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ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions
Open accessAbstract
High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and G37R were previously shown to mediate disease in mice through an acquired toxic property. We report here that even low levels of another mutant, G85R, cause motor neuron disease characterized by an extremely rapid clinical progression, without changes in SOD1 activity. Initial indicators of disease are astrocytic inclusions that stain intensely with SOD1 antibodies and ubiquitin and SOD1-containing aggregates in motor neurons, features common with some cases of SOD1 mutant-mediated ALS. Astrocytic inclusions escalate markedly as disease progresses, concomitant with a decrease in the glial glutamate transporter (GLT-1). Thus, the G85R SOD1 mutant mediates direct damage to astrocytes, which may promote the nearly synchronous degeneration of motor neurons.
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“implying that astrocytes are both necessary and sufficient for neurodegeneration in this ALS model.”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [414]
In the citing paper
“Selective clearance of SOD1-positive inclusions from astrocytes is sufficient to slow disease progression [414]”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [414]
In the citing paper
“In transgenic mice overexpressing mutant SOD1 associated with ALS, astrocytes display more SOD1-positive inclusions than do neurons, and at an earlier stage [414].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [414]
Citations
Cites
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
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
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
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
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
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
Self-citationCellular defenses against damage from reactive oxygen species
Yu, B. (1994) · Physiological Reviews · doi:10.1152/physrev.1994.74.1.139 · Cited by 2,382
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
Self-citationAmyotrophic 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
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
Self-citation
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Items 901–1,000 of 1,000
Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
Moss, K. R., & Saxena, S. (2025) · Cells · doi:10.3390/cells14010047 · Cited by 17
Leyton-Jaimes, M. F., Ivert, P., Hoeber, J., Han, Y., Feiler, A., Zhou, C., Pankratova, S., Shoshan-Barmatz, V., Israelson, A., & Kozlova, E. (2020) · Scientific Reports · doi:10.1038/s41598-020-77578-x · Cited by 14
Insights into Human-Induced Pluripotent Stem Cell-Derived Astrocytes in Neurodegenerative Disorders
Kumar, M., Nguyen, T. P. N., Milanese, M., & Bonanno, G. (2022) · Biomolecules · doi:10.3390/biom12030344 · Cited by 20
Neither serotonin nor adenosine-dependent mechanisms preserve ventilatory capacity in ALS rats
Nichols, N. L., Johnson, R. A., Satriotomo, I., & Mitchell, G. S. (2014) · Respiratory Physiology & Neurobiology · doi:10.1016/j.resp.2014.03.005 · Cited by 17
Cognitive impairment in amyotrophic lateral sclerosis, clues from the SOD1 mouse
Spalloni, A., & Longone, P. (2015) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2015.11.006 · Cited by 19
Howells, J., Sangari, S., Matamala, J. M., Kiernan, M. C., Marchand‐Pauvert, V., & Burke, D. J. (2020) · Clinical Neurophysiology · doi:10.1016/j.clinph.2020.03.028 · Cited by 17
Killoy, K. M., Pehar, M., Harlan, B. A., & Vargas, M. R. (2021) · The FASEB Journal · doi:10.1096/fj.202000386rr · Cited by 17
Exploring the alternative: Fish, flies and worms as preclinical models for ALS
Braems, E., Tziortzouda, P., & Bosch, L. V. D. (2021) · Neuroscience Letters · doi:10.1016/j.neulet.2021.136041 · Cited by 20
Spinal cord and parkinsonism: Neuromorphological evidences in humans and experimental studies
Vivacqua, G., Casini, A., Vaccaro, R., Salvi, E. P., Pasquali, L., Fornai, F., Yu, S., & D’Este, L. (2011) · Journal of Chemical Neuroanatomy · doi:10.1016/j.jchemneu.2011.03.001 · Cited by 16
Glyoxal inactivates glutamate transporter‐1 in cultured rat astrocytes
Kawaguchi, M., Shibata, N., Horiuchi, S., & Kobayashi, M. (2005) · Neuropathology · doi:10.1111/j.1440-1789.2004.00579.x · Cited by 17
Fan, D., Sui, Y., Zhao, Z., Liu, R., & Cai, B. (2014) · Neural Regeneration Research · doi:10.4103/1673-5374.143421 · Cited by 17
Sumi, H., Nagano, S., Fujimura, H., Kato, S., & Sakoda, S. (2006) · Acta Neuropathologica · doi:10.1007/s00401-006-0056-x · Cited by 17
Tanase, K., Teng, Q., Krishnaney, A. A., Liu, J. K., Garrity‐Moses, M. E., & Boulis, N. M. (2004) · Journal of Neurosurgery Spine · doi:10.3171/spi.2004.1.1.0128 · Cited by 17
Ueda, T., Ito, T., Inden, M., Kurita, H., Yamamoto, A., & Hozumi, I. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2022.805379 · Cited by 16
Cortical Hyperexcitability in the Driver’s Seat in ALS
Gunes, Z. I., Kan, V. W. Y., Jiang, S., Logunov, E., Ye, X., & Liebscher, S. (2022) · Clinical and Translational Neuroscience · doi:10.3390/ctn6010005 · Cited by 18
Protein kinetics of superoxide dismutase‐1 in familial and sporadic amyotrophic lateral sclerosis
Ly, C. V., Ireland, M. D., Self, W. K., Bollinger, J., Jockel, J., Herzog, H., Allred, P., Miller, L., Doyle, M., Anez-Bruzual, I., et al. (2023) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.51784 · Cited by 15
Singh, S. B., Rajput, S. S., Sharma, A., Kataria, S., Dutta, P., Ananthanarayanan, V., Nandi, A., Patil, S., Majumdar, A., & Subramanyam, D. (2024) · eLife · doi:10.7554/elife.98363 · Cited by 18
Models of Amyotrophic Lateral Sclerosis
Jackson, M. T., Ganel, R., & Rothstein, J. D. (2002) · Current Protocols in Neuroscience · doi:10.1002/0471142301.ns0913s20 · Cited by 16
Self-citationGlutamate pathway implication in amyotrophic lateral sclerosis: what is the signal in the noise?
Ré, D. B., Verche, V. L., Ikiz, B., Jacquier, A., & Przedborski, S. (2010) · Journal of Receptor Ligand and Channel Research · doi:10.2147/jrlcr.s6504 · Cited by 16
Broering, T. J., Wang, H., Boatright, N. K., Wang, Y., Baptista, K., Shayan, G., Garrity, K. A., Kayatekin, C., Bosco, D. A., Matthews, C. R., et al. (2013) · PLoS ONE · doi:10.1371/journal.pone.0061210 · Cited by 16
Rohde, G., Kermer, P., Reed, J. C., Bähr, M., & Weishaupt, J. H. (2008) · Neuroscience · doi:10.1016/j.neuroscience.2008.09.055 · Cited by 16
Banerjee, V., Oren, O., Ben‐Zeev, E., Taube, R., Engel, S., & Papo, N. (2017) · Journal of Biological Chemistry · doi:10.1074/jbc.m117.789610 · Cited by 16
High content analysis in amyotrophic lateral sclerosis
Rinaldi, F., Motti, D., Ferraiuolo, L., & Kaspar, B. K. (2016) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2016.12.001 · Cited by 17
Amyotrophic Lateral Sclerosis: From Disease Mechanisms to Therapies
Bruijn, L. I. (2002) · BioTechniques · doi:10.2144/02325dd01 · Cited by 16
Self-citationAlzheimer's disease, amyotrophic lateral sclerosis, and transgenic mice
Bajaj, N. P. S., Irving, N. G., Leigh, P. N., & MILLER, C. C. J. (1998) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.64.6.711 · Cited by 16
Kennedy’s disease: a triplet repeat disorder or a motor neuron disease?
Gallo, J. (2001) · Brain Research Bulletin · doi:10.1016/s0361-9230(01)00575-5 · Cited by 16
Luan, T., Li, Q., Huang, Z., Feng, Y., Xu, D., Zhou, Y., Hu, Y., & Wang, T. (2024) · Neuroscience Bulletin · doi:10.1007/s12264-024-01267-2 · Cited by 15
Drosophilaas a model to study the role of glia in neurodegeneration
Lee, Y.-M., & Sun, Y. (2015) · Journal of Neurogenetics · doi:10.3109/01677063.2015.1076816 · Cited by 15
Rojas, P., Ramirez, A. I., Cadena, M., Fernández‐Albarral, J. A., Salobrar‐García, E., López‐Cuenca, I., Santos‐García, I., de Lago, E., Urcelay-Segura, J. L., Ramirez, J. M., et al. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22041663 · Cited by 17
Jensen, B. K., McAvoy, K. J., Heinsinger, N. M., Lepore, A. C., Ilieva, H. S., Haeusler, A. R., Trotti, D., & Pasinelli, P. (2022) · Glia · doi:10.1002/glia.24183 · Cited by 14
Wong, P. C., Chiang, P., Martin, L. J., Koliatsos, V. E., & Price, D. L. (2012) · Elsevier eBooks · doi:10.1016/b978-0-12-374947-5.00045-6 · Cited by 15
Self-citationFilipchuk, A., Pambo-Pambo, A. B., Gaudel, F., Liabeuf, S., Brocard, C., Guéritaud, J. P., & Durand, J. (2021) · Neuroscience · doi:10.1016/j.neuroscience.2021.01.039 · Cited by 16
Kunishige, M., Hill, K. A., Riemer, A. M., Hagman, K. D. F., Halangoda, A., Heinmöller, E., Moore, S. R., Turner, D. M., & Sommer, S. S. (2001) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/s0027-5107(00)00120-2 · Cited by 15
Research and perspectives in neurosciences
Reul, J., & Linthorst, A. C. E. (2000) · Cited by 15
A model for the incorporation of metal from the copper chaperone CCS into Cu,Zn superoxide dismutase
Falconi, M., Iovino, M., & Desideri, A. (1999) · Structure · doi:10.1016/s0969-2126(99)80117-8 · Cited by 15
Pathophysiology of Amyotrophic Lateral Sclerosis
Fabian, H., Clara, M., & Alvaro, G. (2013) · InTech eBooks · doi:10.5772/56562 · Cited by 17
Dong, L., Liu, L., Li, Y., Li, W., Zhou, L., & Qin, X. (2022) · Human Molecular Genetics · doi:10.1093/hmg/ddac008 · Cited by 14
Marini, C., Cossu, V., Kumar, M., Milanese, M., Cortese, K., Bruno, S., Bellese, G., Carta, S., Zerbo, R. A., Torazza, C., et al. (2021) · Antioxidants · doi:10.3390/antiox10091392 · Cited by 14
Glutamate signaling mediates C. elegans behavioral plasticity to pathogens
Yu, C., & Chang, H. C.-H. (2022) · iScience · doi:10.1016/j.isci.2022.103919 · Cited by 19
Alterations of striatal glutamate transmission in rotenone-treated mice: MRI/MRS in vivo studies
Moussa, C., Rusnak, M., Hailu, A., Sidhu, A., & Fricke, S. T. (2007) · Experimental Neurology · doi:10.1016/j.expneurol.2007.09.023 · Cited by 14
Dupuis, L., Müller, A., Meininger, V., & Loeffler, J. P. (2004) · Revue Neurologique · doi:10.1016/s0035-3787(04)70845-0 · Cited by 14
Human stem cells as a model of motoneuron development and diseases
Liu, Y., & Zhang, S. (2010) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2010.05537.x · Cited by 14
Wong, P. C., Cai, H., Borcheit, D. R., & Price, D. L. (2001) · Journal of Molecular Neuroscience · doi:10.1385/jmn:17:2:233 · Cited by 14
Self-citationJenkins, B. G., Chen, Y. I., Küestermann, E. H. G. K., Makris, N., Nguyen, T., Kraft, E., Brownell, A., Rosas, H. D., Kennedy, D. N., Rosen, B. R., et al. (1999) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1999.tb07828.x · Cited by 14
Vargas, M. R., Burton, N. C., Gan, L., Johnson, D. A., Schäfer, M., Werner, S., & Johnson, J. A. (2013) · PLoS ONE · doi:10.1371/annotation/28f68b10-e23d-4519-8d52-8cc94fe372b1 · Cited by 14
Promising animal models for amyotrophic lateral sclerosis drug discovery: a comprehensive update
Lescouzères, L., & Patten, S. A. (2024) · Expert Opinion on Drug Discovery · doi:10.1080/17460441.2024.2387791 · Cited by 16
Ayers, J. I., Xu, G. M., Dillon, K. D., Lu, Q., Chen, Z., Beckman, J., Moreno-Romero, A. K., Zamora, D. L., Galaleldeen, A., & Borchelt, D. (2021) · Acta Neuropathologica Communications · doi:10.1186/s40478-021-01191-w · Cited by 13
Self-citationKilloy, K. M., Harlan, B. A., Pehar, M., & Vargas, M. R. (2022) · The FASEB Journal · doi:10.1096/fj.202101275r · Cited by 12
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 14
The Serotonergic System and Amyotrophic Lateral Sclerosis: A Review of Current Evidence
Lu, Y., Cheng, Y., Zhu, Y., Cui, L., & Li, X. (2023) · Cellular and Molecular Neurobiology · doi:10.1007/s10571-023-01320-0 · Cited by 12
Pathological Sequelae Associated with Skeletal Muscle Atrophy and Histopathology in G93A*SOD1 Mice
Aishwarya, R., Abdullah, C. S., Remex, N. S., Nitu, S. S., Hartman, B., King, J., Bhuiyan, M. A. N., Rom, O., Miriyala, S., Panchatcharam, M., et al. (2023) · Muscles · doi:10.3390/muscles2010006 · Cited by 14
Ebselen analogues delay disease onset and its course in fALS by on-target SOD-1 engagement
Watanabe, S., Amporndanai, K., Awais, R., Latham, C., Awais, M., O’Neill, P. M., Yamanaka, K., & Hasnain, S. S. (2024) · Scientific Reports · doi:10.1038/s41598-024-62903-5 · Cited by 12
Co-Existence of GABA and Glu Transporters in the Central Nervous System
Bonanno, G., Raiteri, L., Paluzzi, S., Zappettini, S., Usai, C., & Raiteri, M. (2006) · Current Topics in Medicinal Chemistry · doi:10.2174/156802606777323746 · Cited by 12
Lehmann, M., Marklund, M., Bolender, A., Bidhendi, E. E., Zetterström, P., Andersen, P. M., Brännström, T., Marklund, S. L., Gilthorpe, J. D., & Nordström, U. (2020) · Acta Neuropathologica Communications · doi:10.1186/s40478-020-01032-2 · Cited by 12
Mitochondrial Alterations in Transgenic Mice With an H46R Mutant Cu/Zn Superoxide Dismutase Gene
Sasaki, S., Aoki, M., Nagai, M., Kobayashi, M., & Itoyama, Y. (2009) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e31819ba185 · Cited by 13
The protective effects of beta-lactam antibiotics in motor neuron disorders
Hedlund, E. (2011) · Experimental Neurology · doi:10.1016/j.expneurol.2011.06.002 · Cited by 12
Rafałowska, J., Fidziańska, A., Dziewulska, D., Gadamski, R., Ogonowska, W., & Grieb, P. (2006) · PubMed · Cited by 12
Turner, B. J., Lopes, E. C., & Cheema, S. S. (2004) · Journal of Cellular Biochemistry · doi:10.1002/jcb.10782 · Cited by 12
Benn, S. C., & Brown, R. H. (2004) · Nature Medicine · doi:10.1038/nm0404-345 · Cited by 12
Yamashita, S., Mita, S., Kato, S., Okado, H., Ohama, E., & Uchino, M. (2002) · Neuroscience Letters · doi:10.1016/s0304-3940(02)00454-8 · Cited by 12
Aberrant protein aggregation in amyotrophic lateral sclerosis
Wang, H., & Zeng, R. (2024) · Journal of Neurology · doi:10.1007/s00415-024-12485-z · Cited by 11
Ashford, B., Simpson, J. E., Dawson, C., Boche, D., Cooper‐Knock, J., Heath, P. R., Fillingham, D. J., Appleby‐Mallinder, C., Wei, W., Dunning, M., et al. (2025) · Brain Pathology · doi:10.1111/bpa.70019 · Cited by 13
Hedegaard, A., Lehnhoff, J., Moldovan, M., Grøndahl, L., Petersen, N., & Meehan, C. F. (2015) · Journal of Neurophysiology · doi:10.1152/jn.00745.2014 · Cited by 11
Mathew, B., Ruiz, P., Dutta, S., Entrekin, J. T., Zhang, S., Patel, K. D., Simmons, M. S., Augelli‐Szafran, C. E., Cowell, R. M., & Suto, M. J. (2020) · European Journal of Medicinal Chemistry · doi:10.1016/j.ejmech.2020.112952 · Cited by 11
Obál, I., Klausz, G., Mándi, Y., Deli, M. A., Siklós, L., & Engelhardt, J. I. (2016) · Journal of Neuroinflammation · doi:10.1186/s12974-016-0586-7 · Cited by 11
Animal Models for the Study of Human Neurodegenerative Diseases
Colpo, G. D., Ribeiro, F. M., Rocha, N. P., & Teixeira, A. L. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-809468-6.00042-5 · Cited by 12
Giusto, E., Codrich, M., de Leo, G., Francardo, V., Coradazzi, M., Parenti, R., Gulisano, M., Vicario, N., Gulino, R., & Leanza, G. (2019) · The Journal of Comparative Neurology · doi:10.1002/cne.24751 · Cited by 11
S1P2-Gα12Signaling Controls Astrocytic Glutamate Uptake and Mitochondrial Oxygen Consumption
Jonnalagadda, D., Kihara, Y., Rivera, R. R., & Chun, J. J. M. (2021) · eNeuro · doi:10.1523/eneuro.0040-21.2021 · Cited by 11
Reactive astrocytes, their roles in CNS injury, and repair mechanisms
Ridet, J., & Privat, A. (2000) · Advances in structural biology · doi:10.1016/s1064-6000(00)80008-3 · Cited by 11
Lee, J. C., Jin, Y., Jin, J., Kang, B. G., Nam, D., Joo, K. M., & Ik, C. (2010) · Neurological Research · doi:10.1179/016164110x12807570509899 · Cited by 11
DNA-Triggered Aggregation of Copper, Zinc Superoxide Dismutase in the Presence of Ascorbate
Yin, J., Hu, S., Jiang, W., Liu, L., Lan, S., Song, X., & Liu, C. (2010) · PLoS ONE · doi:10.1371/journal.pone.0012328 · Cited by 11
Age-related Changes in Tau Expression in Transgenic Mouse Model of Amyotrophic Lateral Sclerosis
Barańczyk‐Kuźma, A., Usarek, E., Kuźma‐Kozakiewicz, M., Kaźmierczak, B., Gajewska, B., Schwalenstöcker, B., & Ludolph, A. C. (2007) · Neurochemical Research · doi:10.1007/s11064-006-9242-4 · Cited by 11
Motor Neuron Disease: A Chemical Perspective
Wood, L. K., & Langford, S. J. (2014) · Journal of Medicinal Chemistry · doi:10.1021/jm5001584 · Cited by 11
Xu, G. M., Fromholt, S., Ayers, J. I., Brown, H. H., Siemienski, Z. B., Crosby, K. W., Mayer, C. A., Janus, C., & Borchelt, D. (2014) · Journal of Neurochemistry · doi:10.1111/jnc.13022 · Cited by 11
Self-citationGene-environment interplay in neurogenesis and neurodegeneration
Palomo, T., Archer, T., Beninger, R. J., & Kostrzewa, R. M. (2004) · Neurotoxicity Research · doi:10.1007/bf03033279 · Cited by 11
Sagot, Y., Vejsada, R., & Kato, A. C. (1997) · Trends in Pharmacological Sciences · doi:10.1016/s0165-6147(97)90659-3 · Cited by 11
The role of microglia in the prion-like transmission of protein aggregates in neurodegeneration
Öztürk, M. M., Emgård, J., García‐Revilla, J., Fernández‐Calle, R., Yang, Y., Deierborg, T., & Roos, T. T. (2025) · Brain Communications · doi:10.1093/braincomms/fcaf087 · Cited by 14
Keskin, I., Bidhendi, E. E., Marklund, M., Andersen, P. M., Brännström, T., Marklund, S. L., & Nordström, U. (2021) · Acta Neuropathologica Communications · doi:10.1186/s40478-021-01211-9 · Cited by 10
Emerging Roles of Ubiquitination in Biomolecular Condensates
Liang, P., Zhang, J., & Wang, B. (2023) · Cells · doi:10.3390/cells12182329 · Cited by 13
Liguori, F., Alberti, F., Amadio, S., Angelini, D. F., Pilesi, E., Vitale, G., Tesoriere, G., Borsellino, G., Vernı̀, F., & Volonté, C. (2024) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2024.167192 · Cited by 10
HariKrishnaReddy, D., Misra, S., Upadhyay, S., Modi, M., & Medhi, B. (2015) · Reviews in the Neurosciences · doi:10.1515/revneuro-2014-0057 · Cited by 10
Enge, T. G., Ecroyd, H. W., Jolley, D. F., Yerbury, J. J., Kalmár, B., & Dosseto, A. (2018) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2018.03.001 · Cited by 10
Tian, Y., Jin, S., Promes, V., Liu, X., & Zhang, Y. (2020) · Naunyn-Schmiedeberg s Archives of Pharmacology · doi:10.1007/s00210-020-02022-w · Cited by 9
Lepore, A. C. (2011) · Journal of Visualized Experiments · doi:10.3791/3069-v · Cited by 9
Specialized Neurotransmitter Transporters in Astrocytes
Yang, Y., & Rothstein, J. D. (2008) · Springer eBooks · doi:10.1007/978-0-387-79492-1_4 · Cited by 9
Self-citationBocci, T., Pecori, C., Giorli, E., Briscese, L., Tognazzi, S., Caleo, M., & Sartucci, F. (2011) · International Journal of Molecular Sciences · doi:10.3390/ijms12129203 · Cited by 10
Mera‐Adasme, R., Suomivuori, C., Fierro, A., Pesonen, J., & Sundholm, D. (2013) · JBIC Journal of Biological Inorganic Chemistry · doi:10.1007/s00775-013-1039-8 · Cited by 10
Genetic analysis of the cystatin C gene in familial and sporadic ALS patients
Watanabe, M., Jackson, M. T., Ikeda, M., Mizushima, K., Amari, M., Takatama, M., Hirai, S., Ikeda, Y., Shizuka‐Ikeda, M., & Okamoto, K. (2006) · Brain Research · doi:10.1016/j.brainres.2005.12.046 · Cited by 10
Data mining in a behavioral test detects early symptoms in a model of amyotrophic lateral sclerosis.
Kafkafi, N., Yekutieli, D., Yarowsky, P., & Elmer, G. I. (2008) · Behavioral Neuroscience · doi:10.1037/0735-7044.122.4.777 · Cited by 10
Rajan, T. S., Tripathi, P. P., Arya, U., Mishra, H. K., & Subramaniam, J. R. (2011) · Annals of Neurosciences · doi:10.5214/ans.0972.7531.1118205 · Cited by 10
Shibata, N., Oda, H., Hirano, A., Kato, Y., Kawaguchi, M., Canto, M. C. D., Uchida, K., Sawada, T., & Kobayashi, M. (2002) · Neuropathology · doi:10.1046/j.1440-1789.2002.00449.x · Cited by 10
Gluck, M. R., Thomas, R. G., & Sivak, M. A. (2000) · Journal of Neural Transmission · doi:10.1007/s007020070007 · Cited by 10
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Trotti, D. (2003) · Advances in experimental medicine and biology · doi:10.1007/978-1-4615-0123-7_8 · Cited by 10
Chapter 2 Pathology of Motor Neuron Disorders
Wharton, S. B., & Ince, P. G. (2003) · Blue books of practical neurology · doi:10.1016/s1877-3419(09)70103-5 · Cited by 10
Antiglutamate therapy of ALS — which is the next step?
Ludolph, A. C., Meyer, T., & Riepe, M. W. (1999) · Springer eBooks · doi:10.1007/978-3-7091-6369-6_8 · Cited by 10
Cytopathology of the motor neuron
Ince, P. G., & Wharton, S. B. (2004) · Handbook of clinical neurophysiology · doi:10.1016/s1567-4231(04)04007-9 · Cited by 9
Verkhratsky, A., & Butt, A. M. (2023) · Elsevier eBooks · doi:10.1016/b978-0-12-821565-4.00012-2 · Cited by 10
Oxidative stress sensitivity in ALS muscle cells
Dupuis, L. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.08.023 · Cited by 9
Elmquist (1998) · doi:10.1002/(sici)1096-9861(19980615)395:4
Broca's region revisited: Cytoarchitecture and intersubject variability
Amunts, K., Schleicher, A., B�rgel, U., Mohlberg, H., Uylings, H. B. M., & Zilles, K. (1999) · The Journal of Comparative Neurology · doi:10.1002/(sici)1096-9861(19990920)412:2
Metadata
- Authors
- 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.
- Affiliations
- University of California San Diego (US)
- Johns Hopkins University (US)
- National Cancer Institute (US)
- Corresponding author
- Cleveland, D.W.
- Venue
- Neuron
- Publisher
- Elsevier BV
- Published
- 1997-02
- Volume
- 18
- Issue
- 2
- Pages
- 327-338
- ISSN
- 0896-6273 (ISSN-L)
- OpenAlex
- W2012851706
- Cited by
- 1,341
- References
- 39
- Citations by year
- 2026: 10 · 2025: 22 · 2024: 23 · 2023: 30 · 2022: 41 · 2021: 40 · 2020: 36 · 2019: 35 · 2018: 36 · 2017: 40 · 2016: 47 · 2015: 44 · 2014: 52 · 2013: 52 · 2012: 64
- FWCI
- 37.57
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Neuron (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Funders
- Muscular Dystrophy Association
- National Institute on Aging (P50AG005146, AG 05146)
- National Institute of Neurological Disorders and Stroke (NS10580, NS 27036)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- amyotrophic lateral sclerosis, SOD1 G93A, astrocyte damage, motor neuron degeneration, disease progression, glutamate transporter GLT-1, ubiquitin, familial ALS
- MeSH terms
- Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Axons, Disease Models, Animal, Genes, Dominant, Humans, Mice, Transgenic, Microscopy, Electron, Nerve Degeneration, Neurons, Spinal Cord, Superoxide Dismutase, Ubiquitins, Point Mutation, ATP-Binding Cassette Transporters, Amino Acid Transport System X-AG, Mice
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