. F., De Vos K. J., Mitchell J. C., Ackerley S., Hortobgyi T., Vmos E., Leigh P. N., Klasen C., McLoughlin D. M., Shaw C. E., Miller C. C. J., Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology Neuroscience 2010 167 3 774 785
-
(2010)
Neuroscience
, vol.167
, Issue.3
, pp. 774-785
-
-
Tudor, E.L.1
Galtrey, C.M.2
Perkinton, M.S.3
Lau, K.F.4
De Vos, K.J.5
Mitchell, J.C.6
Ackerley, S.7
Hortobgyi, T.8
Vmos, E.9
Leigh, P.N.10
Klasen, C.11
McLoughlin, D.M.12
Shaw, C.E.13
Miller, C.C.J.14
-
55
-
-
38449097283
-
glued causes dysfunction of dynactin in mice
-
DOI 10.1523/JNEUROSCI.4226-07.2007
-
Lai C., Lin X., Chandran J., Shim H., Yang W. J., Cai H., The G59S mutation in p150 causes dysfunction of dynactin in mice Journal of Neuroscience 2007 27 51 13982 13990 (Pubitemid 351377874)
-
(2007)
Journal of Neuroscience
, vol.27
, Issue.51
, pp. 13982-13990
-
-
Lai, C.1
Lin, X.2
Chandran, J.3
Shim, H.4
Yang, W.-J.5
Cai, H.6
-
56
-
-
39849107361
-
Motor neuron disease occurring in a mutant dynactin mouse model is characterized by defects in vesicular trafficking
-
DOI 10.1523/JNEUROSCI.4231-07.2008
-
Laird F. M., Farah M. H., Ackerley S., Hoke A., Maragakis N., Rothstein J. D., Griffin J., Price D. L., Martin L. J., Wong P. C., Motor neuron disease occurring in a mutant dynactin mouse model is characterized by defects in vesicular trafficking Journal of Neuroscience 2008 28 9 1997 2005 (Pubitemid 351317634)
-
(2008)
Journal of Neuroscience
, vol.28
, Issue.9
, pp. 1997-2005
-
-
Laird, F.M.1
Farah, M.H.2
Ackerley, S.3
Hoke, A.4
Maragakis, N.5
Rothstein, J.D.6
Griffin, J.7
Price, D.L.8
Martin, L.J.9
Wong, P.C.10
-
57
-
-
45749094601
-
Glued subunit of dynactin
-
DOI 10.1093/hmg/ddn092
-
Chevalier-Larsen E. S., Wallace K. E., Pennise C. R., Holzbaur E. L. F., Lysosomal proliferation and distal degeneration in motor neurons expressing the G59S mutation in the p150 subunit of dynactin Human Molecular Genetics 2008 17 13 1946 1955 (Pubitemid 351865843)
-
(2008)
Human Molecular Genetics
, vol.17
, Issue.13
, pp. 1946-1955
-
-
Chevalier-Larsen, E.S.1
Wallace, K.E.2
Pennise, C.R.3
Holzbaur, E.L.F.4
-
58
-
-
0028116467
-
A mutant neurofilament subunit causes massive, selective motor neuron death: Implications for the pathogenesis of human motor neuron disease
-
DOI 10.1016/0896-6273(94)90263-1
-
Lee M. K., Marszalek J. R., Cleveland D. W., A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease Neuron 1994 13 4 975 988 (Pubitemid 24347310)
-
(1994)
Neuron
, vol.13
, Issue.4
, pp. 975-988
-
-
Lee, M.K.1
Marszalek, J.R.2
Cleveland, D.W.3
-
59
-
-
0033230327
-
Late onset death of motor neurons in mice overexpressing wild-type peripherin
-
DOI 10.1083/jcb.147.3.531
-
Beaulieu J. M., Nguyen M. D., Julien J. P., Late onset death of motor neurons in mice overexpressing wild-type peripherin Journal of Cell Biology 1999 147 3 531 544 (Pubitemid 29523460)
-
(1999)
Journal of Cell Biology
, vol.147
, Issue.3
, pp. 531-544
-
-
Beaulieu, J.-M.1
Nguyen, M.D.2
Julien, J.-P.3
-
60
-
-
0034978562
-
Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration
-
DOI 10.1038/88842
-
Oosthuyse B., Moons L., Storkebaum E., Beck H., Nuyens D., Brusselmans K., Dorpe JO. V., Hellings P., Gorselink M., Heymans S., Theilmeier G., Dewerchin M., Laudenbach V., Vermylen P., Raat H., Acker T., Vleminckx V., Bosch L. V. D., Cashman N., Fujisawa H., Drost M. R., Sciot R., Bruyninckx F., Hicklin D. J., Ince C., Gressens P., Lupu F., Plate K. H., Robberecht W., Herbert J. M., Collen D., Carmeliet P., Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration Nature Genetics 2001 28 2 131 138 (Pubitemid 32538057)
-
(2001)
Nature Genetics
, vol.28
, Issue.2
, pp. 131-138
-
-
Oosthuyse, B.1
Moons, L.2
Storkebaum, E.3
Beck, H.4
Nuyens, D.5
Brusselmans, K.6
Dorpe, J.V.7
Hellings, P.8
Gorselink, M.9
Heymans, S.10
Theilmeier, G.11
Dewerchin, M.12
Laudenbach, V.13
Vermylen, P.14
Raat, H.15
Acker, T.16
Vleminckx, V.17
Bosch, L.V.D.18
Cashman, N.19
Fujisawa, H.20
Drost, M.R.21
Sciot, R.22
Bruyninckx, F.23
Hicklin, D.J.24
Ince, C.25
Gressens, P.26
Lupu, F.27
Plate, K.H.28
Robberecht, W.29
Herbert, J.-M.30
Collen, D.31
Carmeliet, P.32
more..
-
61
-
-
0032786370
-
Prominent axonopathy in the brain and spinal cord of transgenic mice overexpressing four-repeat human tau protein
-
Spittaels K., Van Den Haute C., Van Dorpe JO., Bruynseels K., Vandezande K., Laenen I., Geerts H., Mercken M., Sciot R., Van Lommel A., Loos R., Van Leuven F., Prominent axonopathy in the brain and spinal cord of transgenic mice overexpressing four-repeat human tau protein American Journal of Pathology 1999 155 6 2153 2165
-
(1999)
American Journal of Pathology
, vol.155
, Issue.6
, pp. 2153-2165
-
-
Spittaels, K.1
Van Den Haute, C.2
Van Dorpe, J.O.3
Bruynseels, K.4
Vandezande, K.5
Laenen, I.6
Geerts, H.7
Mercken, M.8
Sciot, R.9
Van Lommel, A.10
Loos, R.11
Van Leuven, F.12
-
62
-
-
2442642836
-
Retarded axonal transport of R406W mutant tau in transgenic mice with a neurodegenerative tauopathy
-
DOI 10.1523/JNEUROSCI.0797-04.2004
-
Zhang B., Higuchi M., Yoshiyama Y., Ishihara T., Forman M. S., Martinez D., Joyce S., Trojanowski J. Q., Lee V. M. Y., Retarded axonal transport of R406W mutant tau in transgenic mice with a neurodegenerative tauopathy Journal of Neuroscience 2004 24 19 4657 4667 (Pubitemid 38656621)
-
(2004)
Journal of Neuroscience
, vol.24
, Issue.19
, pp. 4657-4667
-
-
Zhang, B.1
Higuchi, M.2
Yoshiyama, Y.3
Ishihara, T.4
Forman, M.S.5
Martinez, D.6
Joyce, S.7
Trojanowski, J.Q.8
Lee, V.M.-Y.9
-
63
-
-
0037108953
-
Tau filament formation and associative memory deficit in aged mice expressing mutant (R406W) human tau
-
DOI 10.1073/pnas.202205599
-
Tatebayashi Y., Miyasaka T., Chui DE. H., Akagi T., Mishima K. I., Iwasaki K., Fujiwara M., Tanemura K., Murayama M., Ishiguro K., Planel E., Sato S., Hashikawa T., Takashima A., Tau filament formation and associative memory deficit in aged mice expressing mutant (R406W) human tau Proceedings of the National Academy of Sciences of the United States of America 2002 99 21 13896 13901 (Pubitemid 35215477)
-
(2002)
Proceedings of the National Academy of Sciences of the United States of America
, vol.99
, Issue.21
, pp. 13896-13901
-
-
Tatebayashi, Y.1
Miyasaka, T.2
Chui, D.-H.3
Akagi, T.4
Mishima, K.-I.5
Iwasaki, K.6
Fujiwara, M.7
Tanemura, K.8
Murayama, M.9
Ishiguro, K.10
Planel, E.11
Sato, S.12
Hashikawa, T.13
Takashima, A.14
-
64
-
-
27944491109
-
Age-dependent neurofibrillary tangle formation, neuron loss, and memory impairment in a mouse model of human tauopathy (P301L)
-
DOI 10.1523/JNEUROSCI.3279-05.2005
-
Ramsden M., Kotilinek L., Forster C., Paulson J., McGowan E., SantaCruz K., Guimaraes A., Yue M., Lewis J., Carlson G., Hutton M., Ashe K. H., Age-dependent neurofibrillary tangle formation, neuron loss, and memory impairment in a mouse model of human tauopathy (P301L) Journal of Neuroscience 2005 25 46 10637 10647 (Pubitemid 41681911)
-
(2005)
Journal of Neuroscience
, vol.25
, Issue.46
, pp. 10637-10647
-
-
Ramsden, M.1
Kotilinek, L.2
Forster, C.3
Paulson, J.4
McGowan, E.5
SantaCruz, K.6
Guimaraes, A.7
Yue, M.8
Lewis, J.9
Carlson, G.10
Hutton, M.11
Ashe, K.H.12
-
65
-
-
33746652117
-
Alzheimer's disease-like tau neuropathology leads to memory deficits and loss of functional synapses in a novel mutated tau transgenic mouse without any motor deficits
-
DOI 10.2353/ajpath.2006.060002
-
Schindowski K., Bretteville A., Leroy K., Bgard S., Brion J. P., Hamdane M., Bue L., Alzheimer's disease-like tau neuropathology leads to memory deficits and loss of functional synapses in a novel mutated tau transgenic mouse without any motor deficits American Journal of Pathology 2006 169 2 599 616 (Pubitemid 44156317)
-
(2006)
American Journal of Pathology
, vol.169
, Issue.2
, pp. 599-616
-
-
Schindowski, K.1
Bretteville, A.2
Leroy, K.3
Begard, S.4
Brion, J.-P.5
Hamdane, M.6
Buee, L.7
-
66
-
-
0036138044
-
Neurodegeneration with tau accumulation in a transgenic mouse expressing V337M human tau
-
Tanemura K., Murayama M., Akagi T., Hashikawa T., Tominaga T., Ichikawa M., Yamaguchi H., Takashima A., Neurodegeneration with tau accumulation in a transgenic mouse expressing V337M human tau Journal of Neuroscience 2002 22 1 133 141 (Pubitemid 34033491)
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.1
, pp. 133-141
-
-
Tanemura, K.1
Murayama, M.2
Akagi, T.3
Hashikawa, T.4
Tominaga, T.5
Ichikawa, M.6
Yamaguchi, H.7
Takashima, A.8
-
67
-
-
33846538660
-
Synapse Loss and Microglial Activation Precede Tangles in a P301S Tauopathy Mouse Model
-
DOI 10.1016/j.neuron.2007.01.010, PII S089662730700030X
-
Yoshiyama Y., Higuchi M., Zhang B., Huang S. M., Iwata N., Saido T., Maeda J., Suhara T., Trojanowski J. Q., Lee V. M. Y., Synapse Loss and Microglial Activation Precede Tangles in a P301S Tauopathy Mouse Model Neuron 2007 53 3 337 351 (Pubitemid 46161268)
-
(2007)
Neuron
, vol.53
, Issue.3
, pp. 337-351
-
-
Yoshiyama, Y.1
Higuchi, M.2
Zhang, B.3
Huang, S.-M.4
Iwata, N.5
Saido, TakaomiC.6
Maeda, J.7
Suhara, T.8
Trojanowski, J.Q.9
Lee, V.M.-Y.10
-
68
-
-
0037734370
-
Mutations in dynein link motor neuron degeneration to defects in retrograde transport
-
DOI 10.1126/science.1083129
-
Hafezparast M., Klocke R., Ruhrberg C., Marquardt A., Ahmad-Annuar A., Bowen S., Lalli G., Witherden A. S., Hummerich H., Nicholson S., Morgan P. J., Oozageer R., Priestley J. V., Averill S., King V. R., Ball S., Peters JO., Toda T., Yamamoto A., Hiraoka Y., Augustin M., Korthaus D., Wattler S., Wabnitz P., Dickneite C., Lampel S., Boehme F., Peraus G., Popp A., Rudelius M., Schlegel J., Fuchs H., De Angelis M. H., Schiavo G., Shima D. T., Russ A. P., Stumm G., Martin J. E., Fisher E. M. C., Mutations in dynein link motor neuron degeneration to defects in retrograde transport Science 2003 300 5620 808 812 (Pubitemid 36532118)
-
(2003)
Science
, vol.300
, Issue.5620
, pp. 808-812
-
-
Hafezparast, M.1
Klocke, R.2
Ruhrberg, C.3
Marquardt, A.4
Ahmad-Annuar, A.5
Bowen, S.6
Lalli, G.7
Witherden, A.S.8
Hummerich, H.9
Nicholson, S.10
Morgan, P.J.11
Oozageer, R.12
Priestley, J.V.13
Averill, S.14
King, V.R.15
Ball, S.16
Peters, J.17
Toda, T.18
Yamamoto, A.19
Hiraoka, Y.20
Augustin, M.21
Korthaus, D.22
Wattler, S.23
Wabnitz, P.24
Dickneite, C.25
Lampel, S.26
Boehme, F.27
Peraus, G.28
Popp, A.29
Rudelius, M.30
Schlegel, J.31
Fuchs, H.32
De Angelis, M.H.33
Schiavo, G.34
Shima, D.T.35
Russ, A.P.36
Stumm, G.37
Martin, J.E.38
Fisher, E.M.C.39
more..
-
69
-
-
50449101429
-
Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death
-
Ilieva H. S., Yamanaka K., Malkmus S., Kakinohana O., Yaksh T., Marsala M., Cleveland D. W., Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death Proceedings of the National Academy of Sciences of the United States of America 2008 105 34 12599 12604
-
(2008)
Proceedings of the National Academy of Sciences of the United States of America
, vol.105
, Issue.34
, pp. 12599-12604
-
-
Ilieva, H.S.1
Yamanaka, K.2
Malkmus, S.3
Kakinohana, O.4
Yaksh, T.5
Marsala, M.6
Cleveland, D.W.7
-
70
-
-
21344472483
-
A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice
-
DOI 10.1083/jcb.200501085
-
Kieran D., Hafezparast M., Bohnert S., Dick J. R. T., Martin J., Schiavo G., Fisher E. M. C., Greensmith L., A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice Journal of Cell Biology 2005 169 4 561 567 (Pubitemid 41002849)
-
(2005)
Journal of Cell Biology
, vol.169
, Issue.4
, pp. 561-567
-
-
Kieran, D.1
Hafezparast, M.2
Bohnert, S.3
Dick, J.R.T.4
Martin, J.5
Schiavo, G.6
Fisher, E.M.C.7
Greensmith, L.8
-
71
-
-
33751229311
-
The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis
-
DOI 10.1016/j.bbadis.2006.05.002, PII S0925443906000822
-
Van Den Bosch L., Van Damme P., Bogaert E., Robberecht W., The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis Biochimica et Biophysica Acta 2006 1762 11-12 1068 1082 (Pubitemid 44781176)
-
(2006)
Biochimica et Biophysica Acta - Molecular Basis of Disease
, vol.1762
, Issue.11-12
, pp. 1068-1082
-
-
Van Den Bosch, L.1
Van Damme, P.2
Bogaert, E.3
Robberecht, W.4
-
72
-
-
57649107154
-
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease
-
Dupuis L., Fergani A., Braunstein K. E., Eschbach J., Holl N., Rene F., Gonzalez De Aguilar J. L., Zoerner B., Schwalenstocker B., Ludolph A. C., Loeffler J. P., Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease Experimental Neurology 2009 215 1 146 152
-
(2009)
Experimental Neurology
, vol.215
, Issue.1
, pp. 146-152
-
-
Dupuis, L.1
Fergani, A.2
Braunstein, K.E.3
Eschbach, J.4
Holl, N.5
Rene, F.6
Gonzalez De Aguilar, J.L.7
Zoerner, B.8
Schwalenstocker, B.9
Ludolph, A.C.10
Loeffler, J.P.11
-
73
-
-
77958479351
-
A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons
-
Braunstein K. E., Eschbach J., Rna-Vrs K., Soylu R., Mikrouli E., Larmet Y., Ren F., de Aguilar J. G., Loeffler J.-P., Mller H.-P., Bucher S., Kaulisch T., Niessen H. G., Tillmanns J., Fischer K., Schwalenstcker B., Kassubek J., Pichler B., Stiller D., Petersen., Ludolph A. C., Dupuis L., A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons Human Molecular Genetics 2010 19 22 4385 4398
-
(2010)
Human Molecular Genetics
, vol.19
, Issue.22
, pp. 4385-4398
-
-
Braunstein, K.E.1
Eschbach, J.2
Rna-Vrs, K.3
Soylu, R.4
Mikrouli, E.5
Larmet, Y.6
Ren, F.7
De Aguilar, J.G.8
Loeffler, J.-P.9
Mller, H.-P.10
Bucher, S.11
Kaulisch, T.12
Niessen, H.G.13
Tillmanns, J.14
Fischer, K.15
Schwalenstcker, B.16
Kassubek, J.17
Pichler, B.18
Stiller, D.19
Petersen, .20
Ludolph, A.C.21
Dupuis, L.22
more..
-
74
-
-
0036212119
-
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading
-
DOI 10.1038/nn823
-
Subramaniam J. R., Lyons W. E., Liu J., Bartnikas T. B., Rothstein J., Price D. L., Cleveland D. W., Gitlin J. D., Wong P. C., Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading Nature Neuroscience 2002 5 4 301 307 (Pubitemid 34279809)
-
(2002)
Nature Neuroscience
, vol.5
, Issue.4
, pp. 301-307
-
-
Subramaniam, J.R.1
Lyons, W.E.2
Liu, J.3
Bartnikas, T.B.4
Rothstein, J.5
Price, D.L.6
Cleveland, D.W.7
Gitlin, J.D.8
Wong, P.C.9
-
75
-
-
0035873076
-
Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment
-
Pramatarova A., Laganire J., Roussel J., Brisebois K., Rouleau G. A., Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment Journal of Neuroscience 2001 21 10 3369 3374 (Pubitemid 32422802)
-
(2001)
Journal of Neuroscience
, vol.21
, Issue.10
, pp. 3369-3374
-
-
Pramatarova, A.1
Laganiere, J.2
Roussel, J.3
Brisebois, K.4
Rouleau, G.A.5
-
76
-
-
0034651102
-
Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration
-
Gong Y. H., Parsadanian A. S., Andreeva A., Snider W. D., Elliott J. L., Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration Journal of Neuroscience 2000 20 2 660 665 (Pubitemid 30225568)
-
(2000)
Journal of Neuroscience
, vol.20
, Issue.2
, pp. 660-665
-
-
Gong, Y.H.1
Parsadanian, A.S.2
Andreeva, A.3
Snider, W.D.4
Elliott, J.L.5
-
77
-
-
39149135870
-
Restricted expression of mutant SOD1 in spinal motor neurons and interneurons induces motor neuron pathology
-
DOI 10.1016/j.nbd.2007.10.004, PII S0969996107002367
-
Wang L., Sharma K., Deng H. X., Siddique T., Grisotti G., Liu E., Roos R. P., Restricted expression of mutant SOD1 in spinal motor neurons and interneurons induces motor neuron pathology Neurobiology of Disease 2008 29 3 400 408 (Pubitemid 351258350)
-
(2008)
Neurobiology of Disease
, vol.29
, Issue.3
, pp. 400-408
-
-
Wang, L.1
Sharma, K.2
Deng, H.-X.3
Siddique, T.4
Grisotti, G.5
Liu, E.6
Roos, R.P.7
-
78
-
-
33744798774
-
Onset and progression in inherited ALS determined by motor neurons and microglia
-
DOI 10.1126/science.1123511
-
Boille S., Yamanaka K., Lobsiger C. S., Copeland N. G., Jenkins N. A., Kassiotis G., Kollias G., Cleveland D. W., Onset and progression in inherited ALS determined by motor neurons and microglia Science 2006 312 5778 1389 1392 (Pubitemid 43839552)
-
(2006)
Science
, vol.312
, Issue.5778
, pp. 1389-1392
-
-
Boillee, S.1
Yamanaka, K.2
Lobsiger, C.S.3
Copeland, N.G.4
Jenkins, N.A.5
Kassiotis, G.6
Kollias, G.7
Cleveland, D.W.8
-
79
-
-
39749188753
-
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
-
DOI 10.1038/nn2047, PII NN2047
-
Yamanaka K., Chun S. J., Boillee S., Fujimori-Tonou N., Yamashita H., Gutmann D. H., Takahashi R., Misawa H., Cleveland D. W., Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis Nature Neuroscience 2008 11 3 251 253 (Pubitemid 351311795)
-
(2008)
Nature Neuroscience
, vol.11
, Issue.3
, pp. 251-253
-
-
Yamanaka, K.1
Chun, S.J.2
Boillee, S.3
Fujimori-Tonou, N.4
Yamashita, H.5
Gutmann, D.H.6
Takahashi, R.7
Misawa, H.8
Cleveland, D.W.9
-
80
-
-
79551588652
-
Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice
-
Wang L., Gutmann D. H., Roos R. P., Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice Human Molecular Genetics 2010 19 24
-
(2010)
Human Molecular Genetics
, vol.19
, Issue.24
-
-
Wang, L.1
Gutmann, D.H.2
Roos, R.P.3
-
81
-
-
63149141363
-
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice
-
Lobsiger C. S., Boillee S., McAlonis-Downes M., Khan A. M., Feltri M. L., Yamanaka K., Cleveland D. W., Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice Proceedings of the National Academy of Sciences of the United States of America 2009 106 11 4465 4470
-
(2009)
Proceedings of the National Academy of Sciences of the United States of America
, vol.106
, Issue.11
, pp. 4465-4470
-
-
Lobsiger, C.S.1
Boillee, S.2
McAlonis-Downes, M.3
Khan, A.M.4
Feltri, M.L.5
Yamanaka, K.6
Cleveland, D.W.7
-
82
-
-
33845876647
-
Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis
-
DOI 10.1073/pnas.0609411103
-
Miller T. M., Kim S. H., Yamanaka K., Hester M., Umapathi P., Arnson H., Rizo L., Mendell J. R., Gage F. H., Cleveland D. W., Kaspar B. K., Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis Proceedings of the National Academy of Sciences of the United States of America 2006 103 51 19546 19551 (Pubitemid 46026068)
-
(2006)
Proceedings of the National Academy of Sciences of the United States of America
, vol.103
, Issue.51
, pp. 19546-19551
-
-
Miller, T.M.1
Kim, S.H.2
Yamanaka, K.3
Hester, M.4
Umapathi, P.5
Arnson, H.6
Rizo, L.7
Mendell, J.R.8
Gage, F.H.9
Cleveland, D.W.10
Kaspar, B.K.11
-
83
-
-
77953530958
-
Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice
-
Wong M., Martin L. J., Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice Human Molecular Genetics 2010 19 11 2284 2302
-
(2010)
Human Molecular Genetics
, vol.19
, Issue.11
, pp. 2284-2302
-
-
Wong, M.1
Martin, L.J.2
-
84
-
-
54849404282
-
Skeletal muscle is a primary target of SOD1-mediated toxicity
-
Dobrowolny G., Aucello M., Rizzuto E., Beccafico S., Mammucari C., Bonconpagni S., Belia S., Wannenes F., Nicoletti C., Del Prete Z., Rosenthal N., Molinaro M., Protasi F., Fan G., Sandri M., Musar A., Skeletal muscle is a primary target of SOD1-mediated toxicity Cell Metabolism 2008 8 5 425 436
-
(2008)
Cell Metabolism
, vol.8
, Issue.5
, pp. 425-436
-
-
Dobrowolny, G.1
Aucello, M.2
Rizzuto, E.3
Beccafico, S.4
Mammucari, C.5
Bonconpagni, S.6
Belia, S.7
Wannenes, F.8
Nicoletti, C.9
Del Prete, Z.10
Rosenthal, N.11
Molinaro, M.12
Protasi, F.13
Fan, G.14
Sandri, M.15
Musar, A.16
-
85
-
-
0037007645
-
Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice
-
DOI 10.1038/417074a
-
Zhu S., Stavrovskaya I. G., Drozda M., Kim B. Y. S., Ona V., Li M., Sarang S., Liu A. S., Hartley D. M., Wu DU. C., Guilans S., Ferrante R. J., Przedborski S., Kristal B. S., Friedlander R. M., Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice Nature 2002 417 6884 74 78 (Pubitemid 34498818)
-
(2002)
Nature
, vol.417
, Issue.6884
, pp. 74-78
-
-
Zhu, S.1
Stavrovskaya, I.G.2
Drozda, M.3
Kim, B.Y.S.4
Ona, V.5
Li, M.6
Sarang, S.7
Liu, A.S.8
Hartley, D.M.9
Wu, D.C.10
Guilans, S.11
Ferrante, R.J.12
Przedborski, S.13
Kristal, B.S.14
Friedlander, R.M.15
-
86
-
-
0037067009
-
Minocycline delays disease onset and mortality in a transgenic model of ALS
-
Van Den Bosch L., Tilkin P., Lemmens G., Robberecht W., Minocycline delays disease onset and mortality in a transgenic model of ALS NeuroReport 2002 13 8 1067 1070 (Pubitemid 34587742)
-
(2002)
NeuroReport
, vol.13
, Issue.8
, pp. 1067-1070
-
-
Van Den Bosch, L.1
Tilkin, P.2
Lemmens, G.3
Robberecht, W.4
-
87
-
-
0036406903
-
Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis
-
DOI 10.1006/nbdi.2002.0487
-
Kriz J., Nguyen M. D., Julien J. P., Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis Neurobiology of Disease 2002 10 3 268 278 (Pubitemid 35217580)
-
(2002)
Neurobiology of Disease
, vol.10
, Issue.3
, pp. 268-278
-
-
Kriz, J.1
Nguyen, M.D.2
Julien, J.-P.3
-
88
-
-
36148960127
-
Efficacy of minocycline in patients with amyotrophic lateral sclerosis: A phase III randomised trial
-
DOI 10.1016/S1474-4422(07)70270-3, PII S1474442207702703
-
Gordon P. H., Moore D. H., Miller R. G., Florence J. M., Verheijde J. L., Doorish C., Hilton J. F., Spitalny G. M., MacArthur R. B., Mitsumoto H., Neville H. E., Boylan K., Mozaffar T., Belsh J. M., Ravits J., Bedlack R. S., Graves M. C., McCluskey L. F., Barohn R. J., Tandan R., Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial Lancet Neurology 2007 6 12 1045 1053 (Pubitemid 350116691)
-
(2007)
Lancet Neurology
, vol.6
, Issue.12
, pp. 1045-1053
-
-
Gordon, P.H.1
Moore, D.H.2
Miller, R.G.3
Florence, J.M.4
Verheijde, J.L.5
Doorish, C.6
Hilton, J.F.7
Spitalny, G.M.8
MacArthur, R.B.9
Mitsumoto, H.10
Neville, H.E.11
Boylan, K.12
Mozaffar, T.13
Belsh, J.M.14
Ravits, J.15
Bedlack, R.S.16
Graves, M.C.17
McCluskey, L.F.18
Barohn, R.J.19
Tandan, R.20
more..
-
89
-
-
33947323759
-
Lost in translation: Treatment trials in the SOD1 mouse and in human ALS
-
DOI 10.1016/j.nbd.2006.12.015, PII S0969996106003287
-
Benatar M., Lost in translation: treatment trials in the SOD1 mouse and in human ALS Neurobiology of Disease 2007 26 1 1 13 (Pubitemid 46437445)
-
(2007)
Neurobiology of Disease
, vol.26
, Issue.1
, pp. 1-13
-
-
Benatar, M.1
-
90
-
-
39349107014
-
Design, power, and interpretation of studies in the standard murine model of ALS
-
DOI 10.1080/17482960701856300, PII 789666722
-
Scott S., Kranz J. E., Cole J., Lincecum J. M., Thompson K., Kelly N., Bostrom A., Theodoss J., Al-Nakhala B. M., Vieira F. G., Ramasubbu J., Heywood J. A., Design, power, and interpretation of studies in the standard murine model of ALS Amyotrophic Lateral Sclerosis 2008 9 1 4 15 (Pubitemid 351260367)
-
(2008)
Amyotrophic Lateral Sclerosis
, vol.9
, Issue.1
, pp. 4-15
-
-
Scott, S.1
Kranz, J.E.2
Cole, J.3
Lincecum, J.M.4
Thompson, K.5
Kelly, N.6
Bostrom, A.7
Theodoss, J.8
Al-Nakhala, B.M.9
Vieira, F.G.10
Ramasubbu, J.11
Heywood, J.A.12
-
91
-
-
77649285022
-
Guidelines for preclinical animal research in ALS/MND: A consensus meeting
-
Ludolph A. C., Bendotti C., Blaugrund E., Chio A., Greensmith L., Loeffler J. P., Mead R., Niessen H. G., Petri S., Pradat P. F., Robberecht W., Ruegg M., Schwalenstcker B., Stiller D., Van Den Berg L., Vieira F., Von Horsten S., Guidelines for preclinical animal research in ALS/MND: a consensus meeting Amyotrophic Lateral Sclerosis 2010 11 1-2 38 45
-
(2010)
Amyotrophic Lateral Sclerosis
, vol.11
, Issue.12
, pp. 38-45
-
-
Ludolph, A.C.1
Bendotti, C.2
Blaugrund, E.3
Chio, A.4
Greensmith, L.5
Loeffler, J.P.6
Mead, R.7
Niessen, H.G.8
Petri, S.9
Pradat, P.F.10
Robberecht, W.11
Ruegg, M.12
Schwalenstcker, B.13
Stiller, D.14
Van Den Berg, L.15
Vieira, F.16
Von Horsten, S.17
-
92
-
-
29144492854
-
Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS
-
DOI 10.1016/j.nbd.2005.06.019, PII S0969996105001932
-
Llad J., Haenggeli C., Pardo A., Wong V., Benson L., Coccia C., Rothstein J. D., Shefner J. M., Maragakis N. J., Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS Neurobiology of Disease 2006 21 1 110 118 (Pubitemid 41817311)
-
(2006)
Neurobiology of Disease
, vol.21
, Issue.1
, pp. 110-118
-
-
Llado, J.1
Haenggeli, C.2
Pardo, A.3
Wong, V.4
Benson, L.5
Coccia, C.6
Rothstein, J.D.7
Shefner, J.M.8
Maragakis, N.J.9
-
93
-
-
77954953414
-
Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats
-
Lepore A. C., Tolmie C., O'Donnell J., Wright M. C., Dejea C., Rauck B., Hoke A., Ignagni A. R., Onders R. P., Maragakis N. J., Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats Neurobiology of Disease 2010 39 3 252 264
-
(2010)
Neurobiology of Disease
, vol.39
, Issue.3
, pp. 252-264
-
-
Lepore, A.C.1
Tolmie, C.2
O'Donnell, J.3
Wright, M.C.4
Dejea, C.5
Rauck, B.6
Hoke, A.7
Ignagni, A.R.8
Onders, R.P.9
Maragakis, N.J.10
-
94
-
-
0041664055
-
Two families with familial amyotrophic lateral sclerosis are linked to a novel locus on chromosome 16q
-
DOI 10.1086/377157
-
Ruddy D. M., Parton M. J., Al-Chalabi A., Lewis C. M., Vance C., Smith B. N., Leigh P. N., Powell J. F., Siddique T., Meyjes E. P., Baas F., De Jong V., Shaw C. E., Two families with familial amyotrophic lateral sclerosis are linked to a novel locus on chromosome 16q American Journal of Human Genetics 2003 73 2 390 396 (Pubitemid 36921001)
-
(2003)
American Journal of Human Genetics
, vol.73
, Issue.2
, pp. 390-396
-
-
Ruddy, D.M.1
Parton, M.J.2
Al-Chalabi, A.3
Lewis, C.M.4
Vance, C.5
Smith, B.N.6
Leigh, P.N.7
Powell, J.F.8
Siddique, T.9
Meyjes, E.P.10
Baas, F.11
De Jong, V.12
Shaw, C.E.13
-
95
-
-
15744378126
-
The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology
-
DOI 10.1016/j.cub.2005.01.058
-
Fujii R., Okabe S., Urushido T., Inoue K., Yoshimura A., Tachibana T., Nishikawa T., Hicks G. G., Takumi T., The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology Current Biology 2005 15 6 587 593 (Pubitemid 40413401)
-
(2005)
Current Biology
, vol.15
, Issue.6
, pp. 587-593
-
-
Fujii, R.1
Okabe, S.2
Urushido, T.3
Inoue, K.4
Yoshimura, A.5
Tachibana, T.6
Nishikawa, T.7
Hicks, G.G.8
Takumi, T.9
-
96
-
-
33749632259
-
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
-
DOI 10.1126/science.1134108
-
Neumann M., Sampathu D. M., Kwong L. K., Truax A. C., Micsenyi M. C., Chou T. T., Bruce J., Schuck T., Grossman M., Clark C. M., McCluskey L. F., Miller B. L., Masliah E., Mackenzie I. R., Feldman H., Feiden W., Kretzschmar H. A., Trojanowski J. Q., Lee V. M. Y., Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Science 2006 314 5796 130 133 (Pubitemid 44547757)
-
(2006)
Science
, vol.314
, Issue.5796
, pp. 130-133
-
-
Neumann, M.1
Sampathu, D.M.2
Kwong, L.K.3
Truax, A.C.4
Micsenyi, M.C.5
Chou, T.T.6
Bruce, J.7
Schuck, T.8
Grossman, M.9
Clark, C.M.10
McCluskey, L.F.11
Miller, B.L.12
Masliah, E.13
Mackenzie, I.R.14
Feldman, H.15
Feiden, W.16
Kretzschmar, H.A.17
Trojanowski, J.Q.18
Lee, V.M.-Y.19
-
97
-
-
62149141328
-
Rethinking ALS: The FUS about TDP-43
-
Lagier-Tourenne C., Cleveland D. W., Rethinking ALS: the FUS about TDP-43 Cell 2009 136 6 1001 1004
-
(2009)
Cell
, vol.136
, Issue.6
, pp. 1001-1004
-
-
Lagier-Tourenne, C.1
Cleveland, D.W.2
-
98
-
-
77949897022
-
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies
-
Geser F., Lee V. M. Y., Trojanowski J. Q., Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies Neuropathology 2010 30 2 103 112
-
(2010)
Neuropathology
, vol.30
, Issue.2
, pp. 103-112
-
-
Geser, F.1
Lee, V.M.Y.2
Trojanowski, J.Q.3
-
99
-
-
69549114542
-
The molecular links between TDP-43 dysfunction and neurodegeneration
-
Buratti E., Baralle F. E., The molecular links between TDP-43 dysfunction and neurodegeneration Advances in Genetics 2009 66 1 34
-
(2009)
Advances in Genetics
, vol.66
, pp. 1-34
-
-
Buratti, E.1
Baralle, F.E.2
-
100
-
-
33644551565
-
A motor neuron disease-associated mutation in p150 perturbs dynactin function and induces protein aggregation
-
Levy J. R., Sumner C. J., Caviston J. P., Tokito M. K., Ranganathan S., Ligon L. A., Wallace K. E., LaMonte B. H., Harmison G. G., Puls I., Fischbeck K. H., Holzbaur E. L. F., A motor neuron disease-associated mutation in p150 perturbs dynactin function and induces protein aggregation Journal of Cell Biology 2006 172 5 733 745
-
(2006)
Journal of Cell Biology
, vol.172
, Issue.5
, pp. 733-745
-
-
Levy, J.R.1
Sumner, C.J.2
Caviston, J.P.3
Tokito, M.K.4
Ranganathan, S.5
Ligon, L.A.6
Wallace, K.E.7
Lamonte, B.H.8
Harmison, G.G.9
Puls, I.10
Fischbeck, K.H.11
Holzbaur, E.L.F.12
-
101
-
-
0021167918
-
Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis
-
Hirano A., Donnenfeld H., Sasaki S., Nakano I., Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis Journal of Neuropathology and Experimental Neurology 1984 43 5 461 470 (Pubitemid 14027581)
-
(1984)
Journal of Neuropathology and Experimental Neurology
, vol.43
, Issue.5
, pp. 461-470
-
-
Hirano, A.1
Donnenfeld, H.2
Sasaki, S.3
Nakano, I.4
-
102
-
-
0030034545
-
SOD1 mutation is associated with accumulation of neurofilaments in amyotrophic lateral sclerosis
-
DOI 10.1002/ana.410390119
-
Rouleau G. A., Clark A. W., Rooke K., Pramatarova A., Krizus A., Suchowersky O., Julien J. P., Figlewicz D., SOD1 mutation is associated with accumulation of neurofilaments in amyotrophic lateral sclerosis Annals of Neurology 1996 39 1 128 131 (Pubitemid 26052227)
-
(1996)
Annals of Neurology
, vol.39
, Issue.1
, pp. 128-131
-
-
Rouleau, G.A.1
Clark, A.W.2
Rooke, K.3
Pramatarova, A.4
Krizus, A.5
Suchowersky, O.6
Julien, J.-P.7
Figlewicz, D.8
-
103
-
-
0032926368
-
Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis
-
DOI 10.1093/hmg/8.2.157
-
Al-Chalabi A., Andersen P. M., Nilsson P., Chioza B., Andersson J. L., Russ C., Shaw C. E., Powell J. F., Leigh P. N., Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis Human Molecular Genetics 1999 8 2 157 164 (Pubitemid 29054257)
-
(1999)
Human Molecular Genetics
, vol.8
, Issue.2
, pp. 157-164
-
-
Al-Chalabi, A.1
Andersen, P.M.2
Nilsson, P.3
Chioza, B.4
Andersson, J.L.5
Russ, C.6
Shaw, C.E.7
Powell, J.F.8
Leigh, P.N.9
-
104
-
-
0035936804
-
Amyotrophic lateral sclerosis: Unfolding the toxicity of the misfolded
-
Julien J. P., Amyotrophic lateral sclerosis: unfolding the toxicity of the misfolded Cell 2001 104 4 581 591
-
(2001)
Cell
, vol.104
, Issue.4
, pp. 581-591
-
-
Julien, J.P.1
-
105
-
-
0033911099
-
A new variant of Charcot-Marie-Tooth disease type 2 is probably the result of a mutation in the neurofilament-light gene
-
DOI 10.1086/302962
-
Mersiyanova I. V., Perepelov A. V., Polyakov A. V., Sitnikov V. F., Dadali E. L., Oparin R. B., Petrin A. N., Evgrafov O. V., A new variant of Charcot-Marie-Tooth disease type 2 is probably the result of a mutation in the neurofilament-light gene American Journal of Human Genetics 2000 67 1 37 46 (Pubitemid 30481542)
-
(2000)
American Journal of Human Genetics
, vol.67
, Issue.1
, pp. 37-46
-
-
Mersiyanova, I.V.1
Perepelov, A.V.2
Polyakov, A.V.3
Sitnikov, V.F.4
Dadali, E.L.5
Oparin, R.B.6
Petrin, A.N.7
Evgrafov, O.V.8
-
106
-
-
73449137883
-
Post-transcriptional control of neurofilaments: New roles in development, regeneration and neurodegenerative disease
-
Szaro B. G., Strong M. J., Post-transcriptional control of neurofilaments: new roles in development, regeneration and neurodegenerative disease Trends in Neurosciences 2010 33 1 27 37
-
(2010)
Trends in Neurosciences
, vol.33
, Issue.1
, pp. 27-37
-
-
Szaro, B.G.1
Strong, M.J.2
-
107
-
-
0036759174
-
Untranslated element in neurofilament mRNA has neuropathic effect on motor neurons of transgenic mice
-
Nie Z., Wu J., Zhai J., Lin H., Ge W., Schlaepfer W. W., Caete-Soler R., Untranslated element in neurofilament mRNA has neuropathic effect on motor neurons of transgenic mice Journal of Neuroscience 2002 22 17 7662 7670 (Pubitemid 35386365)
-
(2002)
Journal of Neuroscience
, vol.22
, Issue.17
, pp. 7662-7670
-
-
Nie, Z.1
Wu, J.2
Zhai, J.3
Lin, H.4
Ge, W.5
Schlaepfer, W.W.6
Canete-Soler, R.7
-
108
-
-
4143140690
-
A pathogenic peripherin gene mutation in a patient with amyotrophic lateral sclerosis
-
Leung C. L., He C. Z., Kaufmann P., Chin S. S., Naini A., Liem R. K. H., Mitsumoto H., Hays A. P., A pathogenic peripherin gene mutation in a patient with amyotrophic lateral sclerosis Brain Pathology 2004 14 3 290 296 (Pubitemid 39095991)
-
(2004)
Brain Pathology
, vol.14
, Issue.3
, pp. 290-296
-
-
Leung, C.L.1
He, C.Z.2
Kaufmann, P.3
Chin, S.S.4
Naini, A.5
Liem, R.K.H.6
Mitsumoto, H.7
Hays, A.P.8
-
109
-
-
8544222694
-
A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis
-
DOI 10.1074/jbc.M408139200
-
Gros-Louis F., Larivire R., Gowing G., Laurent S., Camu W., Bouchard J. P., Meininger V., Rouleau G. A., Julien J. P., A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis Journal of Biological Chemistry 2004 279 44 45951 45956 (Pubitemid 39491588)
-
(2004)
Journal of Biological Chemistry
, vol.279
, Issue.44
, pp. 45951-45956
-
-
Gros-Louis, F.1
Lariviere, R.2
Gowing, G.3
Laurent, S.4
Camu, W.5
Bouchard, J.-P.6
Meininger, V.7
Rouleau, G.A.8
Julien, J.-P.9
-
110
-
-
0027484249
-
Disruption of the CNTF gene results in motor neuron degeneration
-
DOI 10.1038/365027a0
-
Masu Y., Wolf E., Holtmann B., Sendtner M., Brem G., Thoenen H., Disruption of the CNTF gene results in motor neuron degeneration Nature 1993 365 6441 27 32 (Pubitemid 23305564)
-
(1993)
Nature
, vol.365
, Issue.6441
, pp. 27-32
-
-
Masu, Y.1
Wolf, E.2
Holtmann, B.3
Sendtner, M.4
Brem, G.5
Thoenen, H.6
-
112
-
-
77952986361
-
Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouse
-
Taes I., Goris A., Lemmens R., Van Es M. A., Van Den Berg L. H., Chio A., Traynor B. J., Birve A., Andersen P., Slowik A., Tomik B., Brown R. H., Shaw C. E., Al-Chalabi A., Boonen S., Van Den Bosch L., Dubois B., Van Damme P., Robberecht W., Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouse Neurology 2010 74 21 1687 169
-
(2010)
Neurology
, vol.74
, Issue.21
, pp. 1687-1693
-
-
Taes, I.1
Goris, A.2
Lemmens, R.3
Van Es, M.A.4
Van Den Berg, L.H.5
Chio, A.6
Traynor, B.J.7
Birve, A.8
Andersen, P.9
Slowik, A.10
Tomik, B.11
Brown, R.H.12
Shaw, C.E.13
Al-Chalabi, A.14
Boonen, S.15
Van Den Bosch, L.16
Dubois, B.17
Van Damme, P.18
Robberecht, W.19
|