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Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
Open accessAbstract
Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease that is characterized by the loss of motor neurons. Using mice carrying a deletable mutant gene, diminished mutant expression in astrocytes did not affect onset, but delayed microglial activation and sharply slowed later disease progression. These findings demonstrate that mutant astrocytes are viable targets for therapies for slowing the progression of non-cell autonomous killing of motor neurons in ALS.
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“Selectively deleting mutant SOD1 transgene expression in astrocytes, sharply slows the disease progression in the SOD1 mouse model of ALS [22].”
From Distinct repertoires of microRNAs present in mouse astrocytes compared to astrocyte-secreted exosomes (2017) · cites this paper as [22]
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“In pre-symptomatic mutant SOD1 transgenic mice, activated microglia initially display neuroprotective properties that slow disease progression;”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [436]
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“however, with time the continued and unabridged neuroinflammation only enhances neurodegeneration and accelerates disease progression [434–436].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [436]
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Citations
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InfluentialSelf-citationOligodendroglia metabolically support axons and contribute to neurodegeneration
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ResultInnate immune activation in neurodegenerative disease
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Astrocyte barriers to neurotoxic inflammation
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InfluentialSelf-citationAstrocyte reactivity and reactive astrogliosis: costs and benefits
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Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957
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Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
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“Further examples include (1) astrocyteselective expression of mutant SOD causes neuronal dysfunction and degeneration (Lobsiger and Cleveland 2007; Nagai et al. 2007; Yamanaka et al. 2008), (2) astrocyte-selective deletion of the endoribonuclease, Dicer, cases of severe ataxia, progressive cerebellar degeneration, sei…”
BackgroundMicroglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis
Frakes, A. E., Ferraiuolo, L., Haidet-Phillips, A. M., Schmelzer, L., Braun, L., Miranda, C. J., Ladner, K. J., Bevan, A. K., Foust, K. D., Godbout, J. P., et al. (2014) · Neuron · doi:10.1016/j.neuron.2014.01.013 · Cited by 638
“We observed robust RFP expression in GFAP+ and EAAT2+ cells (Figure S2D); RFP expression was absent in Iba-1+ microglia as well as in ChAT+ neurons in the spinal cord as previously reported (Figure S2C) (Yamanaka et al., 2008; Zhuo et al., 2001).”
InfluentialNeuroinflammation 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
Astrocyte–neuron metabolic relationships: for better and for worse
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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
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A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice
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Self-citationRethinking ALS: The FUS about TDP-43
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“…emerged as to how SOD1 mutations lead to selective premature death of motor neurons, except that damage within motor neurons expressing mutant SOD1 drives disease onset, whereas damage within their glial cell neighbors expressing mutant SOD1 accelerates disease progression (Yamanaka et al., 2008).”
BackgroundSelf-citationNishitoh, H., Kadowaki, H., Nagai, A., Maruyama, T., Yokota, T., Fukutomi, H., Noguchi, T., Matsuzawa, A., Takeda, K., & Ichijo, H. (2008) · Genes & Development · doi:10.1101/gad.1640108 · Cited by 530
“Recent studies have emphasized the importance of non-cell-autonomous mechanisms (Clement et al. 2003; Boillee et al. 2006; Yamanaka et al. 2008).”
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Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS
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“Future Cre-Lox crosses harnessing astrocyte, myelinating oligodendrocyte and Schwann cell, vascular endothelium or other neuronal promoters are highly anticipated (Yamanaka et al., 2008).”
Reactive Glia not only Associates with Plaques but also Parallels Tangles in Alzheimer’s Disease
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“The relevance of this finding has also been confirmed in vivo: specific deletion of the mutant SOD1 gene from astrocytes, within otherwise transgenic animals, substantially slows the progressive phase of the dis- ease (Yamanaka et al., 2008).”
BackgroundVargas, 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
“According to the current notion of non-cell autonomous toxicity in ALS (Clement et al., 2003; Boillée et al., 2006; Yamanaka et al., 2008), a modification that directly affects astrocytes will predict a shift in the disease duration by affecting progression.”
InfluentialBeers, D. R., Henkel-Tigges, J., Zhao, W., Wang, J., & Appel, S. H. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0807419105 · Cited by 485
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“In accordancewith our observations, recent data have also shown that EAAT2 expression in astrocytes is not responsible for the recovery of motor function in the loxSOD1/GFAP-Cre+ mouse model (Yamanaka et al., 2008).”
BackgroundMethodAstrocytes in Alzheimer's Disease
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“Even more importantly, selective silencing of the SOD1 mutant gene in astrocytes significantly slowed the progression of ALS in transgenic mice.(132) Late stages of ALS are characterized by significant astrogliosis and astroglial proliferation.”
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Astrocytes in human central nervous system diseases: a frontier for new therapies
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“560,561 Similarly, grafting astrocytes hosting SOD1 G93A mutant into the spinal cord of the healthy mice promoted motor neurones demise resulting in ALS symptoms.”
BackgroundThe Nrf2/ARE Pathway as a Potential Therapeutic Target in Neurodegenerative Disease
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“Furthermore, knocking down expression of the mutated allele in astrocytes leads to diminished microglial activation and overall disease progression (67).”
Osaki, T., Uzel, S. G. M., & Kamm, R. D. (2018) · Science Advances · doi:10.1126/sciadv.aat5847 · Cited by 429
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
“…vivo experiments in mouse models of ALS in which the mutant SOD1 transgene was deleted in a cell-specific manner revealed that reducing the levels of SOD1 expression in astrocytes was sufficient to delay disease onset and /or progression (Yamanaka et al. 2008; Ilieva et al. 2009; Wang et al. 2011).”
BackgroundMesenchymal stem cell transplantation in amyotrophic lateral sclerosis: A Phase I clinical trial
Mazzini, L., Ferrero, I., Luparello, V., Rustichelli, D., Gunetti, M., Mareschi, K., Testa, L., Stecco, A., Tarletti, R., Miglioretti, M., et al. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.08.007 · Cited by 396
“Despite the relative selectivity of motor neuron cell death, animal and tissue culture models of familial ALS suggest that non-neuronal cells contribute significantly to neuronal dysfunction and death (Boillée et al., 2006; Yamanaka et al., 2008).”
BackgroundMicroglial CB2 cannabinoid receptors are neuroprotective in Huntington's disease excitotoxicity
Palazuelos, J., Aguado, T., Pazos, M. R., Julien, B., Carrasco, C., Resel, E., Sagredo, O., Benito, C., Romero, J., Azcoitia, I., et al. (2009) · Brain · doi:10.1093/brain/awp239 · Cited by 382
“However, conflicting findings on the role of astroglial cells have also been reported in, for example, models of motor neuron degeneration (Lepore et al., 2008; Yamanaka et al., 2008).”
BackgroundThe Seeds of Neurodegeneration: Prion-like Spreading in ALS
Polymenidou, M., & Cleveland, D. W. (2011) · Cell · doi:10.1016/j.cell.2011.10.011 · Cited by 382
“…ALS It is now well established that a released or secreted toxic factor produced by astrocytes or microglia mediates the killing of motor neurons in ALS-like disease in mutant SOD1 mice, a mechanism that was shown to drive rapid disease progression (Boillee et al., 2006; Yamanaka et al., 2008).”
Self-citationReactive 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 381
“sulted in delayed microglial activation and slowed late disease progression resulting in increased lifespan.(70) No-”
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Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms
Bradford, J. W., Shin, J., Redd, M. A., Wang, C.-E., Li, X.-J., & Li, S. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0911503106 · Cited by 362
“This is consistent with the finding that the GFAP promoter drives the expression of transgenes in astrocytes in the spinal cord of adult mice (18).”
BackgroundResultInfluentialMicroglia 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
“Furthermore, Yamanaka et al. (2008a) dem- onstrated that the presence of WT non-neuronal cells substantially delayed motoneuron degeneration, again indicating that other cells, such as glial cells, contribute to disease.”
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“We next sought to determine whether mutant M337V astrocytes exert a non-cell-autonomous toxic effect on MNs, similar to that described for mutant SOD1 rodent astrocytes (1, 8, 9, 31, 32).”
BackgroundBlood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis
Winkler, E. A., Sengillo, J. D., Sullivan, J. S., Henkel, J. S., Appel, S. H., & Zlokovic, B. V. (2013) · Acta Neuropathologica · doi:10.1007/s00401-012-1039-8 · Cited by 354
“identified in transgenic ALS mouse models [5, 9, 12, 34, 65]”
Antisense Oligonucleotide Therapies for Neurodegenerative Diseases
Bennett, C. F., Krainer, A. R., & Cleveland, D. W. (2019) · Annual Review of Neuroscience · doi:10.1146/annurev-neuro-070918-050501 · Cited by 353
Self-citationUnderstanding 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
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“α-syn expression in oligodendrocytes causes neuronal degeneration [16], while astrocytes expressing mutant Cu/Zn superoxide dismutase (SOD1) drive disease progression [51].”
BackgroundMotor neuron vulnerability and resistance in amyotrophic lateral sclerosis
Nijssen, J., Comley, L. H., & Hedlund, E. (2017) · Acta Neuropathologica · doi:10.1007/s00401-017-1708-8 · Cited by 353
miRNA malfunction causes spinal motor neuron disease
Haramati, S., Chapnik, E., Sztainberg, Y., Eilam, R., Zwang, R., Gershoni, N., McGlinn, E., Heiser, P. W., Wills, A.-M. A., Wirguin, I., et al. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1006151107 · Cited by 340
Self-citationClinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis
D’Amico, E., Factor-Litvak, P. R., Santella, R. M., & Mitsumoto, H. (2013) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2013.06.029 · Cited by 328
Emerging mechanisms of molecular pathology in ALS
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“Thus, although the number of Olig / ::SOD1G37R chimeric mice produced for this study was relatively small because of the complexity of the experimental design, our data establish that onset of motor neuron degeneration is non-cell autonomous and that non-autonomous cell contributors to onset may be distinguishable fro…”
BackgroundInfluentialSelf-citationMisfolded 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 329
“Finally, it is well established that although motor neurons are the final targets in ALS, mutant damage within astrocytes and microglia contributes to driving rapid disease progression (Beers et al., 2006; Boillée et al., 2006a, 2006b; Clement et al., 2003; Yamanaka et al., 2008a, 2008b).”
BackgroundSelf-citationGenetics of motor neuron disorders: new insights into pathogenic mechanisms
Dion, P. A., Daoud, H., & Rouleau, G. A. (2009) · Nature Reviews Genetics · doi:10.1038/nrg2680 · Cited by 317
“Reports describing chimeric mice with mixtures of normal and mutant SOD1-expressing cells showed that toxicity in motor neurons requires their proximity to non-neuronal cells expressing mutant SOD1, in particular astrocyte”
BackgroundAdenosine Kinase: Exploitation for Therapeutic Gain
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“…share 1) astrogliosis as a histopathological hallmark (Schiffer et al., 1996; Blumcke et al., 1999; Renkawek et al., 1999; Ala et al., 2000; Yamanaka et al., 2008) and 2) cognitive impairment as comorbidity or key pathologic feature (Palop and Mucke, 2009; Aarsland and Kurz, 2010; Rusina…”
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Foran, E., & Trotti, D. (2009) · Antioxidants & Redox Signaling · doi:10.1089/ars.2009.2444 · Cited by 268
“In mutant SOD1 mice, several studies have shown decreased EAAT2 protein and downregulation of glutamate-transport activity in affected CNS areas (9, 15, 20, 23, 41, 151, 153, 155).”
Neuroglia in neurodegeneration
Heneka, M. T., Rodriguez, J. J., & Verkhratsky, A. (2009) · Brain Research Reviews · doi:10.1016/j.brainresrev.2009.11.004 · Cited by 316
Immune-mediated Mechanisms in the Pathoprogression of Amyotrophic Lateral Sclerosis
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“Reduction of mSOD1 expression in either microglia or astrocytes by a Cre/Lox approach delayed the progression of the disease and extended the lifespan of ALS mice (Yamanaka et al. 2008).”
Zhang, H., Yang, B., Mu, X., Ahmed, S. S., Su, Q., He, R., Wang, H., Mueller, C., Sena-Esteves, M., Brown, R., et al. (2011) · Molecular Therapy · doi:10.1038/mt.2011.98 · Cited by 291
The Nrf2–ARE cytoprotective pathway in astrocytes
Vargas, M. R., & Johnson, J. A. (2009) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399409001094 · Cited by 295
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
“Several studies support the importance of both microglia and astroglia in mediating motoneuron injury (Beers et al., 2006; Boillee et al., 2006; Nagai et al., 2007; Yamanaka et al., 2008b; Zhao et al., 2004).”
BackgroundResultAstrocytic adenosine receptor A2A and Gs-coupled signaling regulate memory
Orr, A. G., Hsiao, E. C., Wang, M. M., Ho, K., Kim, D. H., Wang, X., Guo, W., Kang, J., Yu, G.-Q., Adame, A., et al. (2015) · Nature Neuroscience · doi:10.1038/nn.3930 · Cited by 289
“72 for interaction effect, F (1, 20) = 14.”
Neuropathology of amyotrophic lateral sclerosis and its variants
Saberi, S., Stauffer, J. E., Schulte, D. J., & Ravits, J. (2015) · Neurologic Clinics · doi:10.1016/j.ncl.2015.07.012 · Cited by 282
Basso, M., Pozzi, S., Tortarolo, M., Fiordaliso, F., Bisighini, C., Pasetto, L., Spaltro, G., Lidonnici, D., Gensano, F., Battaglia, E., et al. (2013) · Journal of Biological Chemistry · doi:10.1074/jbc.m112.425066 · Cited by 295
“In vivo studies usingmutant-SOD1mousemodels have demonstrated that astrocytes have a role in the progression (13) and more recently also in the onset of ALS (14).”
Protective and Toxic Neuroinflammation in Amyotrophic Lateral Sclerosis
Hooten, K. G., Beers, D. R., Zhao, W., & Appel, S. H. (2015) · Neurotherapeutics · doi:10.1007/s13311-014-0329-3 · Cited by 294
“The addition of mSOD1-expressing astrocytes and microglial cells has been shown to accelerate disease progression, and wild-typemicroglia and astrocytes have been shown to slow disease [37, 39, 41, 42].”
BackgroundAstrocytes as a Source for Extracellular Matrix Molecules and Cytokines
Wiese, S., Karus, M., & Faissner, A. (2012) · Frontiers in Pharmacology · doi:10.3389/fphar.2012.00120 · Cited by 262
“Even more important, the selective silencing of the SOD1 mutant gene in astrocytes significantly slowed the progression of ALS in transgenic mice (Yamanaka et al., 2008).”
BackgroundEnergy metabolism in ALS: an underappreciated opportunity?
Vandoorne, T., Bock, K. D., & Bosch, L. V. D. (2018) · Acta Neuropathologica · doi:10.1007/s00401-018-1835-x · Cited by 260
“Rat mutant SOD1G93A astrocytes were previously shown to be toxic to wild-type motor neurons [72, 214].”
BackgroundMicroglia and neuroprotection: From in vitro studies to therapeutic applications
Polazzi, E., & Monti, B. (2010) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2010.06.009 · Cited by 269
Badawy, A. A., Abdelfattah, N. A. H., Salem, S. S., Awad, M. F., & Fouda, A. (2021) · Biology · doi:10.3390/biology10030233 · Cited by 256
“[75], who stated that toxicity may be attributed to the ability of NPs to pass through epithelial and endothelial cells via transcytosis.”
BackgroundGlial cells in amyotrophic lateral sclerosis
Philips, T., & Rothstein, J. D. (2014) · Experimental Neurology · doi:10.1016/j.expneurol.2014.05.015 · Cited by 266
“Similar to microglia, deletion of mSOD1 G37R from astrocytes delayed disease progression and extended survival (Yamanaka et al., 2008a).”
BackgroundResultNeuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes
Chiò, A., Pagani, M., Agosta, F., Calvo, A., Cistaro, A., & Filippi, M. (2014) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70167-x · Cited by 253
Neurological Diseases as Primary Gliopathies: A Reassessment of Neurocentrism
Verkhratsky, A., Sofroniew, M. V., Messing, A., deLanerolle, N. C., Rempe, D. A., Rodriguez, J. J., & Nedergaard, M. (2012) · ASN NEURO · doi:10.1042/an20120010 · Cited by 254
“Astrocytes selectively expressing hSOD1 demonstrated glutamate excitotoxicity, and inhibition of SOD1 expression selectively in astrocytes retarded the ALS progression in transgenic animals (Yamanaka et al., 2008).”
BackgroundAstrocyte IKKβ/NF-κB signaling is required for diet-induced obesity and hypothalamic inflammation
Douglass, J. D., Dorfman, M. D., Fasnacht, R., Shaffer, L. D., & Thaler, J. P. (2017) · Molecular Metabolism · doi:10.1016/j.molmet.2017.01.010 · Cited by 248
TDP-43 activates microglia through NF-κB and NLRP3 inflammasome
Zhao, W., Beers, D. R., Bell, S., Wang, J., Wen, S., Baloh, R. H., & Appel, S. H. (2015) · Experimental Neurology · doi:10.1016/j.expneurol.2015.07.019 · Cited by 241
Stratification of astrocytes in healthy and diseased brain
Verkhratsky, A., Zorec, R., & Parpura, V. (2017) · Brain Pathology · doi:10.1111/bpa.12537 · Cited by 236
Palamiuc, L., Schlagowski, A.-I., Ngo, S. T., Vernay, A., Dirrig‐Grosch, S., Henriques, A., Boutillier, A., Zoll, J., Echaniz‐Laguna, A., Loeffler, J., et al. (2015) · EMBO Molecular Medicine · doi:10.15252/emmm.201404433 · Cited by 253
“In recent years, several non-neuronal cells including microglia (Boillée et al, 2006), astrocytes (Yamanaka et al, 2008), and skeletal muscle (Wong & Martin, 2010) have been proposed to influence the course of the disease.”
Garwood, C. J., Ratcliffe, L. E., Simpson, J. E., Heath, P. R., Ince, P. G., & Wharton, S. B. (2017) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12338 · Cited by 233
Mitochondria‐associated membrane collapse is a common pathomechanism in SIGMAR1‐ and SOD1‐linked ALS
Watanabe, S., Ilieva, H. S., Tamada, H., Nomura, H., Komine, O., Endo, F., Jin, S., Mancías, P., Kiyama, H., & Yamanaka, K. (2016) · EMBO Molecular Medicine · doi:10.15252/emmm.201606403 · Cited by 231
“The change on disease progression suggests involvement of glial cells (Yamanaka et al, 2008a,b).”
BackgroundSelf-citationAstrocyte-immune cell interactions in physiology and pathology
Han, R. T., Kim, R. D., Molofsky, A. V., & Liddelow, S. A. (2021) · Immunity · doi:10.1016/j.immuni.2021.01.013 · Cited by 223
Membrane recruitment of endogenous LRRK2 precedes its potent regulation of autophagy
Schapansky, J., Nardozzi, J. D., Felizia, F., & LaVoie, M. J. (2014) · Human Molecular Genetics · doi:10.1093/hmg/ddu138 · Cited by 226
Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies
Vucic, S., Rothstein, J. D., & Kiernan, M. C. (2014) · Trends in Neurosciences · doi:10.1016/j.tins.2014.05.006 · Cited by 233
Treatment of a Mouse Model of ALS by In Vivo Base Editing
Lim, C. K. W., Gapinske, M., Brooks, A. K., Woods, W. S., Powell, J. E., C., M. A. Z., Winter, J., Pérez‐Piñera, P., & Gaj, T. (2020) · Molecular Therapy · doi:10.1016/j.ymthe.2020.01.005 · Cited by 229
“Astrocytes have been reported to be a determinant of disease progression in SOD1-linked ALS, as disrupting mutant SOD1 expression in these cells using Cre-mediated gene excision can sharply slow latestage disease progression in a model of the disorder.(9) We thus packaged the N- and C-terminal split-intein CBEs into A…”
MethodResultWinkler, E. A., Sengillo, J. D., Sagare, A. P., Zhao, Z., Ma, Q., Zuniga, E., Wang, Y., Zhong, Z., Sullivan, J. S., Griffin, J. H., et al. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1401595111 · Cited by 238
Self-citationAstrocytic dysfunction: Insights on the role in neurodegeneration
Rossi, D., & Volterra, A. (2009) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2009.07.012 · Cited by 225
Endo, F., Komine, O., Fujimori-Tonou, N., Katsuno, M., Jin, S., Watanabe, S., Sobue, G., Dezawa, M., Wyss-Coray, T., & Yamanaka, K. (2015) · Cell Reports · doi:10.1016/j.celrep.2015.03.053 · Cited by 207
“, 2009) or astrocytes (Wang et al., 2011; Yamanaka et al., 2008) significantly extends the survival time of ALS mice.”
BackgroundInfluentialSelf-citationPapadeas, S. T., Kraig, S. E., O'Banion, C., Lepore, A. C., & Maragakis, N. J. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1103141108 · Cited by 213
“This is in line with previous observations in LoxSOD1/GFAP-Cre mice, in which microgliosis was greatest in areas containing the highest concentration of mSOD1-expressing astrocytes (7), and may suggest cross-talk between mutant astrocytes and microglia.”
MethodResultSong, W., Song, Y., Kincaid, B., Bossy, B., & Bossy‐Wetzel, E. (2012) · Neurobiology of Disease · doi:10.1016/j.nbd.2012.07.004 · Cited by 220
Motor Neuron Susceptibility in ALS/FTD
Ragagnin, A., Shadfar, S., Vidal, M., Jamali, M. S., & Atkin, J. D. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.00532 · Cited by 232
The multi-dimensional roles of astrocytes in ALS
Yamanaka, K., & Komine, O. (2017) · Neuroscience Research · doi:10.1016/j.neures.2017.09.011 · Cited by 220
Self-citationMercury Involvement in Neuronal Damage and in Neurodegenerative Diseases
Cariccio, V. L., Samà, A., Bramanti, P., & Mazzon, E. (2018) · Biological Trace Element Research · doi:10.1007/s12011-018-1380-4 · Cited by 246
Human Stem Cell-Derived Spinal Cord Astrocytes with Defined Mature or Reactive Phenotypes
Roybon, L., Lamas, N. J., Garcia-Diaz, A., Yang, E. J., Sattler, R., Jackson-Lewis, V., Kim, Y. A., Kachel, C. A., Rothstein, J. D., Przedborski, S., et al. (2013) · Cell Reports · doi:10.1016/j.celrep.2013.06.021 · Cited by 208
“In vitro, astrocytes from mutant SOD1 mouse models of ALS release toxic factors for motor neurons (Di Giorgio et al., 2007; Nagai et al., 2007) while in vivo, reduction of SOD1 levels in GFAP+ (and therefore reactive) cells leads to an increase in survival of the mice (Yamanaka et al., 2008).”
BackgroundFoust, K. D., Salazar, D. L., Likhite, S. B., Ferraiuolo, L., Ditsworth, D., Ilieva, H. S., Meyer, K., Schmelzer, L., Braun, L., Cleveland, D. W., et al. (2013) · Molecular Therapy · doi:10.1038/mt.2013.211 · Cited by 211
Self-citationGoldman, S. A. (2016) · Cell stem cell · doi:10.1016/j.stem.2016.01.012 · Cited by 207
“…reports have highlighted the causal contribution of glial cells to the pathogenesis of ALS (Di Giorgio et al., 2007, 2008; Meyer et al., 2014; Yamanaka et al., 2008), which has led to a number of trials and trial plans focused on spinal glial addition, whether from transplanted astrocytes or…”
BackgroundBonafede, R., & Mariotti, R. (2017) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2017.00080 · Cited by 215
“…or the transplantation of healthy astroglial cells, slowed down the disease progression, attenuated motoneuron loss and increased the lifespan in SOD1 transgenic mice (Lepore et al., 2008; Yamanaka et al., 2008), indicating the involvement of astroglial cells in the neurodegeneration process.”
BackgroundFerraiuolo, L., Higginbottom, A., Heath, P. R., Barber, S. C., Greenald, D., Kirby, J., & Shaw, P. J. (2011) · Brain · doi:10.1093/brain/awr193 · Cited by 204
“In recent years, the use of mixed co-cultures and chimeric mice has highlighted the importance of non-neuronal cells in the motoneuron injury occurring in ALS (Gong et al., 2000; Pramatarova et al., 2001; Lino et al., 2002; Boillee et al., 2006; Nagai et al., 2007; Yamanaka et al., 2008).”
Background
Metadata
- Authors
- Yamanaka, K., Chun, S. J., Boillee, S., Fujimori-Tonou, N., Yamashita, H., Gutmann, D. H., Takahashi, R., Misawa, H., & Cleveland, D. W.
- Affiliations
- University of California San Diego (US)
- RIKEN Center for Brain Science (JP)
- Corresponding author
- Yamanaka, Koji
- Cleveland, Don W
- Venue
- Nature Neuroscience
- Published
- 2008-02-03
- Volume
- 11
- Issue
- 3
- Pages
- 251-253
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- PMC
- PMC3137510
- OpenAlex
- W2068147651
- Cited by
- 1,140
- References
- 15
- Citations by year
- 2026: 29 · 2025: 30 · 2024: 40 · 2023: 68 · 2022: 50 · 2021: 46 · 2020: 60 · 2019: 63 · 2018: 66 · 2017: 71 · 2016: 64 · 2015: 77 · 2014: 86 · 2013: 72 · 2012: 77
- FWCI
- 51.08
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of Neurological Disorders and Stroke (NS 27036, R37NS027036, R01NS027036)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- astrocytes, amyotrophic lateral sclerosis, superoxide dismutase 1, SOD1 mutations, non-cell autonomous neurodegeneration, motor neuron degeneration, microglial activation, disease progression, astrocyte-targeted therapy, mouse model
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Disease Models, Animal, Genes, Dominant, Glial Fibrillary Acidic Protein, Gliosis, Humans, Mice, Transgenic, Mutation, Nerve Degeneration, Nitric Oxide, Spinal Cord, Superoxide Dismutase, Genetic Therapy, Survival Rate, Cell Death, Microglia, Oxidative Stress, Disease Progression, Integrases, Mice, Nitric Oxide Synthase Type II
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