ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions

Superoxide dismutase: Improved assays and an assay applicable to acrylamide gels

Beauchamp, C., & Fridovich, I. (1971) · Analytical Biochemistry · doi:10.1016/0003-2697(71)90370-8 · Cited by 12,892 · see citing works

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062 · see citing works

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143 · see citing works

GDNF: a Glial Cell Line-Derived Neurotrophic Factor for Midbrain Dopaminergic Neurons

Lin, L.-F. H., Doherty, D. H., Lile, J. D., Bektesh, S. L., & Collins, F. (1993) · Science · doi:10.1126/science.8493557 · Cited by 3,206 · see citing works

Free radicals, antioxidants, and human disease: curiosity, cause, or consequence?

Halliwell, B. B. (1994) · The Lancet · doi:10.1016/s0140-6736(94)92211-x · Cited by 2,926 · see citing works

Knockout of Glutamate Transporters Reveals a Major Role for Astroglial Transport in Excitotoxicity and Clearance of Glutamate

Rothstein, J. D., Dykes-Hoberg, M., Pardo, C. A., Bristol, L. A., Jin, L., Kuncl, R. W., Kanai, Y., Hediger, M. A., Wang, Y., Schielke, J. P., et al. (1996) · Neuron · doi:10.1016/s0896-6273(00)80086-0 · Cited by 2,534 · see citing works

Cellular defenses against damage from reactive oxygen species

Yu, B. (1994) · Physiological Reviews · doi:10.1152/physrev.1994.74.1.139 · Cited by 2,378 · see citing works

Localization of neuronal and glial glutamate transporters

Rothstein, J. D., Martin, L. J., Levey, A. I., Dykes‐Hoberg, M., Jin, L., Wu, D. M., Nash, N. R., & Kuncl, R. W. (1994) · Neuron · doi:10.1016/0896-6273(94)90038-8 · Cited by 1,703 · see citing works

Amyotrophic Lateral Ssclerosis and Structural Defects in Cu,Zn Superoxide Dismutase

Deng, H., Hentati, A., Tainer, J. A., Iqbal, Z., Cayabyab, A., Hung, W.-Y., Getzoff, E. D., Hu, P., Herzfeldt, B., Roos, R. P., et al. (1993) · Science · doi:10.1126/science.8351519 · Cited by 1,479 · see citing works

Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis

Rothstein, J. D., Van Kammen, M., Levey, A. I., Martin, L. J., & Kuncl, R. W. (1995) · Annals of Neurology · doi:10.1002/ana.410380114 · Cited by 1,471 · see citing works

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., Lee, M. K., Copeland, N. G., Jenkins, N. A., Sisodia, S. S., Cleveland, D. W., & Price, D. L. (1995) · Neuron · doi:10.1016/0896-6273(95)90259-7 · Cited by 1,439 · see citing works

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

Reaume, A. G., Elliott, J. L., Hoffman, E. K., Kowall, N. W., Ferrante, R. J., Siwek, D. F., Wilcox, H. M., Flood, D. G., Beal, M. F., Brown, R. H., et al. (1996) · Nature Genetics · doi:10.1038/ng0596-43 · Cited by 1,285 · see citing works

Cloning and expression of a rat brain L-glutamate transporter

Pines, G., Danbolt, N. C., Bjørås, M., Zhang, Y., Bendahan, A., Eide, L., Koepsell, H., Storm‐Mathisen, J., Seeberg, E. C., & Kanner, B. I. (1992) · Nature · doi:10.1038/360464a0 · Cited by 1,232 · see citing works

Structure, expression, and functional analysis of a Na(+)-dependent glutamate/aspartate transporter from rat brain.

Storck, T., Schulte, S., Hofmann, K., & Stoffel, W. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.22.10955 · Cited by 1,185 · see citing works

Decreased Glutamate Transport by the Brain and Spinal Cord in Amyotrophic Lateral Sclerosis

Rothstein, J. D., Martin, L. J., & Kuncl, R. W. (1992) · New England Journal of Medicine · doi:10.1056/nejm199205283262204 · Cited by 1,180 · see citing works

ALS, SOD and peroxynitrite

Beckman, J. S., Carson, M. A., Smith, C. D. L., & Koppenol, W. H. (1993) · Nature · doi:10.1038/364584a0 · Cited by 876 · see citing works

Superoxide dismutase delays neuronal apoptosis: A role for reactive oxygen species in programmed neuronal death

Greenlund, L. J. S., Deckwerth, T. L., & Johnson, E. M. (1995) · Neuron · doi:10.1016/0896-6273(95)90287-2 · Cited by 738 · see citing works

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., & Gordon, J. W. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.3.689 · Cited by 725 · see citing works

Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis

Wiedau‐Pazos, M., Goto, J. J., Rabizadeh, S., Gralla, E. B., Roe, J. A., Lee, M. K., Valentine, J. S., & Bredesen, D. E. (1996) · Science · doi:10.1126/science.271.5248.515 · Cited by 720 · see citing works

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

Rothstein, J. D., Tsai, G. E., Kuncl, R. W., Clawson, L., Cornblath, D. R., Drachman, D. B., Pestronk, A., Stauch, B. L., & Coyle, J. T. (1990) · Annals of Neurology · doi:10.1002/ana.410280106 · Cited by 637 · see citing works

Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis

Bowling, A. C., Schulz, J. B., Brown, R. H., & Beal, M. F. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb07478.x · Cited by 629 · see citing works

Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity.

Borchelt, D., Lee, M. K., Slunt, H. S., Guarnieri, M., Xu, Z. S., Wong, P. C., Brown, R. H., Price, D. L., Sisodia, S. S., & Cleveland, D. W. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.17.8292 · Cited by 622 · see citing works

Transgenic mice with increased Cu/Zn-superoxide dismutase activity: animal model of dosage effects in Down syndrome.

Epstein, C. J., Avraham, K. B., Lovett, M., Smith, S. H., Elroy‐Stein, O., Rotman, G., Bry, C., & Groner, Y. (1987) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.84.22.8044 · Cited by 454 · see citing works

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.

Canto, M. C. D., & Gurney, M. E. (1994) · PubMed · Cited by 395 · see citing works

Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons.

Pardo, C. A., Xu, Z., Borchelt, D., Price, D. L., Sisodia, S. S., & Cleveland, D. W. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.4.954 · Cited by 314 · see citing works

Intense Superoxide Dismutase-1 Immunoreactivity in Intracytoplasmic Hyaline Inclusions of Familial Amyotrophic Lateral Sclerosis with Posterior Column Involvement

Shibata, N., Hirano, A., Kobayashi, M., Siddique, T., Deng, H., Hung, W.-Y., Kato, T., & Asayama, K. (1996) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199604000-00011 · Cited by 294 · see citing works

Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons.

Rothstein, J. D., Bristol, L. A., Hosler, B. A., Brown, R. H., & Kuncl, R. W. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.10.4155 · Cited by 292 · see citing works

In vivo microtubules are copolymers of available beta-tubulin isotypes: localization of each of six vertebrate beta-tubulin isotypes using polyclonal antibodies elicited by synthetic peptide antigens.

Lopata, M. A., & Cleveland, D. W. (1987) · The Journal of Cell Biology · doi:10.1083/jcb.105.4.1707 · Cited by 232 · see citing works

A new family of neurotransmitter transporters: the high‐affinity glutamate transporters

Kanai, Y., Smith, C. P., & Hediger, M. A. (1993) · The FASEB Journal · doi:10.1096/fasebj.7.15.7903261 · Cited by 226 · see citing works

Reduction of Naturally Occurring Motoneuron Death in Vivo by a Target-Derived Neurotrophic Factor

Oppenheim, R. W., Haverkamp, L. J., Prevette, D. M., McManaman, J. L., & Appel, S. H. (1988) · Science · doi:10.1126/science.3363373 · Cited by 208 · see citing works

Neurofibrillary axonal swellings and amyotrophic lateral sclerosis.

Delisle, M., & Carpenter, S. (1984) · Journal of Neurological Sciences · doi:10.1016/0022-510x(84)90199-0 · Cited by 167 · see citing works

Rapid Communication: Cu/Zn Superoxide Dismutase Activity in Familial and Sporadic Amyotrophic Lateral Sclerosis

Robberecht, W., Sapp, P. C., Viaene, M. K., Rosen, D. R., McKenna‐Yasek, D., Haines, J. L., Horvitz, H. R., Theys, P., & Brown, R. H. (1994) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1994.62010384.x · Cited by 163 · see citing works

Superoxide Dismutase 1 Subunits with Mutations Linked to Familial Amyotrophic Lateral Sclerosis Do Not Affect Wild-type Subunit Function

Borchelt, D., Guarnieri, M., Wong, P. C., Lee, M. K., Slunt, H. S., Xu, Z., Sisodia, S. S., Price, D. L., & Cleveland, D. W. (1995) · Journal of Biological Chemistry · doi:10.1074/jbc.270.7.3234 · Cited by 152 · see citing works

Filaments of Lewy bodies contain insoluble cytoskeletal elements.

Galloway, P. G., Mulvihill, P., & Perry, G. L. (1992) · PubMed Central · Cited by 131 · see citing works

Familial Amyotrophic Lateral Sclerosis with a Two Base Pair Deletion in Superoxide Dismutase 1 Gene: Multisystem Degeneration with Intracytoplasmic Hyaline Inclusions in Astrocytes

Kato, S., Shimoda, M., Watanabe, Y., Nakashima, K., Takahashi, K., & Ohama, E. (1996) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199655100-00009 · Cited by 111 · see citing works

Nitric oxide inhibits 3H‐glutamate transport in synaptosomes

Pöğün, Ş., Dawson, V. L., & Kuhar, M. J. (1994) · Synapse · doi:10.1002/syn.890180104 · Cited by 102 · see citing works

Characterization of Wild‐Type and Amyotrophic Lateral Sclerosis‐Related Mutant Cu,Zn‐Superoxide Dismutases Overproduced in Baculovirus‐Infected Insect Cells

Fujii, J., Myint, T., Seo, H. G., Kayanoki, Y., Ikeda, Y., & Taniguchi, N. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.64041456.x · Cited by 34 · see citing works

[21] Automated assay of superoxide dismutase in blood

L’Abbé, M. R., & Fischer, P. W. F. (1990) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/0076-6879(90)86113-a · Cited by 33 · see citing works

Involvement of neurofilaments in motor neuron disease

Xu, Z., Cork, L. C., Griffin, J. W., & Cleveland, D. W. (1993) · Journal of Cell Science · doi:10.1242/jcs.1993.supplement_17.15 · Cited by 33 · see citing works

Glutamate uptake

Danbolt, N. C. (2001) · Progress in Neurobiology · doi:10.1016/s0301-0082(00)00067-8 · Cited by 4,068 · see citing works

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

Davies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L., & Bates, G. P. (1997) · Cell · doi:10.1016/s0092-8674(00)80513-9 · Cited by 2,188 · see citing works

Decoding ALS: from genes to mechanism

Taylor, J. P., Brown, R. H., & Cleveland, D. W. (2016) · Nature · doi:10.1038/nature20413 · Cited by 2,047 · see citing works

Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

Boillée, S., Yamanaka, K., Lobsiger, C. S., Copeland, N. G., Jenkins, N. A., Kassiotis, G., Kollias, G., & Cleveland, D. W. (2006) · Science · doi:10.1126/science.1123511 · Cited by 1,623 · see citing works

ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

Boillée, S., Velde, C. V., & Cleveland, D. W. (2006) · Neuron · doi:10.1016/j.neuron.2006.09.018 · Cited by 1,426 · see citing works

From charcot to lou gehrig: deciphering selective motor neuron death in als

Cleveland, D. W., & Rothstein, J. D. (2001) · Nature reviews. Neuroscience · doi:10.1038/35097565 · Cited by 1,389 · see citing works

UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS

Bruijn, L. I., Miller, T. M., & Cleveland, D. W. (2004) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.27.070203.144244 · Cited by 1,371 · see citing works

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

Nagai, M., Re, D. B., Nagata, T., Chalazonitis, A., Jessell, T. M., Wichterle, H., & Przedborski, S. (2007) · Nature Neuroscience · doi:10.1038/nn1876 · Cited by 1,220 · see citing works

Superoxide dismutases and superoxide reductases

Sheng, Y., Abreu, I. A., Cabelli, D. E., Maroney, M. J., Miller, A.-F., Teixeira, M., & Valentine, J. S. (2014) · Chemical Reviews · doi:10.1021/cr4005296 · Cited by 1,081 · see citing works

Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

Clement, A. M., Nguyen, M. D., Roberts, E. A., Garcia, M. L., Boillée, S., Rule, M., McMahon, A. P., Doucette, W. T., Siwek, D. F., Ferrante, R. J., et al. (2003) · Science · doi:10.1126/science.1086071 · Cited by 1,101 · see citing works

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

Ilieva, H., Polymenidou, M., & Cleveland, D. W. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200908164 · Cited by 1,008 · see citing works

Degradation of misfolded proteins in neurodegenerative diseases: Therapeutic targets and strategies

Ciechanover, A., & Kwon, Y. T. (2015) · Experimental & Molecular Medicine · doi:10.1038/emm.2014.117 · Cited by 898 · see citing works

The changing scene of amyotrophic lateral sclerosis

Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957 · see citing works

Retrograde Viral Delivery of IGF-1 Prolongs Survival in a Mouse ALS Model

Kaspar, B. K., Lladó, J., Sherkat, N., Rothstein, J. D., & Gage, F. H. (2003) · Science · doi:10.1126/science.1086137 · Cited by 872 · see citing works

The Copper Chaperone for Superoxide Dismutase

Culotta, V., Klomp, L. W. J., Strain, J. J., Casareno, R. L. B., Krems, B., & Gitlin, J. D. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.38.23469 · Cited by 851 · see citing works

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)

Howland, D., Liu, J., She, Y., Goad, B., Maragakis, N. J., Kim, B. B., Erickson, J., Kulik, J., DeVito, L., Psaltis, G. C., et al. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.032539299 · Cited by 841 · see citing works

Mechanisms of Disease: astrocytes in neurodegenerative disease

Maragakis, N., & Rothstein, J. (2006) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0355 · Cited by 834 · see citing works

Non–cell autonomous effect of glia on motor neurons in an embryonic stem cell–based ALS model

Di Giorgio, F. P., Carrasco, M. A., Siao, M. C., Maniatis, T., & Eggan, K. (2007) · Nature Neuroscience · doi:10.1038/nn1885 · Cited by 799 · see citing works

Astrocyte dysfunction in neurological disorders: a molecular perspective

Seifert, G., Schilling, K., & Steinhäuser, C. (2006) · Nature reviews. Neuroscience · doi:10.1038/nrn1870 · Cited by 798 · see citing works

COPPER-ZINC SUPEROXIDE DISMUTASE AND AMYOTROPHIC LATERAL SCLEROSIS

Valentine, J. S., Doucette, P. A., & Potter, S. Z. (2003) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.72.121801.161647 · Cited by 790 · see citing works

Axonal transport deficits and neurodegenerative diseases

Millecamps, S., & Julien, J.-P. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3380 · Cited by 755 · see citing works

Role of Axonal Transport in Neurodegenerative Diseases

Vos, K. J. D., Grierson, A. J., Ackerley, S., & Miller, C. C. J. (2008) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.31.061307.090711 · Cited by 734 · see citing works

Tumor necrosis factor alpha: A link between neuroinflammation and excitotoxicity

Olmos, G., & Lladó, J. (2014) · Mediators of Inflammation · doi:10.1155/2014/861231 · Cited by 730 · see citing works

The Role of Excitotoxicity in Neurodegenerative Disease

Doble, A. (1999) · Pharmacology & Therapeutics · doi:10.1016/s0163-7258(98)00042-4 · Cited by 723 · see citing works

Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1

Kong, J., & Xu, Z. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-09-03241.1998 · Cited by 723 · see citing works

Current hypotheses for the underlying biology of amyotrophic lateral sclerosis

Rothstein, J. D. (2009) · Annals of Neurology · doi:10.1002/ana.21543 · Cited by 510 · see citing works

Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

Pun, S., Santos, A. F., Saxena, S., Xu, L., & Caroni, P. (2006) · Nature Neuroscience · doi:10.1038/nn1653 · Cited by 659 · see citing works

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

Philips, T., & Robberecht, W. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70015-1 · Cited by 646 · see citing works

Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons

Williamson, T. L., & Cleveland, D. W. (1999) · Nature Neuroscience · doi:10.1038/4553 · Cited by 638 · see citing works

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

Barber, S. C., & Shaw, P. J. (2009) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2009.11.018 · Cited by 617 · see citing works

Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis

Johnston, J. A., Dalton, M. J., Gurney, M. E., & Kopito, R. R. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.220417997 · Cited by 607 · see citing works

Development of Multifunctional Molecules as Potential Therapeutic Candidates for Alzheimer’s Disease, Parkinson’s Disease, and Amyotrophic Lateral Sclerosis in the Last Decade

Savelieff, M. G., Nam, G., Kang, J., Lee, H. J., Lee, M., & Lim, M. H. (2018) · Chemical Reviews · doi:10.1021/acs.chemrev.8b00138 · Cited by 603 · see citing works

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis

Kang, S. H., Li, Y., Fukaya, M., Lorenzini, I., Cleveland, D. W., Ostrow, L. W., Rothstein, J. D., & Bergles, D. E. (2013) · Nature Neuroscience · doi:10.1038/nn.3357 · Cited by 599 · see citing works

The role of glutamate transporters in neurodegenerative diseases and potential opportunities for intervention

Sheldon, A. L., & Robinson, M. B. (2007) · Neurochemistry International · doi:10.1016/j.neuint.2007.03.012 · Cited by 593 · see citing works

VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model

Azzouz, M., Ralph, G. S., Storkebaum, E., Walmsley, L. E., Mitrophanous, K. A., Kingsman, S. M., Carmeliet, P., & Mazarakis, N. D. (2004) · Nature · doi:10.1038/nature02544 · Cited by 588 · see citing works

Coenzyme Q ₁₀ administration increases brain mitochondrial concentrations and exerts neuroprotective effects

Matthews, R. T., Yang, L., Browne, S., Baik, M., & Beal, M. F. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.15.8892 · Cited by 557 · see citing works

Lithium delays progression of amyotrophic lateral sclerosis

Fornai, F., Longone, P., Cafaro, L., Kastsiuchenka, O., Ferrucci, M., Manca, M. L., Lazzeri, G., Spalloni, A., Bellio, N., Lenzi, P., et al. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0708022105 · Cited by 550 · see citing works

Misfolded CuZnSOD and amyotrophic lateral sclerosis

Valentine, J. S., & Hart, P. J. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0730423100 · Cited by 546 · see citing works

Targeting programmed cell death in neurodegenerative diseases

Vila, M., & Przedborski, S. (2003) · Nature reviews. Neuroscience · doi:10.1038/nrn1100 · Cited by 545 · see citing works

Disruption of Dynein/Dynactin Inhibits Axonal Transport in Motor Neurons Causing Late-Onset Progressive Degeneration

LaMonte, B. H., Wallace, K. E., Holloway, B. A., Shelly, S. S., Ascaño, J., Tokito, M. K., Winkle, T. V., Howland, D., & Holzbaur, E. L. F. (2002) · Neuron · doi:10.1016/s0896-6273(02)00696-7 · Cited by 540 · see citing works

Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

Mead, R. J., Shan, N., Reiser, H. J., Marshall, F., & Shaw, P. J. (2023) · Nature Reviews Drug Discovery · doi:10.1038/s41573-022-00612-2 · Cited by 505 · see citing works

Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase

Estévez, A. G., Crow, J. P., Sampson, J. B., Reiter, C., Zhuang, Y., Richardson, G. J., Tarpey, M. M., Barbeito, L., & Beckman, J. S. (1999) · Science · doi:10.1126/science.286.5449.2498 · Cited by 596 · see citing works

Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model

Ralph, G. S., Radcliffe, P. A., Day, D. M., Carthy, J. M., Leroux, M. A., Lee, D. C. P., Wong, L., Bilsland, L. G., Greensmith, L., Kingsman, S. M., et al. (2005) · Nature Medicine · doi:10.1038/nm1205 · Cited by 506 · see citing works

Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria

Liu, J., Lillo, C., Jonsson, P. A., Velde, C. V., Ward, C. M., Miller, T. M., Subramaniam, J. R., Rothstein, J. D., Marklund, S. L., Andersen, P. M., et al. (2004) · Neuron · doi:10.1016/j.neuron.2004.06.016 · Cited by 506 · see citing works

Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

Turner, B. J., & Talbot, K. (2008) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2008.01.001 · Cited by 495 · see citing works

The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis

Bosch, L. V. D., Damme, P. V., Bogaert, E., & Robberecht, W. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.05.002 · Cited by 491 · see citing works

Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment

Pramatarova, A., Laganière, J., Roussel, J., Brisebois, K., & Rouleau, G. A. (2001) · The Journal of Neuroscience · doi:10.1523/jneurosci.21-10-03369.2001 · Cited by 482 · see citing works

Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target

Barber, S. C., Mead, R. J., & Shaw, P. J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2006.03.008 · Cited by 475 · see citing works

Glutamate transporters are oxidant-vulnerable: a molecular link between oxidative and excitotoxic neurodegeneration?

Trotti, D., Danbolt, N. C., & Volterra, A. (1998) · Trends in Pharmacological Sciences · doi:10.1016/s0165-6147(98)01230-9 · Cited by 469 · see citing works

Focal transplantation–based astrocyte replacement is neuroprotective in a model of motor neuron disease

Lepore, A. C., Rauck, B., Dejea, C., Pardo, A. C., Rao, M. S., Rothstein, J. D., & Maragakis, N. J. (2008) · Nature Neuroscience · doi:10.1038/nn.2210 · Cited by 441 · see citing works

Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease

Lobsiger, C. S., & Cleveland, D. W. (2007) · Nature Neuroscience · doi:10.1038/nn1988 · Cited by 438 · see citing works

Motoneuron Death Triggered by a Specific Pathway Downstream of Fas

Raoul, C., Estévez, Á. G., Nishimune, H., Cleveland, D. W., deLapeyrière, O., Henderson, C. E., Haase, G., & Pettmann, B. (2002) · Neuron · doi:10.1016/s0896-6273(02)00905-4 · Cited by 432 · see citing works

Histological Evidence of Protein Aggregation in Mutant SOD1 Transgenic Mice and in Amyotrophic Lateral Sclerosis Neural Tissues

Watanabe, M., Dykes‐Hoberg, M., Culotta, V., Price, D. L., Wong, P. C., & Rothstein, J. D. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0443 · Cited by 432 · see citing works

Astrocytes in human central nervous system diseases: a frontier for new therapies

Verkhratsky, A., Butt, A., Li, B., Illes, P., Zorec, R., Semyanov, A., Tang, Y., & Sofroniew, M. V. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01628-9 · Cited by 428 · see citing works

Endogenous regulatory T lymphocytes ameliorate amyotrophic lateral sclerosis in mice and correlate with disease progression in patients with amyotrophic lateral sclerosis

Beers, D. R., Henkel-Tigges, J., Zhao, W., Wang, J., Huang, A., Wen, S., Liao, B., & Appel, S. H. (2011) · Brain · doi:10.1093/brain/awr074 · Cited by 427 · see citing works

Astrocytes in Neurodegenerative Disease: Table 1.

Phatnani, H., & Maniatis, T. (2015) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a020628 · Cited by 420 · see citing works

Accumulation of SOD1 Mutants in Postnatal Motoneurons Does Not Cause Motoneuron Pathology or Motoneuron Disease

Lino, M. M., Schneider, C., & Caroni, P. (2002) · The Journal of Neuroscience · doi:10.1523/jneurosci.22-12-04825.2002 · Cited by 424 · see citing works

The molecular bases of Alzheimer’s disease and other neurodegenerative disorders

Maccioni, R. B., Muñoz, J. P., & Barbeito, L. (2001) · Archives of Medical Research · doi:10.1016/s0188-4409(01)00316-2 · Cited by 474 · see citing works

ALS Genetics: Gains, Losses, and Implications for Future Therapies

Kim, G., Gautier, O., Tassoni-Tsuchida, E., Ma, X. R., & Gitler, A. D. (2020) · Neuron · doi:10.1016/j.neuron.2020.08.022 · Cited by 370 · see citing works

Neuroinflammation and regulation of glial glutamate uptake in neurological disorders

Tilleux, S., & Hermans, E. (2007) · Journal of Neuroscience Research · doi:10.1002/jnr.21325 · Cited by 381 · see citing works

Decreased Metallation and Activity in Subsets of Mutant Superoxide Dismutases Associated with Familial Amyotrophic Lateral Sclerosis

Hayward, L. J., Rodriguez, J. A., Kim, J. W., Tiwari, A., Goto, J. J., Cabelli, D. E., Valentine, J. S., & Brown, R. H. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m112087200 · Cited by 377 · see citing works

Role of Neuroinflammation in Amyotrophic Lateral Sclerosis: Cellular Mechanisms and Therapeutic Implications

Liu, J., & Wang, F. (2017) · Frontiers in Immunology · doi:10.3389/fimmu.2017.01005 · Cited by 376 · see citing works

SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter

Trotti, D., Rolfs, A., Danbolt, N. C., Brown, R. H., & Hediger, M. A. (1999) · Nature Neuroscience · doi:10.1038/8091 · Cited by 373 · see citing works

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. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-02-00660.2000 · Cited by 372 · see citing works

Reactive Astrocytes As Therapeutic Targets for CNS Disorders

Hamby, M. E., & Sofroniew, M. V. (2010) · Neurotherapeutics · doi:10.1016/j.nurt.2010.07.003 · Cited by 367 · see citing works

Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase

Wong, P. C., Waggoner, D., Subramaniam, J. R., Tessarollo, L., Bartnikas, T. B., Culotta, V., Price, D. L., Rothstein, J. D., & Gitlin, J. D. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.040461197 · Cited by 365 · see citing works

Microglia in ALS: The Good, The Bad, and The Resting

Henkel, J. S., Beers, D. R., Zhao, W., & Appel, S. H. (2009) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-009-9171-5 · Cited by 353 · see citing works

Dysregulation of Glutathione Homeostasis in Neurodegenerative Diseases

Johnson, W. M., Wilson‐Delfosse, A. L., & Mieyal, J. J. (2012) · Nutrients · doi:10.3390/nu4101399 · Cited by 354 · see citing works

Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice

Ryu, H., Smith, K., Camelo, S. I., Carreras, I., Lee, J., Iglesias, A. H., Dangond, F., Cormier, K. A., Cudkowicz, M. E., H. Brown, R., et al. (2005) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2005.03077.x · Cited by 353 · see citing works

Human Cu/Zn Superoxide Dismutase (SOD1) Overexpression in Mice Causes Mitochondrial Vacuolization, Axonal Degeneration, and Premature Motoneuron Death and Accelerates Motoneuron Disease in Mice Expressing a Familial Amyotrophic Lateral Sclerosis Mutant SOD1

Jaarsma, D., Haasdijk, E. D., Grashorn, J., Hawkins, R. K., van Duijn, W., Verspaget, H. W., London, J., & Holstege, J. C. (2000) · Neurobiology of Disease · doi:10.1006/nbdi.2000.0299 · Cited by 351 · see citing works

Glutamate transporters: animal models to neurologic disease

Maragakis, N. J., & Rothstein, J. D. (2004) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.12.007 · Cited by 352 · see citing works

Mechanisms of Glutamate Transport

Vandenberg, R. J., & Ryan, R. M. (2013) · Physiological Reviews · doi:10.1152/physrev.00007.2013 · Cited by 349 · see citing works

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

Da Cruz, S., & Cleveland, D. W. (2011) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2011.05.029 · Cited by 351 · see citing works

Severe Life Stress and Oxidative Stress in the Brain: From Animal Models to Human Pathology

Schiavone, S., Jaquet, V., Trabace, L., & Krause, K. (2012) · Antioxidants and Redox Signaling · doi:10.1089/ars.2012.4720 · Cited by 350 · see citing works

Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

Pasinelli, P., Houseweart, M. K., Brown, R. H., & Cleveland, D. W. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.240305897 · Cited by 344 · see citing works

Glutamine and its relationship with intracellular redox status, oxidative stress and cell proliferation/death

Matés, J. M., Pérez‐Gómez, C., de Castro, I. N., Asenjo, M., & Márquez, J. (2002) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/s1357-2725(01)00143-1 · Cited by 340 · see citing works

Deregulation of Cdk5 in a Mouse Model of ALS

Nguyen, M. D., Larivière, R., & Julien, J. (2001) · Neuron · doi:10.1016/s0896-6273(01)00268-9 · Cited by 339 · see citing works

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Sharma, A., Lyashchenko, A. K., Lü, L., Nasrabady, S. E., Elmaleh, M., Mendelsohn, M. M., Nemes, A., Tapia, J. C., Mentis, G. Z., & Shneider, N. A. (2016) · Nature Communications · doi:10.1038/ncomms10465 · Cited by 339 · see citing works

The SLC1 high-affinity glutamate and neutral amino acid transporter family

Kanai, Y., Clémençon, B., Simonin, A., Leuenberger, M., Lochner, M., Weisstanner, M., & Hediger, M. A. (2013) · Molecular Aspects of Medicine · doi:10.1016/j.mam.2013.01.001 · Cited by 329 · see citing works

Role of Oxidative Stress and Antioxidants in Neurodegenerative Diseases

Rao, A. V., & Balachandran, B. (2002) · Nutritional Neuroscience · doi:10.1080/1028415021000033767 · Cited by 337 · see citing works

Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease

Vucic, S. (2006) · Brain · doi:10.1093/brain/awl172 · Cited by 334 · see citing works

Oxidation-induced Misfolding and Aggregation of Superoxide Dismutase and Its Implications for Amyotrophic Lateral Sclerosis

Rakhit, R., Cunningham, P., Fürtös, A., Dahan, S., Qi, X., Crow, J. P., Cashman, N. R., Kondejewski, L. H., & Chakrabartty, A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m207356200 · Cited by 333 · see citing works

A role for astrocytes in motor neuron loss in amyotrophic lateral sclerosis

Barbeito, L., Pehar, M., Cassina, P., Vargas, M. R., Peluffo, H., Viera, L., Estévez, Á. G., & Beckman, J. S. (2004) · Brain Research Reviews · doi:10.1016/j.brainresrev.2004.05.003 · Cited by 331 · see citing works

Apoptosis in Neural Development and Disease

Nijhawan, D., Honarpour, N., & Wang, X. (2000) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.23.1.73 · Cited by 326 · see citing works

Glutamate Transporters in Neurologic Disease

Maragakis, N. J., & Rothstein, J. D. (2001) · Archives of Neurology · doi:10.1001/archneur.58.3.365 · Cited by 324 · see citing works

Inflammation in ALS/FTD pathogenesis

McCauley, M. E., & Baloh, R. H. (2019) · Acta Neuropathologica · doi:10.1007/s00401-018-1933-9 · Cited by 316 · see citing works

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. (2001) · Journal of Neuroscience · doi:10.1523/jneurosci.21-23-09246.2001 · Cited by 318 · see citing works

Mechanism of Ceftriaxone Induction of Excitatory Amino Acid Transporter-2 Expression and Glutamate Uptake in Primary Human Astrocytes

Lee, S., Su, Z. Z., Emdad, L., Gupta, P., Sarkar, D., Borjabad, A., Volsky, D. J., & Fisher, P. B. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m707697200 · Cited by 318 · see citing works

Mutant superoxide dismutase 1-induced IL-1β accelerates ALS pathogenesis

Meissner, F., Molawi, K., & Zychlinsky, A. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1002396107 · Cited by 319 · see citing works

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

Saccon, R. A., Bunton-Stasyshyn, R. K. A., Fisher, E., & Fratta, P. (2013) · Brain · doi:10.1093/brain/awt097 · Cited by 318 · see citing works

Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS

Elam, J. S., Taylor, A. B., Strange, R. W., Antonyuk, S. V., Doucette, P. A., Rodriguez, J. A., Hasnain, S. S., Hayward, L. J., Valentine, J. S., Yeates, T. O., et al. (2003) · Nature Structural & Molecular Biology · doi:10.1038/nsb935 · Cited by 317 · see citing works

Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice

Yamanaka, K., Boillee, S., Roberts, E. A., Garcia, M. L., McAlonis-Downes, M., Mikse, O. R., Cleveland, D. W., & Goldstein, L. S. B. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0802556105 · Cited by 309 · see citing works

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

Israelson, A., Arbel, N., Cruz, S. D., Ilieva, H. S., Yamanaka, K., Shoshan‐Barmatz, V., & Cleveland, D. W. (2010) · Neuron · doi:10.1016/j.neuron.2010.07.019 · Cited by 313 · see citing works

Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model

Kikuchi, H., Almer, G., Yamashita, S., Guégan, C., Nagai, M., Xu, Z., Sosunov, A. A., McKhann, G. M., & Przedborski, S. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0509227103 · Cited by 313 · see citing works

A Rhodopsin Mutant Linked to Autosomal Dominant Retinitis Pigmentosa Is Prone to Aggregate and Interacts with the Ubiquitin Proteasome System

Illing, M., Rajan, R. S., Bence, N., & Kopito, R. R. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m204955200 · Cited by 311 · see citing works

GDNF Secreting Human Neural Progenitor Cells Protect Dying Motor Neurons, but Not Their Projection to Muscle, in a Rat Model of Familial ALS

Suzuki, M., McHugh, J., Tork, C. A., Shelley, B., Klein, S. M., Aebischer, P., & Svendsen, C. N. (2007) · PLoS ONE · doi:10.1371/journal.pone.0000689 · Cited by 311 · see citing works

Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature

Wang, J., Slunt, H. H., Gonzales, V., Fromholt, D., Coonfield, M. L., Copeland, N. G., Jenkins, N. A., & Borchelt, D. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg312 · Cited by 306 · see citing works

The UPRER: Sensor and Coordinator of Organismal Homeostasis.

Frakes, A. E., & Dillin, A. (2017) · Molecules and Cells · doi:10.1016/j.molcel.2017.05.031 · Cited by 295 · see citing works

Glutamate Transporters and the Excitotoxic Path to Motor Neuron Degeneration in Amyotrophic Lateral Sclerosis

Foran, E., & Trotti, D. (2009) · Antioxidants and Redox Signaling · doi:10.1089/ars.2009.2444 · Cited by 300 · see citing works

Neurofilaments and Orthograde Transport Are Reduced in Ventral Root Axons of Transgenic Mice that Express Human SOD1 with a G93A Mutation

Zhang, B., Tu, P., Abtahian, F., Trojanowski, J. Q., & Lee, V. M. ‐. (1997) · The Journal of Cell Biology · doi:10.1083/jcb.139.5.1307 · Cited by 299 · see citing works

Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice

Wang, L., Gutmann, D. H., & Roos, R. P. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq463 · Cited by 192 · see citing works

SOD1 Mutations Causing Familial Amyotrophic Lateral Sclerosis Induce Toxicity in Astrocytes: Evidence for Bystander Effects in a Continuum of Astrogliosis

Wallis, N., Lau, C. L., Farg, M. A., Atkin, J. D., Beart, P. M., & O’Shea, R. D. (2017) · Neurochemical Research · doi:10.1007/s11064-017-2385-7 · Cited by 13 · see citing works

Issue Cover (October 2015)

(2015) · Journal of Neurochemistry · doi:10.1111/jnc.12905

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. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-02-00660.2000 · Cited by 372 · see citing works

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

Swami, M. (2011) · Nature Medicine · doi:10.1038/nm.2484 · Cited by 33 · see citing works