ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

Virus‐delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis

Miller, T. M., Kaspar, B. K., Kops, G. J. P. L., Yamanaka, K., Christian, L. J., Gage, F. H., & Cleveland, D. W. (2005) · Annals of Neurology · doi:10.1002/ana.20453 · Cited by 123

Mitochondrial Proteomic Analysis of a Cell Line Model of Familial Amyotrophic Lateral Sclerosis

Fukada, K., Zhang, F., Vien, A., Cashman, N. R., & Zhu, H. (2004) · Molecular & Cellular Proteomics · doi:10.1074/mcp.m400094-mcp200 · Cited by 122

Follow-Up Study on Amyotrophic Lateral Sclerosis in Rochester, Minn., 1925 through 1984

Yoshida, S., Mulder, D. W., Kurland, L. T., Chu, C.-P., & Okazaki, H. (1986) · Neuroepidemiology · doi:10.1159/000110815 · Cited by 121

Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells

Sobue, G., Hashizume, Y., Yasuda, T., Mukai, E., Kumagai, T., Mitsuma, T., & Trojanowski, J. Q. (1990) · Acta Neuropathologica · doi:10.1007/bf00308716 · Cited by 121

Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis

Vielhaber, S., Winkler, K., Kirches, E., Kunz, D., Büchner, M., Feistner, H., Elger, C. E., Ludolph, A. C., Riepe, M. W., & Kunz, W. S. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00236-1 · Cited by 121

Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis

Afifi, A. K., ALEU, F. P., GOODGOLD, J., & MacKay, B. (1966) · Neurology · doi:10.1212/wnl.16.5.475 · Cited by 121

The WldS gene modestly prolongs survival in the SOD1G93A fALS mouse

Fischer, L. R., Culver, D. G., Davis, A. A., Tennant, P., Wang, M.-S., Coleman, M. P., Asress, S., Adalbert, R., Alexander, G. M., & Glass, J. D. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.01.008 · Cited by 116

Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1

Takeuchi, H., Kobayashi, Y., Yoshihara, T., Niwa, J., Doyu, M., Ohtsuka, K., & Sobue, G. (2002) · Brain Research · doi:10.1016/s0006-8993(02)02568-4 · Cited by 114

Viral-Induced Spinal Motor Neuron Death Is Non-Cell-Autonomous and Involves Glutamate Excitotoxicity

Darman, J., Backovic, S., Dike, S., Maragakis, N. J., Krishnan, C., Rothstein, J. D., Irani, D. N., & Kerr, D. A. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.2002-04.2004 · Cited by 114

Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA

Wang, J., Xu, G. M., Slunt, H. H., Gonzales, V., Coonfield, M. L., Fromholt, D., Copeland, N. G., Jenkins, N. A., & Borchelt, D. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.06.005 · Cited by 110

AMYOTROPHIC LATERAL SCLEROSIS: Successful Treatment of Mucous Plugging by Mechanical Insufflation-Exsufflation1

Hanayama, K., Ishikawa, Y., & Bach, J. R. (1997) · American Journal of Physical Medicine & Rehabilitation · doi:10.1097/00002060-199707000-00017 · Cited by 110

Excitatory amino acid transporter 1 and 2 immunoreactivity in the spinal cord in amyotrophic lateral sclerosis

Sasaki, S., Komori, T., & Iwata, M. (2000) · Acta Neuropathologica · doi:10.1007/s004019900159 · Cited by 108

Beneficial effects of intrathecal IGF-1 administration in patients with amyotrophic lateral sclerosis

Nagano, I., Shiote, M., Murakami, T., Kamada, H., Hamakawa, Y., Matsubara, E., Yokoyama, M., Morita, K., Shoji, M., & Abe, K. (2005) · Neurological Research · doi:10.1179/016164105x39860 · Cited by 107

Overloading of Stable and Exclusion of Unstable Human Superoxide Dismutase-1 Variants in Mitochondria of Murine Amyotrophic Lateral Sclerosis Models

Bergemalm, D., Jonsson, P. A., Graffmo, K. S., Andersen, P. M., Brännström, T., Rehnmark, A., & Marklund, S. L. (2006) · Journal of Neuroscience · doi:10.1523/jneurosci.5461-05.2006 · Cited by 106

Origin and distribution of bone marrow-derived cells in the central nervous system in a mouse model of amyotrophic lateral sclerosis

Solomon, J. N., Lewis, C. B., Ajami, B., Corbel, S. Y., Rossi, F. M. V., & Krieger, C. (2006) · Glia · doi:10.1002/glia.20331 · Cited by 105

Can amyotrophic lateral sclerosis patients with respiratory insufficiency exercise?

Pinto, A. C., Alves, M., Nogueira, A., Evangelista, T., Carvalho, J., Coelho, A., de Carvalho, M. A., & Sales-Luis, M. L. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00218-x · Cited by 104

Myostatin inhibition slows muscle atrophy in rodent models of amyotrophic lateral sclerosis

Holzbaur, E. L. F., Howland, D., Weber, N., Wallace, K. E., She, Y., Kwak, S., Tchistiakova, L. A., Murphy, E. K., Hinson, J., Karim, R., et al. (2006) · Neurobiology of Disease · doi:10.1016/j.nbd.2006.05.009 · Cited by 104

Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice

Basso, M., Massignan, T., Samengo, G., Cheroni, C., Biasi, S. D., Salmona, M., Bendotti, C., & Bonetto, V. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m603489200 · Cited by 103

Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis

Liu, J., Shinobu, L. A., Ward, C. M., Young, D., & Cleveland, D. W. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03054.x · Cited by 99

Selective up-regulation of the glial Na+-dependent glutamate transporter GLT1 by a neuroimmunophilin ligand results in neuroprotection

Ganel, R., Ho, T. W., Maragakis, N. J., Jackson, M. T., Steiner, J. P., & Rothstein, J. D. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.08.014 · Cited by 98

Ultrastructural Changes of Mitochondria in the Skeletal Muscle of Patients with Amyotrophic Lateral Sclerosis

Chung, M. J., & Suh, Y.-L. (2002) · Ultrastructural Pathology · doi:10.1080/01913120252934260 · Cited by 96

Therapeutic benefit of intrathecal injection of insulin-like growth factor-1 in a mouse model of Amyotrophic Lateral Sclerosis

Nagano, I., Ilieva, H. S., Shiote, M., Murakami, T., Yokoyama, M., Shoji, M., & Abe, K. (2005) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2005.04.011 · Cited by 96

Upregulation of HSP27 in a Transgenic Model of ALS

Vleminckx, V., Damme, P. V., Goffin, K. E., Delye, H., Bosch, L. V. D., & Robberecht, W. (2002) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/61.11.968 · Cited by 96

Allele-specific RNAi selectively silences mutant SOD1 and achieves significant therapeutic benefit in vivo

Xia, X., Zhou, H., Huang, Y., & Xu, Z. (2006) · Neurobiology of Disease · doi:10.1016/j.nbd.2006.04.019 · Cited by 95

Hsp27 and Hsp70 administered in combination have a potent protective effect against FALS-associated SOD1-mutant-induced cell death in mammalian neuronal cells

Patel, Y. J. K., Smith, M. D. P., de Belleroche, J., & Latchman, D. S. (2004) · Molecular Brain Research · doi:10.1016/j.molbrainres.2004.10.028 · Cited by 93

神経フィラメントの欠如は,家族性筋萎縮性側索硬化症関連スーパオキジドジスムターゼ1変異に対する運動ニューロンの選択的易損性を低下させ,疾患を遅らせる

Williamson, T. L., Bruijn, L. I., Zhu, Q., Anderson, K. L., Anderson, S. D., Julien, J., & Cleveland, D. W. (1998) · Proceedings of the National Academy of Sciences · Cited by 93

Upregulation of Bax protein and increased DNA degradation in ALS spinal cord motor neurons

Ekegren, T., Grundström, E., Lindholm, D., & Aquilonius, S. M. (2009) · Acta Neurologica Scandinavica · doi:10.1111/j.1600-0404.1999.tb00403.x · Cited by 91

Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis

Kong, J., & Xu, Z. (2000) · Neuroscience Letters · doi:10.1016/s0304-3940(00)00808-9 · Cited by 89

A novel locus for late onset amyotrophic lateral sclerosis/motor neurone disease variant at 20q13

Nishimura, A. L., Mitne-Neto, M., Silva, H. C. A., Oliveira, J. R. M., Vainzof, M., & Zatz, M. (2004) · Journal of Medical Genetics · doi:10.1136/jmg.2003.013029 · Cited by 89

Focal accumulation of phosphorylated neurofilaments within anterior horn cell in familial amyotrophic lateral sclerosis

Mizusawa, H., Matsumoto, S., Yen, S. H., Hirano, A., Rojas-Corona, R. R., & Donnenfeld, H. (1989) · Acta Neuropathologica · doi:10.1007/bf00308955 · Cited by 88

Two Families with Familial Amyotrophic Lateral Sclerosis Are Linked to a Novel Locus on Chromosome 16q

Ruddy, D., Parton, M. J., Al‐Chalabi, A., Lewis, C. M., Vance, C. A., Smith, B. N., Leigh, P. N., Powell, J. F., Siddique, T., Meyjes, E. F. P., et al. (2003) · The American Journal of Human Genetics · doi:10.1086/377157 · Cited by 87

Reactions of the Immune System in Chronic Degenerative Neurological Diseases

McGeer, P. L., McGeer, E. G., Kawamata, T., Yamada, T., & Akiyama, H. (1991) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100032479 · Cited by 86

Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS

Lobsiger, C. S., Garcia, M. L., Ward, C. M., & Cleveland, D. W. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0503862102 · Cited by 81

Progressive spinal axonal degeneration and slowness in ALS2‐deficient mice

Yamanaka, K., Miller, T. M., McAlonis‐Downes, M., Chun, S. J., & Cleveland, D. W. (2006) · Annals of Neurology · doi:10.1002/ana.20888 · Cited by 80

Cu/Zn-superoxide dismutase (GLY93→ALA) mutation alters AMPA receptor subunit expression and function and potentiates kainate-mediated toxicity in motor neurons in culture

Spalloni, A., Albo, F., Ferrari, F., Mercuri, N. B., Bernardi, G., Zona, C., & Longone, P. (2004) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.11.012 · Cited by 79

The Neuroprotective Factor Wlds Does Not Attenuate Mutant SOD1-Mediated Motor Neuron Disease

Velde, C. V., Garcia, M. L., Yin, X., Trapp, B. D., & Cleveland, D. W. (2004) · NeuroMolecular Medicine · doi:10.1385/nmm:5:3:193 · Cited by 77

Ultrastructural study of mitochondria in the spinal cord of transgenic mice with a G93A mutant SOD1 gene

Sasaki, S., Warita, H., Murakami, T., Abe, K., & Iwata, M. (2004) · Acta Neuropathologica · doi:10.1007/s00401-004-0837-z · Cited by 76

A quantitative histochemical assay for activities of mitochondrial electron transport chain complexes in mouse spinal cord sections

Jung, C., Higgins, C. M. J., & Xu, Z. (2002) · Journal of Neuroscience Methods · doi:10.1016/s0165-0270(01)00524-6 · Cited by 75

Mitochondrial respiratory chain function in skeletal muscle of ALS patients

Echaniz‐Laguna, A., Zoll, J., Ribera, F., Tranchant, C., Warter, J., Lonsdorfer, J., & Lampert, E. (2002) · Annals of Neurology · doi:10.1002/ana.10357 · Cited by 75

Rapid disease progression correlates with instability of mutant SOD1 in familial ALS

Sato, T., Nakanishi, T., Yamamoto, Y., Andersen, P. M., Ogawa, Y., Fukada, K., Zhou, Z., Aoike, F., Sugai, F., Nagano, S., et al. (2005) · Neurology · doi:10.1212/01.wnl.0000188760.53922.05 · Cited by 72

The adult neural stem and progenitor cell niche is altered in amyotrophic lateral sclerosis mouse brain

Liu, Z., & Martin, L. J. (2006) · The Journal of Comparative Neurology · doi:10.1002/cne.21012 · Cited by 69

Activation of programmed cell death markers in ventral horn motor neurons during early presymptomatic stages of amyotrophic lateral sclerosis in a transgenic mouse model

Wengenack, T. M., Holasek, S. S., Montaño, C., Gregor, D. M., Curran, G. L., & Poduslo, J. F. (2004) · Brain Research · doi:10.1016/j.brainres.2004.08.054 · Cited by 67

Non-neuronal induction of immunoproteasome subunits in an ALS model: Possible mediation by cytokines

Puttaparthi, K., & Elliott, J. L. (2005) · Experimental Neurology · doi:10.1016/j.expneurol.2005.08.027 · Cited by 66

A New Familial Amyotrophic Lateral Sclerosis Locus on Chromosome 16q12.1-16q12.2

Abalkhail, H. A., Mitchell, J., Habgood, J., Orrell, R. W., & de Belleroche, J. (2003) · The American Journal of Human Genetics · doi:10.1086/377156 · Cited by 66

A dynein mutation attenuates motor neuron degeneration in SOD1G93A mice

Teuchert, M., Fischer, D., Schwalenstöcker, B., Habisch, H., Böckers, T. M., & Ludolph, A. C. (2006) · Experimental Neurology · doi:10.1016/j.expneurol.2005.12.005 · Cited by 66

Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA

Kawahara, Y., Sun, H. J., Ito, K., Hideyama, T., Aoki, M., Sobue, G., Tsuji, S., & Kwak, S. (2005) · Neuroscience Research · doi:10.1016/j.neures.2005.09.006 · Cited by 63

Lack of association between VEGF polymorphisms and ALS in a Dutch population

van Vught, P. W. J., Sutedja, N. A., Veldink, J. H., Koeleman, B. P. C., Groeneveld, G. J., Wijmenga, C., Uitdehaag, B. M. J., de Jong, J. M. B. V., Baas, F., Wokke, J. H. J., et al. (2005) · Neurology · doi:10.1212/01.wnl.0000184514.39853.56 · Cited by 52

Inhibition of Chaperone Activity Is a Shared Property of Several Cu,Zn-Superoxide Dismutase Mutants That Cause Amyotrophic Lateral Sclerosis

Tummala, H., Jung, C., Tiwari, A., Higgins, C. M. J., Hayward, L. J., & Xu, Z. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m501705200 · Cited by 51

Neuronophagia in the motor cortex in amyotrophic lateral sclerosis

Troost, D., Claessen, N., van den Oord, J. J., Swaab, D. F., & de Jong, J. M. B. V. (1993) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.1993.tb00459.x · Cited by 44

Minocycline in amyotrophic lateral sclerosis: a pilot study

Pontieri, F. E., Rícci, A., Pellicano, C., Benincasa, D., & Buttarelli, F. R. (2005) · Neurological Sciences · doi:10.1007/s10072-005-0474-x · Cited by 41

Peripherin is not a contributing factor to motor neuron disease in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase

Larivière, R., Beaulieu, J., Nguyen, M. D., & Julien, J. (2003) · Neurobiology of Disease · doi:10.1016/s0969-9961(03)00036-6 · Cited by 40

VEGF PROMOTER HAPLOTYPE AND AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Terry, P., Kamel, F., Umbach, D. M., Lehman, T. A., Hu, H., Sandler, D. P., & Taylor, J. A. (2004) · Journal of Neurogenetics · doi:10.1080/01677060490894450 · Cited by 39

Informatics-assisted Protein Profiling in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

Lukas, T. J., Luo, W., Mao, H., Cole, N., & Siddique, T. (2006) · Molecular & Cellular Proteomics · doi:10.1074/mcp.m500431-mcp200 · Cited by 39

Lack of association of VEGF promoter polymorphisms with sporadic ALS

Chen, W., Saeed, M., Mao, H., Siddique, N. A., Dellefave‐Castillo, L. M., Hung, W.-Y., Deng, H., Sufit, R. L., Heller, S. L., Haines, J. L., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000227926.42370.04 · Cited by 39

Neurotrophin-3 (NT-3) modulates early differentiation of oligodendrocytes in rat brain cortical cultures

Heinrich, M., Gorath, M., & Richter‐Landsberg, C. (1999) · Glia · doi:10.1002/(sici)1098-1136(199912)28:3<215::aid-glia5>3.0.co;2-c · Cited by 38

Bcl-2 Sensitivity Differentiates Two Pathways for Motoneuronal Death in thewobblerMutant Mouse

Coulpier, M., Junier, M., Peschanski, M., & Dreyfus, P. A. (1996) · Journal of Neuroscience · doi:10.1523/jneurosci.16-19-05897.1996 · Cited by 37

Quantitative evidence for neurofilament heavy subunit aggregation in motor neurons of spinal cords of patients with amyotrophic lateral sclerosis

de Mendonça, D. M. F., Chimelli, L. M. C., & Martinez, A. M. B. (2005) · Brazilian Journal of Medical and Biological Research · doi:10.1590/s0100-879x2005000600015 · Cited by 35

Absence of mutations in the hypoxia response element of VEGF in ALS

Gros‐Louis, F., Laurent, S. B., Lopes, A. A. S., et al. (2003) · Muscle & Nerve · doi:10.1002/mus.10498 · Cited by 34

Bcl-2 Overexpression Does Not Protect Neurons from Mutant Neurofilament-Mediated Motor Neuron Degeneration

Houseweart, M. K., & Cleveland, D. W. (1999) · Journal of Neuroscience · doi:10.1523/jneurosci.19-15-06446.1999 · Cited by 28

Paradigms for the identification of new genes in motor neuron degeneration

Hafezparast, M., Ahmad‐Annuar, A., Hummerich, H., Shah, P., Ford, M., Baker, C., Bowen, S., Martin, J. E., & Fisher, E. (2003) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820310016084 · Cited by 21

No evidence of linkage to chromosome 9q21–22 in a Swedish family with frontotemporal dementia and amyotrophic lateral sclerosis

Ostojic, J., Axelman, K., Lannfelt, L., & Froelich-Fabre, S. (2003) · Neuroscience Letters · doi:10.1016/s0304-3940(03)00126-5 · Cited by 13

Role for peroxisome proliferator-activated receptor-gamma in Alzheimer's disease.

van Geel (2001) · Open Repository and Bibliography (University of Luxembourg) · doi:10.1002/1531-8249(20010201)49:2 · Cited by 7

The Blood-Brain Barrier in Health and Chronic Neurodegenerative Disorders

Zlokovic, B. V. (2008) · Neuron · doi:10.1016/j.neuron.2008.01.003 · Cited by 3,280

Neuroinflammation in neurodegenerative disorders: the roles of microglia and astrocytes

Kwon, H. S., & Koh, S.-H. (2020) · Translational Neurodegeneration · doi:10.1186/s40035-020-00221-2 · Cited by 2,595

Differential Roles of M1 and M2 Microglia in Neurodegenerative Diseases

Tang, Y., & Le, W. (2016) · Molecular Neurobiology · doi:10.1007/s12035-014-9070-5 · Cited by 2,190

How neuroinflammation contributes to neurodegeneration

Ransohoff, R. M. (2016) · Science · doi:10.1126/science.aag2590 · Cited by 1,967

Reactive Gliosis and the Multicellular Response to CNS Damage and Disease

Burda, J. E., & Sofroniew, M. V. (2014) · Neuron · doi:10.1016/j.neuron.2013.12.034 · Cited by 1,438