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

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

Apparent hydroxyl radical production by peroxynitrite: Implications for endothelial injury from nitric oxide and superoxide

Beckman, J. S., Beckman, T. W., Chen, J., Marshall, P. A., & Freeman, B. A. (1990) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.87.4.1620 · Cited by 7,029 · see citing works

A redox-based mechanism for the neuroprotective and neurodestructive effects of nitric oxide and related nitroso-compounds

Lipton, S. A., Choi, Y.-B., Pan, Z.-H., Lei, S. Z., Chen, H.-S. V., Sucher, N. J., Loscalzo, J., Singel, D. J., & Stamler, J. S. (1993) · Nature · doi:10.1038/364626a0 · Cited by 2,433 · see citing works

Agricultural and biological chemistry

日本農芸化学会 (1961) · Medical Entomology and Zoology · Cited by 1,713 · see citing works

Peroxynitrite-mediated tyrosine nitration catalyzed by superoxide dismutase

Ischiropoulos, H., Zhu, L., Chen, J., Tsai, M., Martin, J. C., Smith, C. D. L., & Beckman, J. S. (1992) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(92)90431-u · Cited by 1,495 · 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

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

An assay for superoxide dismutase activity in mammalian tissue homogenates

Spitz, D. R., & Oberley, L. W. (1989) · Analytical Biochemistry · doi:10.1016/0003-2697(89)90192-9 · Cited by 752 · 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

Nitric oxide mediates neuronal death after focal cerebral ischemia in the mouse

Nowicki, J.-P., Duval, D., Poignet, H., & Scatton, B. (1991) · European Journal of Pharmacology · doi:10.1016/0014-2999(91)90862-k · Cited by 503 · 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

Overproduction of human Cu/Zn‐superoxide dismutase in transfected cells: extenuation of paraquat‐mediated cytotoxicity and enhancement of lipid peroxidation.

Elroy‐Stein, O., Bernstein, Y., & Groner, Y. (1986) · The EMBO Journal · doi:10.1002/j.1460-2075.1986.tb04255.x · Cited by 316 · see citing works

Assay and Inhibitors of Spinach Superoxide Dismutase

Asada, K., Takahashi, M., & NAGATE, M. (1974) · Agricultural and Biological Chemistry · doi:10.1080/00021369.1974.10861178 · Cited by 248 · see citing works

Familial Amyotrophic Lateral Sclerosis

Hirano, A. (1967) · Archives of Neurology · doi:10.1001/archneur.1967.00470210008002 · Cited by 237 · see citing works

Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase

Avraham, K. B., Schickler, M., Sapoznikov, D., Yarom, R., & Groner, Y. (1988) · Cell · doi:10.1016/s0092-8674(88)91153-1 · Cited by 224 · see citing works

Induction of motor neuron sprouting in vivo by ciliary neurotrophic factor and basic fibroblast growth factor

Gurney, M., Yamamoto, H., & Kwon, Y. (1992) · Journal of Neuroscience · doi:10.1523/jneurosci.12-08-03241.1992 · Cited by 164 · see citing works

Human Cu/Zn superoxide dismutase cDNA: isolation of clones synthesising high levels of active or inactive enzyme from an expression library

Hallewell, R. A., Masiarz, F. R., Najarian, R. C., Purna, J. P., Quiroga, M., Randolph, A., Sánchez-Pescador, R., Scandella, C. J., Smith, B., Steimer, K. S., et al. (1985) · Nucleic Acids Research · doi:10.1093/nar/13.6.2017 · Cited by 147 · see citing works

Neuronal-specific expression of human copper-zinc superoxide dismutase gene in transgenic mice: animal model of gene dosage effects in Down's syndrome

Ceballos-Picot, I., Nicole, A., Briand, P., Grimber, G., Delacourte, A., Défossez, A., Javoy-Agid, F., Lafon, M., Blouin, J., & Sinet, P. (1991) · Brain Research · doi:10.1016/0006-8993(91)90084-9 · Cited by 103 · see citing works

AN INHERITED DISEASE SIMILAR TO AMYOTROPHIC LATERAL SCLEROSIS WITH A PATTERN OF POSTERIOR COLUMN INVOLVEMENT. AN INTERMEDIATE FORM?

Engel, W. K., Kurland, L. T., & KLATZO, I. (1959) · Brain · doi:10.1093/brain/82.2.203 · Cited by 94 · see citing works

Down's syndrome: morphological remodelling and increased complexity in the neuromuscular junction of transgenic CuZn-superoxide dismutase mice

Avraham, K. B., Sugarman, H., Rotshenker, S., & Groner, Y. (1991) · Journal of Neurocytology · doi:10.1007/bf01186993 · Cited by 80 · see citing works

A motor neuron‐specific epitope and the low‐affinity nerve growth factor receptor display reciprocal patterns of during development, axotomy, and regeneration

Chiu, A. Y., Chen, E. W., & Loera, S. (1993) · The Journal of Comparative Neurology · doi:10.1002/cne.903280303 · Cited by 41 · see citing works

Molecular genetics of familial amyotrophic lateral sclerosis.

Siddique, T. (1991) · PubMed · Cited by 25 · see citing works

Nitric Oxide and Peroxynitrite in Health and Disease

Pacher, P., Beckman, J. S., & Liaudet, L. (2007) · Physiological Reviews · doi:10.1152/physrev.00029.2006 · Cited by 6,390 · see citing works

Nitric oxide, superoxide, and peroxynitrite: the good, the bad, and ugly

Beckman, J. S., & Koppenol, W. H. (1996) · American Journal of Physiology-Cell Physiology · doi:10.1152/ajpcell.1996.271.5.c1424 · Cited by 5,513 · see citing works

A Unique Microglia Type Associated with Restricting Development of Alzheimer’s Disease

Keren-Shaul, H., Spinrad, A., Weiner, A., Matcovitch-Natan, O., Dvir-Szternfeld, R., Ulland, T. K., David, E., Baruch, K., Lara-Astaiso, D., Toth, B., et al. (2017) · Cell · doi:10.1016/j.cell.2017.05.018 · Cited by 5,493 · see citing works

Neurodegenerative diseases and oxidative stress

Barnham, K. J., Masters, C. L., & Bush, A. I. (2004) · Nature Reviews Drug Discovery · doi:10.1038/nrd1330 · Cited by 3,683 · see citing works

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630 · see citing works

Amyotrophic lateral sclerosis

Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., Burrell, J. R., & Zoing, M. C. (2011) · The Lancet · doi:10.1016/s0140-6736(10)61156-7 · Cited by 2,603 · see citing works

Pathological roles of MAPK signaling pathways in human diseases

Kim, E. K., & Choi, E.-J. (2010) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2009.12.009 · Cited by 2,472 · see citing works

Oxidative stress hypothesis in Alzheimer’s disease

Markesbery, W. R. (1997) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(96)00629-6 · Cited by 2,383 · see citing works

Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression

Zelko, I. N., Mariani, T. J., & Folz, R. J. (2002) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(02)00905-x · Cited by 2,212 · see citing works

Oxidative stress in neurodegeneration: Cause or consequence?

Andersen, J. K. (2004) · Nature Medicine · doi:10.1038/nrn1434 · Cited by 1,869 · see citing works

Microglia in neurodegeneration

Hickman, S., Izzy, S., Sen, P., Morsett, L., & El Khoury, J. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0242-x · Cited by 1,710 · 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

Apoptosis in neurodegenerative disorders

Mattson, M. P. (2000) · Nature Reviews Molecular Cell Biology · doi:10.1038/35040009 · Cited by 1,605 · see citing works

Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets

Gao, C., Jiang, J., Tan, Y., & Chen, S. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01588-0 · Cited by 1,616 · see citing works

Unfolding the role of protein misfolding in neurodegenerative diseases

Soto, C. (2003) · Nature Reviews Neuroscience · doi:10.1038/nrn1007 · Cited by 1,506 · see citing works

Innate immune activation in neurodegenerative disease

Heneka, M. T., Kummer, M. P., & Latz, E. (2014) · Nature Reviews Immunology · doi:10.1038/nri3705 · Cited by 1,437 · see citing works

Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling.

Lee, J., Giordano, S., & Zhang, J. (2012) · Biochemical Journal · doi:10.1042/bj20111451 · Cited by 1,438 · see citing works

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

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., et al. (1997) · Neuron · doi:10.1016/s0896-6273(00)80272-x · Cited by 1,341 · 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

Multi-target-Directed Ligands To Combat Neurodegenerative Diseases

Cavalli, A., Bolognesi, M. L., Minarini, A., Rosini, M., Tumiatti, V., Recanatini, M., & Melchiorre, C. (2008) · Journal of Medicinal Chemistry · doi:10.1021/jm7009364 · Cited by 1,152 · see citing works

SIRT1 deacetylase protects against neurodegeneration in models for Alzheimer's disease and amyotrophic lateral sclerosis

Kim, D., Nguyen, M. D., Dobbin, M. M., Fischer, A., Sananbenesi, F., Rodgers, J. T., Delalle, I., Baur, J. A., Sui, G., Armour, S. M., et al. (2007) · The EMBO Journal · doi:10.1038/sj.emboj.7601758 · Cited by 1,074 · 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

GDNF, NGF and BDNF as Therapeutic Options for Neurodegeneration

Allen, S. J., Watson, J. J., Shoemark, D. K., Barua, N. U., & Patel, N. K. (2013) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2013.01.004 · Cited by 820 · see citing works

Mitochondrial Dysfunction in Neurodegenerative Diseases

Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806 · 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

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

The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

Yang, Y., Hentati, A., Deng, H.-X., Dabbagh, O., Sasaki, T., Hirano, M., Hung, W.-Y., Ouahchi, K., Yan, J., Azim, A. C., et al. (2001) · Nature Genetics · doi:10.1038/ng1001-160 · Cited by 743 · see citing works

Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

Beers, D. R., Henkel, J. S., Xiao, Q., Zhao, W., Wang, J., Yen, A. A., Siklos, L., McKercher, S. R., & Appel, S. H. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0607423103 · Cited by 733 · see citing works

Autophagosomes initiate distally and mature during transport toward the cell soma in primary neurons

Maday, S., Wallace, K. E., & Holzbaur, E. L. F. (2012) · Journal of Cell Biology · doi:10.1083/jcb.201106120 · Cited by 681 · see citing works

A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model

Chiu, I. M., Morimoto, E. T. A., Goodarzi, H., Liao, J. T., O’Keeffe, S., Phatnani, H. P., Muratet, M., Carroll, M. C., Levy, S., Tavazoie, S., et al. (2013) · Cell Reports · doi:10.1016/j.celrep.2013.06.018 · Cited by 651 · see citing works

Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis

Klivenyi, P., Ferrante, R. J., Matthews, R. T., Bogdanov, M. B., Klein, A. M., Andreassen, O. A., Mueller, G., Wermer, M., Kaddurah-Daouk, R., & Beal, M. F. (1999) · Nature Medicine · doi:10.1038/6568 · Cited by 682 · see citing works

Amyloid-β/Fyn–Induced Synaptic, Network, and Cognitive Impairments Depend on Tau Levels in Multiple Mouse Models of Alzheimer's Disease

Roberson, E. D., Halabisky, B., Yoo, J. W., Yao, J., Chin, J., Yan, F., Wu, T., Hamto, P., Devidze, N., Yu, G.-Q., et al. (2011) · The Journal of Neuroscience · doi:10.1523/jneurosci.4152-10.2011 · Cited by 654 · 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

Microglia in Neuroinflammation and Neurodegeneration: From Understanding to Therapy

Muzio, L., Viotti, A., & Martino, G. (2021) · Frontiers in Neuroscience · doi:10.3389/fnins.2021.742065 · Cited by 581 · see citing works

Animal models of neurodegenerative diseases

Dawson, T. M., Golde, T. E., & Lagier-Tourenne, C. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0236-8 · Cited by 576 · see citing works

Superoxide dismutases—A review of the metal-associated mechanistic variations

Abreu, I. A., & Cabelli, D. E. (2010) · Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics · doi:10.1016/j.bbapap.2009.11.005 · Cited by 563 · see citing works

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1α in Huntington's disease neurodegeneration

Weydt, P., Pineda, V. V., Torrence, A. E., Libby, R. T., Satterfield, T. F., Lazarowski, E. R., Gilbert, M. L., Morton, G. J., Bammler, T. K., Strand, A. D., et al. (2006) · Cell Metabolism · doi:10.1016/j.cmet.2006.10.004 · Cited by 562 · 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

Lipopolysaccharide-induced neuroinflammation as a bridge to understand neurodegeneration

Batista, C. R. A., Gomes, G. F., Candelario-Jalil, E., Fiebich, B. L., & de Oliveira, A. C. P. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20092293 · Cited by 529 · see citing works

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

Dobrowolny, G., Aucello, M., Rizzuto, E., Beccafico, S., Mammucari, C., Bonconpagni, S., Belia, S., Wannenes, F., Nicoletti, C., Del Prete, Z., et al. (2008) · Cell Metabolism · doi:10.1016/j.cmet.2008.09.002 · Cited by 513 · 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

Design, power, and interpretation of studies in the standard murine model of ALS

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., et al. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960701856300 · Cited by 504 · see citing works

Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

Vargas, M. R., Johnson, D. A., Sirkis, D. W., Messing, A., & Johnson, J. A. (2008) · The Journal of Neuroscience · doi:10.1523/jneurosci.4099-08.2008 · Cited by 479 · 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

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration

Zhong, Z., Deane, R., Ali, Z., Parisi, M., Shapovalov, Y., O'Banion, M. K., Stojanovic, K., Sagare, A., Boillee, S., Cleveland, D. W., et al. (2008) · Nature Neuroscience · doi:10.1038/nn2073 · Cited by 476 · see citing works

Inflammatory processes in amyotrophic lateral sclerosis

McGeer, P. L., & McGeer, E. G. (2002) · Muscle & Nerve · doi:10.1002/mus.10191 · Cited by 470 · see citing works

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

Kiskinis, E., Sandoe, J., Williams, L. A., Boulting, G. L., Moccia, R., Wainger, B. J., Han, S., Peng, T., Thams, S., Mikkilineni, S., et al. (2014) · Cell stem cell · doi:10.1016/j.stem.2014.03.004 · Cited by 441 · see citing works

ALS clinical trials review: 20 years of failure. Are we any closer to registering a new treatment?

Petrov, D., Mansfield, C., Moussy, A., & Hermine, O. (2017) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2017.00068 · Cited by 451 · 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

Transient Opening of the Mitochondrial Permeability Transition Pore Induces Microdomain Calcium Transients in Astrocyte Processes

Agarwal, A., Wu, P.-H., Hughes, E. G., Fukaya, M., Tischfield, M. A., Langseth, A. J., Wirtz, D., & Bergles, D. E. (2017) · Neuron · doi:10.1016/j.neuron.2016.12.034 · Cited by 441 · 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

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

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

Urushitani, M., Sik, A., Sakurai, T., Nukina, N., Takahashi, R., & Julien, J.-P. (2006) · Nature Neuroscience · doi:10.1038/nn1603 · Cited by 419 · 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

A Review of the Neural Mechanisms of Action and Clinical Efficiency of Riluzole in Treating Amyotrophic Lateral Sclerosis: What have we Learned in the Last Decade?: Neural Mechanisms of Action and Clinical Efficiency of Riluzole in Treating ALS

Bellingham, M. C. (2011) · CNS Neuroscience & Therapeutics · doi:10.1111/j.1755-5949.2009.00116.x · Cited by 388 · see citing works

ATM deficiency and oxidative stress: a new dimension of defective response to DNA damage

Barzilai, A. (2002) · DNA Repair · doi:10.1016/s1568-7864(01)00007-6 · Cited by 359 · 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

Denervation-Induced Skeletal Muscle Atrophy Is Associated with Increased Mitochondrial Ros Production

Muller, F. L., Song, W., Jang, Y. C., Liu, Y., Sabia, M., Richardson, A., & Van Remmen, H. (2007) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.00767.2006 · Cited by 355 · 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

Ubiquitin-independent function of optineurin in autophagic clearance of protein aggregates

Korac, J., Schaeffer, V., Kovacevic, I., Clement, A. M., Jungblut, B., Behl, C., Terzic, J., & Dikic, I. (2013) · Journal of Cell Science · doi:10.1242/jcs.114926 · Cited by 336 · 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

Modeling ALS with iPSCs reveals that mutant SOD1 misregulates neurofilament balance in motor neurons

Chen, H., Qian, K., Du, Z., Cao, J., Petersen, A., Liu, H., Blackbourn, L. W., Huang, C.-L., Errigo, A., Yin, Y., et al. (2014) · Cell stem cell · doi:10.1016/j.stem.2014.02.004 · Cited by 318 · see citing works

Target intestinal microbiota to alleviate disease progression in amyotrophic lateral sclerosis

Zhang, Y.-G., Wu, S., Yi, J., Xia, Y., Jin, D., Zhou, J., & Sun, J. (2017) · Clinical Therapeutics · doi:10.1016/j.clinthera.2016.12.014 · Cited by 287 · see citing works

Mitochondrial Approaches for Neuroprotection

Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294 · see citing works

Extracellular mutant SOD1 induces microglial‐mediated motoneuron injury

Zhao, W., Beers, D. R., Henkel, J. S., Zhang, W., Urushitani, M., Julien, J., & Appel, S. H. (2010) · Glia · doi:10.1002/glia.20919 · Cited by 288 · see citing works

Antisense oligonucleotides extend survival and reverse decrement in muscle response in ALS models

McCampbell, A., Cole, T., Wegener, A. J., Tomassy, G. S., Setnicka, A., Farley, B. J., Schoch, K. M., Hoye, M. L., Shabsovich, M., Sun, L., et al. (2018) · Journal of Clinical Investigation · doi:10.1172/jci99081 · Cited by 279 · see citing works

Microglial gene signature reveals loss of homeostatic microglia associated with neurodegeneration of Alzheimer’s disease

Sobue, A., Komine, O., Hara, Y., Endo, F., Mizoguchi, H., Watanabe, S., Murayama, S., Saito, T., Saido, T. C., Sahara, N., et al. (2021) · Acta Neuropathologica Communications · doi:10.1186/s40478-020-01099-x · Cited by 253 · see citing works

Knockout of reactive astrocyte activating factors slows disease progression in an ALS mouse model

Guttenplan, K. A., Weigel, M. K., Adler, D. I., Couthouis, J., Liddelow, S. A., Gitler, A. D., & Barres, B. A. (2020) · Nature Communications · doi:10.1038/s41467-020-17514-9 · Cited by 278 · see citing works

Leaky intestine and impaired microbiome in an amyotrophic lateral sclerosis mouse model

Wu, S., Yi, J., Zhang, Y.-G., Zhou, J., & Sun, J. (2015) · Physiological Reports · doi:10.14814/phy2.12356 · Cited by 267 · see citing works

<i>TREM2</i>Variant p.R47H as a Risk Factor for Sporadic Amyotrophic Lateral Sclerosis

Cady, J., Koval, E. D., Benitez, B. A., Zaidman, C., Jockel-Balsarotti, J., Allred, P., Baloh, R. H., Ravits, J., Simpson, E., Appel, S. H., et al. (2014) · JAMA Neurology · doi:10.1001/jamaneurol.2013.6237 · Cited by 262 · see citing works

Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis

Wiedemann, F. R., Winkler, K., Kuznetsov, A. V., Bartels, C., Vielhaber, S., Feistner, H., & Kunz, W. S. (1998) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(98)00008-2 · Cited by 262 · see citing works

T cell-microglial dialogue in Parkinson's disease and amyotrophic lateral sclerosis: are we listening?

Appel, S. H., Beers, D. R., & Henkel, J. S. (2010) · Trends in Immunology · doi:10.1016/j.it.2009.09.003 · Cited by 256 · see citing works

The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins

Deora, V., Lee, J. D., Albornoz, E. A., McAlary, L., Jagaraj, C. J., Robertson, A. A. B., Atkin, J. D., Cooper, M. A., Schroder, K., Yerbury, J. J., et al. (2020) · Glia · doi:10.1002/glia.23728 · Cited by 245 · see citing works

Mitochondrial Medicine for Aging and Neurodegenerative Diseases

Reddy, P. H. (2008) · NeuroMolecular Medicine · doi:10.1007/s12017-008-8044-z · Cited by 230 · see citing works

Increased autophagy in transgenic mice with a G93A mutant SOD1 gene

Morimoto, N., Nagai, M., Ohta, Y., Miyazaki, K., Kurata, T., Morimoto, M., Murakami, T., Takehisa, Y., Ikeda, Y., Kamiya, T., et al. (2007) · Brain Research · doi:10.1016/j.brainres.2007.06.045 · Cited by 223 · see citing works

The bone morphogenetic protein axis is a positive regulator of skeletal muscle mass

Winbanks, C. E., Chen, J. L., Qian, H., Liu, Y., Bernardo, B. C., Beyer, C., Watt, K. I., Thomson, R. E., Connor, T., Turner, B. J., et al. (2013) · Journal of Cell Biology · doi:10.1083/jcb.201211134 · Cited by 225 · see citing works

Lysosomal cell death mechanisms in aging

Gómez-Sintes, R., Ledesma, M. D., & Boya, P. (2016) · Ageing Research Reviews · doi:10.1016/j.arr.2016.02.009 · Cited by 216 · see citing works

Neurodegeneration in amyotrophic lateral sclerosis: the role of oxidative stress and altered homeostasis of metals

Carrı̀, M. T., Ferri, A., Cozzolino, M., Calabrese, L., & Rotilio, G. (2003) · Brain Research Bulletin · doi:10.1016/s0361-9230(03)00179-5 · Cited by 209 · see citing works

Misregulated RNA processing in amyotrophic lateral sclerosis

Polymenidou, M., Lagier-Tourenne, C., Hutt, K. R., Bennett, C. F., Cleveland, D. W., & Yeo, G. W. (2012) · Brain Research · doi:10.1016/j.brainres.2012.02.059 · Cited by 169 · see citing works

In vivo genome editing improves motor function and extends survival in a mouse model of ALS

Gaj, T., Ojala, D. S., Ekman, F. K., Byrne, L. C., Limsirichai, P., & Schaffer, D. V. (2017) · Science Advances · doi:10.1126/sciadv.aar3952 · Cited by 185 · see citing works

The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS)

Redler, R. L., & Dokholyan, N. V. (2012) · Progress in Molecular Biology and Translational Science · doi:10.1016/b978-0-12-385883-2.00002-3 · Cited by 172 · see citing works

Aggregation of Copper–Zinc Superoxide Dismutase in Familial and Sporadic ALS

Chattopadhyay, M., & Valentine, J. S. (2009) · Antioxidants &amp; Redox Signaling · doi:10.1089/ars.2009.2536 · Cited by 163 · see citing works

Cell‐permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis

Petri, S., Kiaei, M., Damiano, M., Hiller, A., Wille, E., Manfredi, G., Calingasan, N. Y., Szeto, H. H., & Beal, M. F. (2006) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2006.04018.x · Cited by 166 · see citing works

Peripheral monocytes are functionally altered and invade the CNS in ALS patients

Zondler, L., Müller, K., Khalaji, S., Bliederhäuser, C., Ruf, W. P., Grozdanov, V., Thiemann, M., Fundel-Clemes, K., Freischmidt, A., Holzmann, K., et al. (2016) · Acta Neuropathologica · doi:10.1007/s00401-016-1548-y · Cited by 154 · see citing works

Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Implications

Cozzolino, M., Ferri, A., & Teresa Carrì, M. (2008) · Antioxidants &amp; Redox Signaling · doi:10.1089/ars.2007.1760 · Cited by 156 · see citing works

RNA oxidation in Alzheimer disease and related neurodegenerative disorders

Nunomura, A., Hofer, T., Moreira, P. I., Castellani, R. J., Smith, M. A., & Perry, G. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0508-1 · Cited by 148 · see citing works

SOD1, an unexpected novel target for cancer therapy.

Papa, L., Manfredi, G., & Germain, D. (2014) · Genes &amp; Cancer · doi:10.18632/genesandcancer.4 · Cited by 147 · see citing works

Involvement of CHOP, an ER-stress apoptotic mediator, in both human sporadic ALS and ALS model mice

Ito, Y., Yamada, M., Tanaka, H., Aida, K., Tsuruma, K., Shimazawa, M., Hozumi, I., Inuzuka, T., Takahashi, H., & Hara, H. (2009) · Neurobiology of Disease · doi:10.1016/j.nbd.2009.08.013 · Cited by 141 · see citing works

Effects of high-intensity endurance exercise training in the G93A mouse model of amyotrophic lateral sclerosis

Mahoney, D. J., Rodriguez, C., Devries, M., Yasuda, N., & Tarnopolsky, M. A. (2004) · Muscle &amp; Nerve · doi:10.1002/mus.20004 · Cited by 140 · see citing works

Neuroprotective efficacy of aminopropyl carbazoles in a mouse model of amyotrophic lateral sclerosis

Tesla, R., Wolf, H. P., Xu, P., Drawbridge, J., Estill, S. J., Huntington, P., McDaniel, L., Knobbe, W., Burket, A., Tran, S., et al. (2012) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1213960109 · Cited by 129 · see citing works

Quantification of oxidized RNAs in Alzheimer's disease

Shan, X., & Lin, C.-L. G. (2006) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2005.03.022 · Cited by 125 · see citing works

Modifying macrophages at the periphery has the capacity to change microglial reactivity and to extend ALS survival

Chiot, A., Zaïdi, S., Iltis, C., Ribon, M., Berriat, F., Schiaffino, L., Jolly, A., de la Grange, P., Mallat, M., Bohl, D., et al. (2020) · Nature Neuroscience · doi:10.1038/s41593-020-00718-z · Cited by 122 · see citing works

Axonal degeneration as a therapeutic target in the CNS

Lingor, P., Koch, J. C., Tönges, L., & Bähr, M. (2012) · Cell and Tissue Research · doi:10.1007/s00441-012-1362-3 · Cited by 119 · see citing works

Activation of interferon signaling pathways in spinal cord astrocytes from an ALS mouse model

Wang, R., Yang, B., & Zhang, D. (2011) · Glia · doi:10.1002/glia.21167 · Cited by 115 · see citing works

Cellular senescence in white matter microglia is induced during ageing in mice and exacerbates the neuroinflammatory phenotype

Matsudaira, T., Nakano, S., Konishi, Y., Kawamoto, S., Uemura, K., Kondo, T., Sakurai, K., Ozawa, T., Hikida, T., Komine, O., et al. (2023) · Communications Biology · doi:10.1038/s42003-023-05027-2 · Cited by 98 · see citing works

Ablation of P2X7 receptor exacerbates gliosis and motoneuron death in the SOD1-G93A mouse model of amyotrophic lateral sclerosis

Apolloni, S., Amadio, S., Montilli, C., Volonte, C., & D'Ambrosi, N. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt259 · Cited by 109 · see citing works

Antisense drugs make sense for neurological diseases

Bennett, C. F., Kordasiewicz, H. B., & Cleveland, D. W. (2021) · Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev-pharmtox-010919-023738 · Cited by 108 · see citing works

The role of the immune system in neurodegenerative disorders: Adaptive or maladaptive?

Doty, K. R., Guillot-Sestier, M.-V., & Town, T. (2015) · Brain Research · doi:10.1016/j.brainres.2014.09.008 · Cited by 104 · see citing works

Progress in the relationship between mutant SOD1 and amyotrophic lateral sclerosis.

Li-yin, C. (2009) · Zhongguo shiyong neike zazhi

Cu,Zn-superoxide Dismutase Associated with Amyotrophic Lateral Sclerosis

Qiu, C. (2008) · Zhongguo shengwu huaxue yu fenzi shengwu xuebao

Perspective on SOD1 mediated toxicity in Amyotrophic Lateral Sclerosis

Sangwan, S., & Eisenberg, D. S. (2016) · Postępy Biochemii · doi:10.18388/pb.2016_37 · Cited by 31 · see citing works

Expression of the copper‐zinc superoxide dismutase gene in amyotrophic lateral sclerosis

Nishiyama, K., Murayama, S., Kwak, S., et al. (1997) · Annals of Neurology · doi:10.1002/ana.410410420 · Cited by 6 · 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