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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]
In the citing paper
“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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Cites
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BackgroundRole and Therapeutic Potential of Astrocytes in Amyotrophic Lateral Sclerosis
Pehar, M., Harlan, B. A., Killoy, K. M., & Vargas, M. R. (2018) · Current Pharmaceutical Design · doi:10.2174/1381612823666170622095802 · Cited by 84
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“The ALS model of ablating mutant SOD1 expression selectively in astrocytes doesn’t affect onset, but sharply slows later disease progression (Yamanaka et al., 2008).”
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CSF glial markers correlate with survival in amyotrophic lateral sclerosis
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Exploring the genetics and non-cell autonomous mechanisms underlying ALS/FTLD
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Glial Cells—The Strategic Targets in Amyotrophic Lateral Sclerosis Treatment
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“In the mSOD1 model, removing of the mSOD1 from astrocytes postponed the activation of microglia and, thus, extended the survival [101,236].”
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“Published by Elsevier Ltd. on behalf of IBRO.”
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“Activatedmicroglia and astrocytes are observed in the spinal cord of symptomatic ALS model mice and contribute to disease progression (Boillee et al., 2006; Frakes et al., 2014; Haidet-Phillips et al., 2011; Hovden et al., 2013; Yamanaka et al., 2008a,b).”
BackgroundAutophagy in Astrocytes and its Implications in Neurodegeneration
Sung, K., & Jiménez-Sánchez, M. (2020) · Journal of Molecular Biology · doi:10.1016/j.jmb.2019.12.041 · Cited by 78
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“Such a hypothesis is related to the non-cell autonomous toxicity exerted by both deteriorated astrocytes and microglial cells on neurons, which is well-known to contribute to ALS progression (Yamanaka et al., 2008; Ilieva et al., 2009; Haidet-Phillips et al., 2011; Lee et al., 2016), and to the way by which the activa…”
BackgroundRising Stars: Astrocytes as a Therapeutic Target for ALS Disease
Izrael, M., Slutsky, S. G., & Revel, M. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00824 · Cited by 73
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Neuroglial Roots of Neurodegenerative Diseases?
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“The role of astroglia in ALS progression is further supported by recent observation that silencing of ALS-related mutant SOD1 gene in astrocytes delayed the appearance of clinical symptoms in transgenic mouse model [83].”
BackgroundGlial Calcium Signalling in Alzheimer’s Disease
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“Specific expression of the mutant human superoxide dismutase (hSOD1) gene (associated with familiar form of ALS) in astrocytes led to an increased vulnerability to glutamate, to activation of microglia and neurotoxicity; whereas silencing of this gene specifically in astrocytes delayed ALS progression (Yamanaka et al.…”
BackgroundMicroglial ferroptotic stress causes non-cell autonomous neuronal death
Liddell, J. R., Hilton, J. B. W., Kysenius, K., Nikseresht, S., McInnes, L. E., Hare, D. J., Paul, B. T., Trist, B. G., Double, K. L., Mercer, S. W., et al. (2024) · Molecular Neurodegeneration · doi:10.1186/s13024-023-00691-8 · Cited by 77
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“A growing body of evidence indicates that non-neuronal cells contribute to the disease process in animal [4,5,6,7,8] and cellular [4,9,10] models overexpressing mutant SOD1.”
BackgroundLobsiger, C. S., Boillée, S., Pozniak, C. D., Khan, A. M., McAlonis‐Downes, M., Lewcock, J. W., & Cleveland, D. W. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1318309110 · Cited by 72
“In addition, insights from mutant SOD1 mice have demonstrated that toxicity acts in a non– cell-autonomous manner, involving mutant-dependent damage within motor neurons to drive disease onset (6) and within neighboring astrocytes and microglia to drive more rapid disease progression (6, 7).”
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“Further confirmation was provided by conditional deletion experiments, demonstrating that diminished mutant SOD1 levels within either microglia or astrocytes significantly modify disease progression (14,15).”
BackgroundSanberg, P. R., Eve, D. J., Willing, A. E., Garbuzova-Davis, S. N., Tan, J., Sanberg, C. D., Allickson, J. G., Cruz, L. E. G., & Borlongan, C. V. (2010) · Cell Transplantation · doi:10.3727/096368910x532855 · Cited by 72
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Stem Cell Transplantation for Motor Neuron Disease: Current Approaches and Future Perspectives
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“Further studies from the same group then revealed that genetic deletion of mutant SOD1 from either microglia or astrocytes had little effect on disease onset, but dramatically increased survival of animals after disease onset suggesting a strong modifying ability of these cells on disease progression [67, 68].”
BackgroundPurinergic contribution to amyotrophic lateral sclerosis
Volonté, C., Apolloni, S., Parisi, C., & Amadio, S. (2015) · Neuropharmacology · doi:10.1016/j.neuropharm.2015.10.026 · Cited by 73
“…mice (Clement et al., 2003), highlighting the main role played particularly by microglia and astrocytes (Boillee et al., 2006; Brites and Vaz, 2014; Yamanaka et al., 2008), but ignoring the crucial role of the other type of glia present in the nervous system, the oligodendrocytes and Schwann…”
BackgroundHenriques, A., Croixmarie, V., Bouscary, A., Mosbach, A., Keime, C., Boursier-Neyret, C., Walter, B., Spedding, M., & Loeffler, J. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2017.00433 · Cited by 70
“Activation of glial cells has been linked to degeneration of motor neurons (Yamanaka et al., 2008; Endo et al., 2015; Cooper-knock et al., 2017) or to defects in the integrity of peripheral motor axons (Nardo et al.”
BackgroundLee, S., Kim, S., Kang, H., Lim, H. R., Kwon, Y., Jo, M., Jeon, Y.-M., Kim, K., Kim, K., Ha, C. M., et al. (2020) · Journal of Neuroinflammation · doi:10.1186/s12974-020-01963-6 · Cited by 73
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“Dysregulation of copper trafficking pathway occurred primarily within motor neurons and copper chaperones were localized in astrocytes as well as microglia in symptomatic stages Recent studies on the variable expression of mutant SOD1 in motor neurons and/or glial cells have demonstrated that these cell types each con…”
BackgroundInfluentialTurner, B. J., Ackerley, S., Davies, K. E., & Talbot, K. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp550 · Cited by 69
Ditsworth, D., Maldonado, M., McAlonis‐Downes, M., Sun, S., Seelman, A., Drenner, K., Arnold, E. S., Ling, S., Pizzo, D. P., Ravits, J., et al. (2017) · Acta Neuropathologica · doi:10.1007/s00401-017-1698-6 · Cited by 72
“To test the degree to which motor phenotypes were caused by cell intrinsic damage from mutant TDP-43 expressed within motor neurons, the TDP-43 animals were bred to VChAT-Cre mice that have previously been shown to postnatally express Cre recombinase selectively within motor neurons [40, 69] (Fig.”
BackgroundMethodInfluentialSelf-citationLuís, J. P., Simões, C. J. V., & Brito, R. M. M. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23031731 · Cited by 74
“Amongst the several intrinsic mechanisms proposed for neuronal death in ALS, the involvement of inflammation is now believed to be essential to disease progression [99,100].”
BackgroundCx43 hemichannels contribute to astrocyte-mediated toxicity in sporadic and familial ALS
Almad, A. A., Taga, A., Joseph, J., Gross, S. K., Welsh, C., Patankar, A. G., Richard, J., Rust, K., Pokharel, A., Plott, C. F., et al. (2022) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2107391119 · Cited by 68
“Astrocytes have been implicated in ALS progression after disease onset (21).”
BackgroundThe Role of Oligodendroglial Dysfunction in Amyotrophic Lateral Sclerosis
Nonneman, A., Robberecht, W., & Bosch, L. V. D. (2014) · Neurodegenerative Disease Management · doi:10.2217/nmt.14.21 · Cited by 76
FABP7 upregulation induces a neurotoxic phenotype in astrocytes
Killoy, K. M., Harlan, B. A., Pehar, M., & Vargas, M. R. (2020) · Glia · doi:10.1002/glia.23879 · Cited by 64
“…< .05) et al., 2011; Kia et al., 2018; Nagai et al., 2007; Pehar et al., 2017; Qian et al., 2017; Vargas et al., 2006; Wang, Gutmann, & Roos, 2011; Yamanaka et al., 2008), and therapeutic strategies that abrogate the neurotoxic phenotype of ALS-astrocytes in vitro, have been shown to increase…”
BackgroundNiu, C., Zhang, J. Y., Gao, F., Yang, L., Jia, M., Zhu, H., & Gong, W. (2012) · PLoS ONE · doi:10.1371/journal.pone.0047056 · Cited by 66
Heterogeneous astrocytes: Active players in CNS
Hu, X., Yuan, Y., Wang, D., & Su, Z. (2016) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2016.03.017 · Cited by 73
“Onset and progression in inherited ALS determined by motor neurons and microglia.”
InfluentialPeripheral and Central Nervous System Immune Response Crosstalk in Amyotrophic Lateral Sclerosis
Liu, Z., Cheng, X., Zhong, S., Zhang, X., Liu, C., Liu, F., & Zhao, C. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00575 · Cited by 68
Spinal cord extracts of amyotrophic lateral sclerosis spread TDP-43 pathology in cerebral organoids
Tamaki, Y., Ross, J. P., Alipour, P., Castonguay, C.-E., Li, B., Catoire, H., Rochefort, D. L., Urushitani, M., Takahashi, R., Sonnen, J. A., et al. (2023) · PLoS Genetics · doi:10.1371/journal.pgen.1010606 · Cited by 67
Self-citationMotor neuron trophic factors: Therapeutic use in ALS?
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Garbuzova-Davis, S. N., Rodrigues, M. C. O., Mirtyl, S., Turner, S., Mitha, S. A., Sodhi, J., Suthakaran, S., Eve, D. J., Sanberg, C. D., Kuzmin‐Nichols, N., et al. (2012) · PLoS ONE · doi:10.1371/journal.pone.0031254 · Cited by 66
“The concept that non-neuronal cells contribute to the disease process, known as the ‘‘non-cell autonomous nature of motor neuron death’’, is supported by several authors [57–59].”
ResultMotor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2
Foran, E., Bogush, A. I., Goffredo, M., Roncaglia, P., Gustincich, S., Pasinelli, P., & Trotti, D. (2011) · Glia · doi:10.1002/glia.21218 · Cited by 69
“In amyotrophic lateral sclerosis (ALS), astrocytes have emerged as key determinants of the progressive degeneration of motor neurons (Yamanaka et al., 2008), either directly by releasing toxic factors (Gandelman et al., 2010; Nagai et al., 2007) and/or contributing indirectly through the loss of…”
BackgroundAstrocytes and microglia in neurodegenerative diseases: Lessons from human in vitro models
Franklin, H., Clarke, B. E., & Patani, R. (2021) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2020.101973 · Cited by 66
“neuron death in ALS in animal models of the disease (Boillée et al., 2006; Yamanaka et al., 2008).”
Pathology of callosal damage in ALS: An ex-vivo, 7 T diffusion tensor MRI study
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SOD1 silencing in motoneurons or glia rescues neuromuscular function in ALS mice
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“These results, however, support previous studies demonstrating that astrocytes play a key role in disease progression, whereas motoneurons are critical for initiation of disease processes.(9,15,19) Previous studies using Cre-mediated recombination to suppress SOD1 expression in motoneurons did not show a higher effect…”
ResultProteomic characterization of lipid raft proteins in amyotrophic lateral sclerosis mouse spinal cord
Zhai, J., Ström, A., Kilty, R., Venkatakrishnan, P., White, J., Everson, W. V., Smart, E. J., & Zhu, H. (2009) · FEBS Journal · doi:10.1111/j.1742-4658.2009.07057.x · Cited by 65
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“In particular, microglia and astrocytes substantially contribute to motor neuron death and disease progression [4,5,6,11,12, 13,14,15].”
BackgroundGenetic Rodent Models of Amyotrophic Lateral Sclerosis
Bosch, L. V. D. (2011) · BioMed Research International · doi:10.1155/2011/348765 · Cited by 70
“This selective silencing of mutant SOD1 expression in astrocytes is obtained by crossbreeding transgenic mice containing floxed mutant SOD1 with mice expressing Cre driven by the glial fibrillary acidic protein (GFAP) promoter [79].”
Rodríguez‐Cueto, C., Santos‐García, I., García‐Toscano, L., Espejo‐Porras, F., Bellido, M., Fernández‐Ruíz, J., Muñóz, E., & de Lago, E. (2018) · Biochemical Pharmacology · doi:10.1016/j.bcp.2018.07.049 · Cited by 66
Miro1 deficiency in amyotrophic lateral sclerosis
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“Some previous studies revealed that either glutamate transporters or glutamatetransport activities were reduced in transgenic mice expressing ALS-associated SOD1 mutants, indicating the involvement of glutamate excitotoxicity in mediating ALS-mutant SOD1 neuronal toxicity (Bruijn et al., 1997; Bendotti et al., 2001; W…”
BackgroundOlivera-Bravo, S., Fernández, A., Sarlabós, M. N., Rosillo, J., Casanova, G., Jiménez, M., & Barbeito, L. (2011) · PLoS ONE · doi:10.1371/journal.pone.0020831 · Cited by 62
“Moreover, astrocytes bearing the amyotrophic lateral sclerosis (ALS) linked to superoxide dismutase 1 G93A (SOD1) mutation also induce motoneuron killing in both coculture conditions and through soluble factors found in the culture media [32,33,34,35].”
BackgroundCSF markers in amyotrophic lateral sclerosis
Tarasiuk, J., Kułakowska, A., Drozdowski, W., Kornhuber, J., & Lewczuk, P. (2012) · Journal of Neural Transmission · doi:10.1007/s00702-012-0806-y · Cited by 64
“…factor (Brettschneider et al. 2006d) S100b ; Modulates the activity of effector proteins or cells (Boillée et al. 2006b; Süssmuth et al. 2010; Yamanaka et al. 2008) Glutamine synthetase : Glutamine synthesis (Tumani et al. 1999) : increased, ; decreased, $ no significant…”
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Cellular calcium signaling in the aging brain
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Motor neuron-derived microRNAs cause astrocyte dysfunction in amyotrophic lateral sclerosis
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“There are many studies examining the roles of astrocytes and microglia in animal models of neurodegenerative disease [41,42].”
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“Importantly, these culture systems complement genetic in vivo models for studies of physiopathological mechanisms and are instrumental in the development of therapeutic strategies (Van Damme et al. 2005, 2007; Boillee et al. 2006; Locatelli et al. 2007; Yamanaka et al. 2008).”
BackgroundNeuronal expression of Fig4 is both necessary and sufficient to prevent spongiform neurodegeneration
Ferguson, C., Lenk, G. M., Jones, J. M., Grant, A. E., Winters, J. J., Dowling, J. J., Giger, R. J., & Meisler, M. H. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds179 · Cited by 62
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“The astroglial role in ALS is further corroborated by the finding that selective silencing of ALS-related mutant gene encoding superoxide dismutase 1 in astrocytes markedly delays pathological development (Wang et al. 2011; Yamanaka et al. 2008).”
Cui, Y., Masaki, K., Yamasaki, R., Imamura, S., Suzuki, S. O., Hayashi, S., Sato, S., Nagara, Y., Kawamura, M. F., & Kira, J. (2014) · Journal of Neuroinflammation · doi:10.1186/1742-2094-11-42 · Cited by 61
“Astrocytes and microglia have been extensively investigated; evidence from studies using selective gene excision [1,2] or bone-marrow grafting [3] has shown that mutant SOD1-derived alterations in either microglia or astrocytes accelerate later disease progression in mice.”
BackgroundLasiene, J., Komine, O., Fujimori-Tonou, N., Powers, B. E., Endo, F., Watanabe, S., Jin, S., Ravits, J., Horner, P. J., Misawa, H., et al. (2016) · Acta Neuropathologica Communications · doi:10.1186/s40478-016-0286-7 · Cited by 62
“Immunofluorescence was performed as described previously [21].”
MethodSelf-citationKomine, O., Yamashita, H., Fujimori-Tonou, N., Koike, M., Jin, S., Moriwaki, Y., Endo, F., Watanabe, S., Uematsu, S., Akira, S., et al. (2018) · Cell Death and Differentiation · doi:10.1038/s41418-018-0098-3 · Cited by 66
“[8–10], astrocytes [11, 12], or oligodendrocytes [13] significantly slows the disease progression of mutant SOD1-”
Self-citationRole of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
Ferrara, D., Pasetto, L., Bonetto, V., & Basso, M. (2018) · Frontiers in Neuroscience · doi:10.3389/fnins.2018.00574 · Cited by 65
“In contrast, the selective removal of mutant SOD1 from microglia or astrocytes in vivo slowed the progression of the disease (Beers et al., 2006; Boillee et al., 2006; Yamanaka et al., 2008; Wang et al., 2009), giving glial cells a central role in disease propagation (Ilieva et al.”
BackgroundFrom nucleation to widespread propagation: A prion-like concept for ALS
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The Links between ALS and NF-κB
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“The addition of in vitro fibrillated proteins to cultured cells has been shown to seed soluble endogenous proteins in a sequence-specific manner (Yang et al., 2002; Danzer et al., 2009; Frost et al., 2009; Ren et al., 2009; Münch et al., 2011; Wang et al., 2012; Nonaka et al., 2013).”
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“Although motoneurons is considered as the prime cell type to be targeted, other cells are also implicated in ALS pathogenesis via non-cell-autonomous mechanisms (Clement et al. , 2003; Boillee et al. , 2006; Yamanaka et al. , 2008).”
de Jongh, R., Spijkers, X. M., Pasteuning-Vuhman, S., Vulto, P., & Pasterkamp, R. J. (2020) · Journal of Neurochemistry · doi:10.1111/jnc.15289 · Cited by 64
“Deterioration at the later stages can be modulated by the expression of mutant SOD1 in microglial cells and astrocytes (Boillee et al., 2006; Ilieva et al., 2009; Yamanaka et al., 2008).”
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Mortal engines: Mitochondrial bioenergetics and dysfunction in neurodegenerative diseases
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Astroglial Calcium Signalling in Alzheimer's Disease
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“Incidentally, a cell-specific silencing of the muta nt SOD1 gene in astrocytes significantly delayed development of clinical symptoms [84].”
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“This property in turn may be related to their hematopoietic origin (Yokoyama et al., 2006).”
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“Importantly, both intrinsic (from motor neurons) and extrinsic (from surrounding non-neuronal glial cells) pathways are implicated in motor neuron cell death in ALS (Boillee et al., 2006; Clement et al., 2003; Kang et al., 2013; Yamanaka et al., 2008).”
InfluentialHossaini, M., Cano, S. C., van Dis, V., Haasdijk, E. D., Hoogenraad, C. C., Holstege, J. C., & Jaarsma, D. (2011) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e31822581ac · Cited by 65
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“Furthermore, deletion of the SOD1 mutant gene in either astrocytes or microglia, while not effecting disease onset, does significantly delay disease progression (Boillee et al. 2006b; Yamanaka et al. 2008).”
Background11 C‐PK11195 PET–based molecular study of microglia activation in SOD1 amyotrophic lateral sclerosis
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“11C-PK11195 is the first, prototypical PET tracer for the in vivo detection of the 18-kDa translocator protein (TSPO), an outer mitochondrial membrane protein which is overexpressed in activated microglia and reactive astrocytes, thus becoming a sensitive marker of neuroinflammation.35 A TSPO overexpression as detecte…”
BackgroundInfluentialBarbosa, M., Gomes, C., Sequeira, C., Gonçalves‐Ribeiro, J., Pina, C. C., Carvalho, L. A. R., Moreira, R., Vaz, S. H., Vaz, A. R., & Brites, D. M. T. O. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2021.634355 · Cited by 58
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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