Immunity in amyotrophic lateral sclerosis: blurred lines between excessive inflammation and inefficient immune responses
Open accessAbstract
Despite wide genetic, environmental and clinical heterogeneity in amyotrophic lateral sclerosis, a rapidly fatal neurodegenerative disease targeting motoneurons, neuroinflammation is a common finding. It is marked by local glial activation, T cell infiltration and systemic immune system activation. The immune system has a prominent role in the pathogenesis of various chronic diseases, hence some of them, including some types of cancer, are successfully targeted by immunotherapeutic approaches. However, various anti-inflammatory or immunosuppressive therapies in amyotrophic lateral sclerosis have failed. This prompted increased scrutiny over the immune-mediated processes underlying amyotrophic lateral sclerosis. Perhaps the biggest conundrum is that amyotrophic lateral sclerosis pathogenesis exhibits features of three otherwise distinct immune dysfunctions—excessive inflammation, autoimmunity and inefficient immune responses. Epidemiological and genome-wide association studies show only minimal overlap between amyotrophic lateral sclerosis and autoimmune diseases, so excessive inflammation is usually thought to be secondary to protein aggregation, mitochondrial damage or other stresses. In contrast, several recently characterized amyotrophic lateral sclerosis-linked mutations, including those in TBK1, OPTN, CYLD and C9orf72, could lead to inefficient immune responses and/or damage pile-up, suggesting that an innate immunodeficiency may also be a trigger and/or modifier of this disease. In such cases, non-selective immunosuppression would further restrict neuroprotective immune responses. Here we discuss multiple layers of immune-mediated neuroprotection and neurotoxicity in amyotrophic lateral sclerosis. Particular focus is placed on individual patient mutations that directly or indirectly affect the immune system, and the mechanisms by which these mutations influence disease progression. The topic of immunity in amyotrophic lateral sclerosis is timely and relevant, because it is one of the few common and potentially malleable denominators in this heterogenous disease. Importantly, amyotrophic lateral sclerosis progression has recently been intricately linked to patient T cell and monocyte profiles, as well as polymorphisms in cytokine and chemokine receptors. For this reason, precise patient stratification based on immunophenotyping will be crucial for efficient therapies.
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Many ALS genetic mutations are associated with immune response, such as C9orf72, TBK1, CYLD, OPTN, and PGRN.
“Many of the genetic mutations are associated with immune response, such as C9orf72, TBK1, CYLD, OPTN, PGRN, and so on.85,272”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [85]
The ‘ALS genetics and mechanisms’ section (page unavailable) links C9orf72, TBK1, CYLD and OPTN to innate immune reactions, but describes these as a small subset of ALS-linked genes rather than ‘many’ mutations. Our copy does not establish the claimed link for PGRN (searched: PGRN and progranulin). (LLM, conf=0.89)
Experimental — accuracy not yet measuredPersistent inflammation damages neurons directly and exacerbates ALS.
“Persistent inflammation damages neurons directly and exacerbates ALS78,85”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [85]
More than 30 genes strongly correlate with ALS, although their exact roles in disease development are still not completely understood.
“More than 30 of these genes strongly correlate with the disease, although their exact roles in disease development are still not completely understood.85”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [85]
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Polazzi, E., Mengoni, I., Caprini, M., Peña‐Altamira, E., Kurtys, E., & Monti, B. (2012) · Neurosignals · doi:10.1159/000337115 · Cited by 9,023 · see citing works
Neurotoxic reactive astrocytes are induced by activated microglia
Liddelow, S. A., Guttenplan, K. A., Clarke, L. E., Bennett, F. C., Bohlen, C. J., Schirmer, L., Bennett, M. L., Münch, A. E., Chung, W.-S., Peterson, T. C., et al. (2017) · Nature · doi:10.1038/nature21029 · Cited by 7,971 · see citing works
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
Resting microglial cells are highly dynamic surveillants of brain parenchyma in vivo
Nimmerjahn, A., Kirchhoff, F., & Helmchen, F. (2005) · Science · doi:10.1126/science.1110647 · Cited by 5,827 · 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
Inflamm‐aging: An Evolutionary Perspective on Immunosenescence
FRANCESCHI, C., BONAFÈ, M., VALENSIN, S., OLIVIERI, F., DE LUCA, M., OTTAVIANI, E., & DE BENEDICTIS, G. (2000) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2000.tb06651.x · Cited by 5,358 · see citing works
DeJesus-Hernandez, M., Mackenzie, I. R., Boeve, B. F., Boxer, A. L., Baker, M., Rutherford, N. J., Nicholson, A. M., Finch, N. A., Flynn, H., Adamson, J., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.011 · Cited by 4,936 · see citing works
Structural and functional features of central nervous system lymphatic vessels
Louveau, A., Smirnov, I., Keyes, T. J., Eccles, J. D., Rouhani, S. J., Peske, J. D., Derecki, N. C., Castle, D., Mandell, J. W., Lee, K. S., et al. (2015) · Nature · doi:10.1038/nature14432 · Cited by 4,413 · see citing works
ATP mediates rapid microglial response to local brain injury in vivo
Davalos, D., Grutzendler, J., Yang, G., Kim, J. V., Zuo, Y., Jung, S., Littman, D. R., Dustin, M. L., & Gan, W.-B. (2005) · Nature Neuroscience · doi:10.1038/nn1472 · Cited by 3,913 · see citing works
Inflammaging: a new immune–metabolic viewpoint for age-related diseases
Franceschi, C., Garagnani, P., Parini, P., Giuliani, C., & Santoro, A. (2018) · Nature Reviews Endocrinology · doi:10.1038/s41574-018-0059-4 · Cited by 3,586 · see citing works
Host microbiota constantly control maturation and function of microglia in the CNS
Erny, D., Hrabě de Angelis, A. L., Jaitin, D., Wieghofer, P., Staszewski, O., David, E., Keren-Shaul, H., Mahlakoiv, T., Jakobshagen, K., Buch, T., et al. (2015) · Nature Neuroscience · doi:10.1038/nn.4030 · Cited by 3,569 · see citing works
Kettenmann, H., Hanisch, U.-K., Noda, M., & Verkhratsky, A. (2011) · Physiological Reviews · doi:10.1152/physrev.00011.2010 · Cited by 3,547 · see citing works
Krasemann, S., Madore, C., Cialic, R., Baufeld, C., Calcagno, N., El Fatimy, R., Beckers, L., O’Loughlin, E., Xu, Y., Fanek, Z., et al. (2017) · Immunity · doi:10.1016/j.immuni.2017.08.008 · Cited by 2,979 · see citing works
The ubiquitin kinase PINK1 recruits autophagy receptors to induce mitophagy
Lazarou, M., Sliter, D. A., Kane, L. A., Sarraf, S. A., Wang, C., Burman, J. L., Sideris, D. P., Fogel, A. I., & Youle, R. J. (2015) · Nature · doi:10.1038/nature14893 · Cited by 2,774 · see citing works
The family of five: TIR-domain-containing adaptors in Toll-like receptor signalling
O'Neill, L. A. J., & Bowie, A. G. (2007) · Nature Reviews Immunology · doi:10.1038/nri2079 · Cited by 2,667 · see citing works
Identification of a unique TGF-β–dependent molecular and functional signature in microglia
Butovsky, O., Jedrychowski, M. P., Moore, C. S., Cialic, R., Lanser, A. J., Gabriely, G., Koeglsperger, T., Dake, B., Wu, P. M., Doykan, C. E., et al. (2014) · Nature Neuroscience · doi:10.1038/nn.3599 · Cited by 2,543 · see citing works
Brown, R. H., & Al‐Chalabi, A. (2017) · New England Journal of Medicine · doi:10.1056/nejmra1603471 · Cited by 2,472 · see citing works
Reactive Astrocytes: Production, Function, and Therapeutic Potential
Liddelow, S. A., & Barres, B. A. (2017) · Immunity · doi:10.1016/j.immuni.2017.06.006 · Cited by 2,336 · see citing works
A dural lymphatic vascular system that drains brain interstitial fluid and macromolecules
Aspelund, A., Antila, S., Proulx, S. T., Karlsen, T. V., Karaman, S., Detmar, M., Wiig, H., & Alitalo, K. (2015) · Journal of Experimental Medicine · doi:10.1084/jem.20142290 · Cited by 2,198 · see citing works
Hardiman, O. M., Al‐Chalabi, A., Chiò, A., Corr, E. M., Logroscino, G., Robberecht, W., Shaw, P. J., Simmons, Z., & van den Berg, L. H. (2017) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2017.71 · Cited by 2,191 · see citing works
Neurofilaments as biomarkers in neurological disorders
Khalil, M., Teunissen, C. E., Otto, M., Piehl, F., Sormani, M. P., Gattringer, T., Barro, C., Kappos, L., Comabella, M., Fazekas, F., et al. (2018) · Nature Reviews Neurology · doi:10.1038/s41582-018-0058-z · Cited by 1,988 · see citing works
How neuroinflammation contributes to neurodegeneration
Ransohoff, R. M. (2016) · Science · doi:10.1126/science.aag2590 · Cited by 1,967 · cited 3× · see citing works
“It is also an early and common hallmark of neurodegenerative diseases (Ransohoff, 2016).”
BackgroundResultConverging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
Ling, S.-C., Polymenidou, M., & Cleveland, D. W. (2013) · Neuron · doi:10.1016/j.neuron.2013.07.033 · Cited by 1,811 · see citing works
New tools for studying microglia in the mouse and human CNS
Bennett, M. L., Bennett, F. C., Liddelow, S. A., Ajami, B., Zamanian, J. L., Fernhoff, N. B., Mulinyawe, S. B., Bohlen, C. J., Adil, A., Tucker, A., et al. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1525528113 · Cited by 1,804 · see citing works
Local self-renewal can sustain CNS microglia maintenance and function throughout adult life
Ajami, B., Bennett, J. L., Krieger, C., Tetzlaff, W., & Rossi, F. M. V. (2007) · Nature Neuroscience · doi:10.1038/nn2014 · Cited by 1,643 · see citing works
Neuroinflammation: The Devil Is in the Details
DiSabato, D. J., Quan, N., & Godbout, J. P. (2016) · Journal of Neurochemistry · doi:10.1111/jnc.13607 · Cited by 1,640 · 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
State of play in amyotrophic lateral sclerosis genetics
Renton, A. E., Chiò, A., & Traynor, B. J. (2013) · Nature Neuroscience · doi:10.1038/nn.3584 · Cited by 1,563 · see citing works
Disease-Associated Microglia: A Universal Immune Sensor of Neurodegeneration
Deczkowska, A., Keren-Shaul, H., Weiner, A., Colonna, M., Schwartz, M., & Amit, I. (2018) · Cell · doi:10.1016/j.cell.2018.05.003 · Cited by 1,474 · 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
Microglia and brain macrophages in the molecular age: from origin to neuropsychiatric disease
Prinz, M., & Priller, J. (2014) · Nature Reviews Neuroscience · doi:10.1038/nrn3722 · Cited by 1,329 · see citing works
Mutations of optineurin in amyotrophic lateral sclerosis
Maruyama, H., Morino, H., Ito, H., Izumi, Y., Kato, H., Watanabe, Y., Kinoshita, Y., Kamada, M., Nodera, H., Suzuki, H., et al. (2010) · Nature · doi:10.1038/nature08971 · Cited by 1,265 · see citing works
Ziv, Y., Ron, N., Butovsky, O., Landa, G., Sudai, E., Greenberg, N., Cohen, H., Kipnis, J., & Schwartz, M. (2006) · Nature Neuroscience · doi:10.1038/nn1629 · Cited by 1,209 · see citing works
Origin, fate and dynamics of macrophages at central nervous system interfaces
Goldmann, T., Wieghofer, P., Jordão, M. J. C., Prutek, F., Hagemeyer, N., Frenzel, K., Amann, L., Staszewski, O., Kierdorf, K., Krueger, M., et al. (2016) · Nature Immunology · doi:10.1038/ni.3423 · Cited by 1,209 · see citing works
Microglia: new roles for the synaptic stripper
Kettenmann, H., Kirchhoff, F., & Verkhratsky, A. (2013) · Neuron · doi:10.1016/j.neuron.2012.12.023 · Cited by 1,173 · see citing works
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
Yamanaka, K., Chun, S. J., Boillee, S., Fujimori-Tonou, N., Yamashita, H., Gutmann, D. H., Takahashi, R., Misawa, H., & Cleveland, D. W. (2008) · Nature Neuroscience · doi:10.1038/nn2047 · Cited by 1,140 · see citing works
Microglial priming in neurodegenerative disease
Perry, V. H., & Holmes, C. (2014) · Nature Reviews Neurology · doi:10.1038/nrneurol.2014.38 · Cited by 1,127 · see citing works
Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice
Clement, A. M., Nguyen, M. D., Roberts, E. A., Garcia, M. L., Boillée, S., Rule, M., McMahon, A. P., Doucette, W. T., Siwek, D. F., Ferrante, R. J., et al. (2003) · Science · doi:10.1126/science.1086071 · Cited by 1,101 · see citing works
Medawar, P. B. (1948) · PubMed · Cited by 1,084 · see citing works
Mrdjen, D., Pavlovic, A., Hartmann, F. J., Schreiner, B., Utz, S. G., Leung, B. P., Lelios, I., Heppner, F. L., Kipnis, J., Merkler, D., et al. (2018) · Immunity · doi:10.1016/j.immuni.2018.01.011 · Cited by 1,010 · see citing works
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.
Zhang, K., Donnelly, C. J., Haeusler, A. R., Grima, J. C., Machamer, J. B., Steinwald, P., Daley, E. L., Miller, S. J., Cunningham, K. M., Vidensky, S., et al. (2015) · Nature · doi:10.1038/nature14973 · Cited by 982 · see citing works
Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways
Cirulli, E. T., Lasseigne, B. N., Petrovski, S., Sapp, P. C., Dion, P. A., Leblond, C. S., Couthouis, J., Lu, Y.-F., Wang, Q., Krueger, B. J., et al. (2015) · Science · doi:10.1126/science.aaa3650 · Cited by 966 · see citing works
Heo, J.-M., Ordureau, A., Paulo, J. A., Rinehart, J., & Harper, J. W. (2015) · Molecular Cell · doi:10.1016/j.molcel.2015.08.016 · Cited by 906 · see citing works
Autoimmune T cells protect neurons from secondary degeneration after central nervous system axotomy
Moalem‐Taylor, G., Leibowitz‐Amit, R., Yoles, E., Mor, F., Cohen, I. R., & Schwartz, M. (1999) · Nature Medicine · doi:10.1038/4734 · Cited by 882 · see citing works
Microglia: Scapegoat, saboteur, or something else?
Aguzzi, A., Barres, B. A., & Bennett, M. L. (2013) · Science · doi:10.1126/science.1227901 · Cited by 836 · see citing works
McGeer, P. L., Itagaki, S., Tago, H., & McGeer, E. G. (1987) · Neuroscience Letters · doi:10.1016/0304-3940(87)90696-3 · Cited by 836 · see citing works
Godbout, J. P., Chen, J., Abraham, J., Richwine, A. F., Berg, B. M., Kelley, K. W., & Johnson, R. W. (2005) · The FASEB Journal · doi:10.1096/fj.05-3776fje · Cited by 813 · see citing works
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia
Freischmidt, A., Wieland, T., Richter, B., Ruf, W., Schaeffer, V., Müller, K., Marroquin, N., Nordin, F., Hübers, A., Weydt, P., et al. (2015) · Nature Neuroscience · doi:10.1038/nn.4000 · Cited by 786 · see citing works
Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
Haidet-Phillips, A. M., Hester, M. E., Miranda, C. J., Meyer, K., Braun, L., Frakes, A., Song, S., Likhite, S., Murtha, M. J., Foust, K. D., et al. (2011) · Nature Biotechnology · doi:10.1038/nbt.1957 · Cited by 782 · see citing works
Layer V cortical neurons require microglial support for survival during postnatal development
Ueno, M., Fujita, Y., Tanaka, T., Nakamura, Y., Kikuta, J., Ishii, M., & Yamashita, T. (2013) · Nature Neuroscience · doi:10.1038/nn.3358 · Cited by 781 · see citing works
Richter, B., Sliter, D. A., Herhaus, L., Stolz, A., Wang, C., Beli, P., Zaffagnini, G., Wild, P., Martens, S., Wagner, S. A., et al. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1523926113 · Cited by 755 · see citing works
ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?
Mejzini, R., Flynn, L. L., Pitout, I. L., Fletcher, S., Wilton, S. D., & Akkari, P. A. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.01310 · Cited by 755 · see citing works
Turner, M. R., Cagnin, A., Turkheimer, F. E., Miller, C. C. J., Shaw, C. E., Brooks, D. J., Leigh, P. N., & Banati, R. B. (2004) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.12.012 · Cited by 737 · see citing works
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
über eigenartige Krankheitsfälle des späteren Alters
Alzheimer, A. (1911) · Zeitschrift für die gesamte Neurologie und Psychiatrie · doi:10.1007/bf02866241 · Cited by 717 · see citing works
Potential roles of gut microbiome and metabolites in modulating ALS in mice
Blacher, E., Bashiardes, S., Shapiro, H., Rothschild, D., Mor, U., Dori-Bachash, M., Kleimeyer, C., Moresi, C., Harnik, Y., Zur, M., et al. (2019) · Nature · doi:10.1038/s41586-019-1443-5 · Cited by 700 · see citing works
Immune activation in brain aging and neurodegeneration: too much or too little?
Lucin, K. M., & Wyss-Coray, T. (2009) · Neuron · doi:10.1016/j.neuron.2009.08.039 · Cited by 679 · see citing works
The ins and outs of T-lymphocyte trafficking to the CNS: anatomical sites and molecular mechanisms
Engelhardt, B., & Ransohoff, R. M. (2005) · Trends in Immunology · doi:10.1016/j.it.2005.07.004 · Cited by 674 · see citing works
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
Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease
Philips, T., & Robberecht, W. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70015-1 · Cited by 646 · see citing works
Microglia 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 · see citing works
Aging-induced type I interferon response at the choroid plexus negatively affects brain function
Baruch, K., Deczkowska, A., David, E., Castellano, J. M., Miller, O., Kertser, A., Berkutzki, T., Barnett-Itzhaki, Z., Bezalel, D., Wyss-Coray, T., et al. (2014) · Science · doi:10.1126/science.1252945 · Cited by 615 · see citing works
RIPK1 mediates axonal degeneration by promoting inflammation and necroptosis in ALS
Ito, Y., Ofengeim, D., Najafov, A., Das, S., Saberi, S., Li, Y., Hitomi, J., Zhu, H., Chen, H., Mayo, L., et al. (2016) · Science · doi:10.1126/science.aaf6803 · Cited by 606 · see citing works
Interplay between innate immunity and Alzheimer disease: APOE and TREM2 in the spotlight
Shi, Y., & Holtzman, D. M. (2018) · Nature Reviews Immunology · doi:10.1038/s41577-018-0051-1 · Cited by 595 · see citing works
Gendron, T. F., Bieniek, K. F., Zhang, Y., Jansen‐West, K. R., Ash, P. E. A., Caulfield, T. R., Daughrity, L. M., Dunmore, J., Castanedes‐Casey, M., Chew, J., et al. (2013) · Acta Neuropathologica · doi:10.1007/s00401-013-1192-8 · Cited by 569 · see citing works
Henkel-Tigges, J., Engelhardt, J., Siklós, L., Simpson, E. P., Kim, S. H., Pan, T., Goodman, J. C., Siddique, T., Beers, D. R., & Appel, S. H. (2003) · Annals of Neurology · doi:10.1002/ana.10805 · Cited by 554 · see citing works
<i>C9orf72</i> is required for proper macrophage and microglial function in mice
O’Rourke, J. G., Bogdanik, L., Yáñez, A., Lall, D., Wolf, A. J., Muhammad, A. K. M. G., Ho, R., Carmona, S., Vit, J. P., Zarrow, J., et al. (2016) · Science · doi:10.1126/science.aaf1064 · Cited by 550 · see citing works
Recognition of Endogenous Nucleic Acids by the Innate Immune System
Roers, A., Hiller, B., & Hornung, V. (2016) · Immunity · doi:10.1016/j.immuni.2016.04.002 · Cited by 541 · see citing works
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
Human endogenous retrovirus-K contributes to motor neuron disease
Li, W., Lee, M.-H., Henderson, L., Tyagi, R., Bachani, M., Steiner, J., Campanac, E., Hoffman, D. A., von Geldern, G., Johnson, K., et al. (2015) · Science Translational Medicine · doi:10.1126/scitranslmed.aac8201 · Cited by 482 · see citing works
Modulating inflammatory monocytes with a unique microRNA gene signature ameliorates murine ALS
Butovsky, O., Siddiqui, S., Gabriely, G., Lanser, A. J., Dake, B., Murugaiyan, G., Doykan, C. E., Wu, P. M., Gali, R. R., Iyer, L. K., et al. (2012) · Journal of Clinical Investigation · doi:10.1172/jci62636 · Cited by 481 · see citing works
Beers, 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 468 · 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
Origin of microglia: current concepts and past controversies
Ginhoux, F., & Prinz, M. (2015) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a020537 · Cited by 446 · see citing works
Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS
Liao, B., Zhao, W., Beers, D. R., Henkel-Tigges, J., & Appel, S. H. (2012) · Experimental Neurology · doi:10.1016/j.expneurol.2012.06.011 · Cited by 438 · see citing works
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
Expression of the histocompatibility glycoprotein HLA-DR in neurological disease
McGeer, P. L., Itagaki, S., & McGeer, E. G. (1988) · Acta Neuropathologica · doi:10.1007/bf00689592 · Cited by 420 · see citing works
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
Sellier, C., Campanari, M., Julie Corbier, C., Gaucherot, A., Kolb‐Cheynel, I., Oulad‐Abdelghani, M., Ruffenach, F., Page, A., Ciura, S., Kabashi, E., et al. (2016) · The EMBO Journal · doi:10.15252/embj.201593350 · Cited by 414 · see citing works
Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS
Ré, D. B., Verche, V. L., Yu, C., Amoroso, M. W., Politi, K. A., Phani, S., Ikiz, B., Hoffmann, L., Koolen, M., Nagata, T., et al. (2014) · Neuron · doi:10.1016/j.neuron.2014.01.011 · Cited by 404 · see citing works
Ontogeny and homeostasis of CNS myeloid cells
Prinz, M., Erny, D., & Hagemeyer, N. (2017) · Nature Immunology · doi:10.1038/ni.3703 · Cited by 391 · see citing works
Immune dysregulation in amyotrophic lateral sclerosis: mechanisms and emerging therapies
Beers, D. R., & Appel, S. H. (2019) · The Lancet Neurology · doi:10.1016/s1474-4422(18)30394-6 · Cited by 387 · see citing works
Geissmann, F., Auffray, C., Palframan, R. T., Wirrig, C., Ciocca, A., Campisi, L., Narni-Mancinelli, É., & Lauvau, G. S. (2008) · Immunology and Cell Biology · doi:10.1038/icb.2008.19 · Cited by 385 · see citing works
Inflammatory neuroprotection following traumatic brain injury
Russo, M. V., & McGavern, D. B. (2016) · Science · doi:10.1126/science.aaf6260 · Cited by 380 · see citing works
C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD
Liu, Y., Pattamatta, A., Zu, T., Reid, T. S., Bardhi, O., Borchelt, D., Yachnis, A. T., & Ranum, L. P. W. (2016) · Neuron · doi:10.1016/j.neuron.2016.04.005 · Cited by 368 · see citing works
Toll‐like receptors in central nervous system glial inflammation and homeostasis
Kielian, T. (2006) · Journal of Neuroscience Research · doi:10.1002/jnr.20767 · Cited by 363 · see citing works
Regulatory T‐lymphocytes mediate amyotrophic lateral sclerosis progression and survival
Henkel-Tigges, J., Beers, D. R., Wen, S., Rivera, A. L., Toennis, K. M., Appel, J. E., Zhao, W., Moore, D. H., Powell, S. Z.-E., & Appel, S. H. (2012) · EMBO Molecular Medicine · doi:10.1002/emmm.201201544 · Cited by 355 · see citing works
Serio, A., Bilican, B., Barmada, S. J., Ando, D. M., Zhao, C., Siller, R., Burr, K., Haghi, G., Story, D., Nishimura, A. L., et al. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1300398110 · Cited by 345 · see citing works
Swarup, V., Phaneuf, D., Dupré, N., Petri, S., Strong, M. J., Križ, J., & Julien, J. (2011) · The Journal of Experimental Medicine · doi:10.1084/jem.20111313 · Cited by 338 · see citing works
Ajami, B., Samusik, N., Wieghofer, P., Ho, P. P., Crotti, A., Bjornson, Z., Prinz, M., Fantl, W. J., Nolan, G. P., & Steinman, L. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0100-x · Cited by 338 · see citing works
Bilican, B., Serio, A., Barmada, S. J., Nishimura, A. L., Sullivan, G. J., Carrasco, M., Phatnani, H. P., Puddifoot, C. A., Story, D., Fletcher, J., et al. (2012) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1202922109 · Cited by 328 · see citing works
TBK1: a new player in ALS linking autophagy and neuroinflammation
Oakes, J. A., Davies, M. C., & Collins, M. O. (2017) · Molecular Brain · doi:10.1186/s13041-017-0287-x · Cited by 319 · see citing works
Douville, R. N., Liu, J., Rothstein, J., & Nath, A. (2010) · Annals of Neurology · doi:10.1002/ana.22149 · Cited by 318 · see citing works
Lymphocytic Infiltrates in the Spinal Cord in Amyotrophic Lateral Sclerosis
Engelhardt, J. I., Tajti, J., & Appel, S. H. (1993) · Archives of Neurology · doi:10.1001/archneur.1993.00540010026013 · Cited by 318 · see citing works
C9orf72 suppresses systemic and neural inflammation induced by gut bacteria
Burberry, A., Wells, M. F., Limone, F., Couto, A., Smith, K. S., Keaney, J., Gillet, G., van Gastel, N., Wang, J.-Y., Pietilainen, O., et al. (2020) · Nature · doi:10.1038/s41586-020-2288-7 · Cited by 314 · see citing works
Pottier, C., Bieniek, K. F., Finch, N., van de Vorst, M., Baker, M., Perkersen, R., Brown, P., Ravenscroft, T., van Blitterswijk, M., Nicholson, A. M., et al. (2015) · Acta Neuropathologica · doi:10.1007/s00401-015-1436-x · Cited by 312 · see citing works
Trial of celecoxib in amyotrophic lateral sclerosis
Cudkowicz, M., Shefner, J. M., Schoenfeld, D., Zhang, H., Andreasson, K. I., Rothstein, J. D., & Drachman, D. B. (2006) · Annals of Neurology · doi:10.1002/ana.20903 · Cited by 302 · see citing works
Spiller, K. J., Restrepo, C. R., Khan, T., Dominique, M. A., Fang, T. C., Canter, R. G., Roberts, C. J., Miller, K. R., Ransohoff, R. M., Trojanowski, J. Q., et al. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0083-7 · Cited by 301 · see citing works
Immune-mediated Mechanisms in the Pathoprogression of Amyotrophic Lateral Sclerosis
Zhao, W., Beers, D. R., & Appel, S. H. (2013) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-013-9489-x · Cited by 298 · see citing works
Sullivan, P. M., Zhou, X., Robins, A. M., Paushter, D. H., Kim, D., Smolka, M. B., & Hu, F. (2016) · Acta Neuropathologica Communications · doi:10.1186/s40478-016-0324-5 · Cited by 295 · 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
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
Zhu, G., Wu, C.-J., Zhao, Y., & Ashwell, J. D. (2007) · Current Biology · doi:10.1016/j.cub.2007.07.041 · Cited by 282 · see citing works
Head Injury and Amyotrophic Lateral Sclerosis
Chen, H., Richard, M., Sandler, D. P., Umbach, D. M., & Kamel, F. (2007) · American Journal of Epidemiology · doi:10.1093/aje/kwm153 · Cited by 279 · see citing works
Casadio, M., Ciancanelli, M. J., Ou, Y.-H., Lorenzo, L., Klaudel‐Dreszler, M., Pauwels, E., Sancho‐Shimizu, V., de Diego, R. P., Abhyankar, A., Israelsson, E., et al. (2012) · The Journal of Experimental Medicine · doi:10.1084/jem.20111316 · Cited by 279 · see citing works
C 9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits
Koppers, M., Blokhuis, A. M., Westeneng, H., Terpstra, M. L., Zundel, C. A. C., de Sá, R. V., Schellevis, R. D., Waite, A. J., Blake, D. J., Veldink, J. H., et al. (2015) · Annals of Neurology · doi:10.1002/ana.24453 · Cited by 268 · see citing works
Brettschneider, J., Toledo, J. B., Van Deerlin, V. M., Elman, L., McCluskey, L., Lee, V. M.-Y., & Trojanowski, J. Q. (2012) · PLoS ONE · doi:10.1371/journal.pone.0039216 · Cited by 268 · see citing works
Sierra, A., de Castro, F., del Río-Hortega, J., Iglesias-Rozas, J. R., Garrosa, M., & Kettenmann, H. (2016) · Glia · doi:10.1002/glia.23046 · Cited by 267 · see citing works
Swarup, V., Phaneuf, D., Bareil, C., Robertson, J., Rouleau, G. A., Križ, J., & Julien, J. (2011) · Brain · doi:10.1093/brain/awr159 · Cited by 264 · see citing works
Atanasio, A., Decman, V., White, D., Ramos, M., Ikiz, B., Lee, H.-C., Siao, C.-J., Brydges, S., LaRosa, E., Bai, Y., et al. (2016) · Scientific Reports · doi:10.1038/srep23204 · Cited by 263 · see citing works
Microglial translational profiling reveals a convergent APOE pathway from aging, amyloid, and tau
Kang, S. S., Ebbert, M. T. W., Baker, K. E., Cook, C., Wang, X., Sens, J. P., Kocher, J.-P., Petrucelli, L., & Fryer, J. D. (2018) · Journal of Experimental Medicine · doi:10.1084/jem.20180653 · Cited by 260 · see citing works
Immune aging, dysmetabolism, and inflammation in neurological diseases
Deleidi, M., Jäggle, M., & Rubino, G. (2015) · Frontiers in Neuroscience · doi:10.3389/fnins.2015.00172 · Cited by 260 · see citing works
Stem cell therapy in amyotrophic lateral sclerosis: a methodological approach in humans
Mazzini, L., Fagioli, F., Boccaletti, R., Mareschi, K., Oliveri, G., Olivieri, C., Pastore, I., Marasso, R., & Madon, E. (2003) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/14660820310014653 · Cited by 257 · see citing works
Self-citationBeers, D. R., Zhao, W., Wang, J., Zhang, X., Wen, S., Neal, D. W., Thonhoff, J. R., Alsuliman, A., Shpall, E. J., Rezvani, K., et al. (2017) · JCI Insight · doi:10.1172/jci.insight.89530 · Cited by 256 · see citing works
Molecular Imaging of Microglial Activation in Amyotrophic Lateral Sclerosis
Corcia, P., Tauber, C., Vercoullie, J., Arlicot, N., Prunier, C., Praline, J., Nicolas, G., Venel, Y., Hommet, C., Baulieu, J.-L., et al. (2012) · PLoS ONE · doi:10.1371/journal.pone.0052941 · Cited by 249 · see citing works
Ezzi, S. A., Urushitani, M., & Julien, J. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.04531.x · Cited by 248 · see citing works
Nguyen, M. D., D'Aigle, T., Gowing, G., Julien, J.-P., & Rivest, S. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.4786-03.2004 · Cited by 248 · see citing works
Costa, A., Toschi, A., Murfuni, I., Pelosi, L., Sica, G., Adamo, S., & Scicchitano, B. M. (2014) · BioMed Research International · doi:10.1155/2014/235426 · Cited by 247 · see citing works
Pro-cognitive properties of T cells
Kipnis, J., Gadani, S., & Derecki, N. C. (2012) · Nature Reviews Immunology · doi:10.1038/nri3280 · Cited by 246 · see citing works
Neurological Disease as a Failure of Brain-Immune Crosstalk: The Multiple Faces of Neuroinflammation
Schwartz, M., & Deczkowska, A. (2016) · Trends in Immunology · doi:10.1016/j.it.2016.08.001 · Cited by 246 · see citing works
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 · see citing works
Systemic inflammatory cells fight off neurodegenerative disease
Schwartz, M., & Shechter, R. (2010) · Nature Reviews Neurology · doi:10.1038/nrneurol.2010.71 · Cited by 240 · see citing works
Nervenkitt: Notes on the history of the concept of neuroglia
Somjen, G. G. (1988) · Glia · doi:10.1002/glia.440010103 · Cited by 233 · see citing works
Gut inflammation and dysbiosis in human motor neuron disease
Rowin, J., Xia, Y., Jung, B., & Sun, J. (2017) · Physiological Reports · doi:10.14814/phy2.13443 · Cited by 224 · see citing works
Mora, J. S., Genge, A. L., Chiò, A., Estol, C. J., Chaverri, D., Hernández, M., Marín, S., Cadavid, J. M., Rodriguez, G. E., Povedano, M., et al. (2019) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2019.1632346 · Cited by 224 · see citing works
Protracted downregulation of CX3CR1 on microglia of aged mice after lipopolysaccharide challenge
Wynne, A. M., Henry, C. J., Huang, Y., Cleland, A., & Godbout, J. P. (2010) · Brain, Behavior, and Immunity · doi:10.1016/j.bbi.2010.05.011 · Cited by 224 · see citing works
IgG Reactivity in the Spinal Cord and Motor Cortex in Amyotrophic Lateral Sclerosis
Engelhardt, J. I., & Appel, S. H. (1990) · Archives of Neurology · doi:10.1001/archneur.1990.00530110068019 · Cited by 221 · see citing works
Papadeas, 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 · see citing works
Lack of Neuronal IFN-β-IFNAR Causes Lewy Body- and Parkinson’s Disease-like Dementia
Ejlerskov, P., Hultberg, J. G., Wang, J., Carlsson, R., Ambjørn, M., Kuss, M., Liu, Y., Porcu, G., Kolkova, K., Friis Rundsten, C., et al. (2015) · Cell · doi:10.1016/j.cell.2015.08.069 · Cited by 211 · see citing works
Hauben, E., Agranov, E., Gothilf, A., Nevo, U., Cohen, A., Smirnov, I., Steinman, L., & Schwartz, M. (2001) · Journal of Clinical Investigation · doi:10.1172/jci12837 · Cited by 208 · see citing works
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 · see citing works
Correlation of Peripheral Immunity With Rapid Amyotrophic Lateral Sclerosis Progression
Murdock, B. J., Zhou, T., Kashlan, S. R., Little, R. J. A., Goutman, S. A., & Feldman, E. L. (2017) · JAMA Neurology · doi:10.1001/jamaneurol.2017.2255 · Cited by 202 · see citing works
Adaptive Immune Neuroprotection in G93A-SOD1 Amyotrophic Lateral Sclerosis Mice
Banerjee, R., Mosley, R. L., Reynolds, A. D., Dhar, A., Jackson-Lewis, V. R., Gordon, P. H., Przedborski, S., & Gendelman, H. E. (2008) · PLoS ONE · doi:10.1371/journal.pone.0002740 · Cited by 198 · see citing works
Neuroinflammation modulates distinct regional and temporal clinical responses in ALS mice
Beers, D. R., Zhao, W., Liao, B., Kano, O., Wang, J., Huang, A., Appel, S. H., & Henkel-Tigges, J. (2010) · Brain Behavior and Immunity · doi:10.1016/j.bbi.2010.12.008 · Cited by 197 · see citing works
Activation of innate and humoral immunity in the peripheral nervous system of ALS transgenic mice
Chiu, I. M., Phatnani, H. P., Kuligowski, M., Tapia, J. C., Carrasco, M. A., Zhang, M., Maniatis, T., & Carroll, M. C. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0911405106 · Cited by 194 · see citing works
Mantovani, S., Garbelli, S., Pasini, A., Alimonti, D., Perotti, C. G., Melazzini, M. G., Bendotti, C., & Mora, G. (2009) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2009.02.012 · Cited by 190 · see citing works
Díaz‐Amarilla, P., Olivera‐Bravo, S., Trías, E., Cragnolini, A. B., Palma, L. M., Cassina, P., Beckman, J. S., & Barbeito, L. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1110689108 · Cited by 186 · see citing works
Autoimmune disease preceding amyotrophic lateral sclerosis: An epidemiologic study
Turner, M. R., Goldacre, R., Ramagopalan, S., Talbot, K., & Goldacre, M. J. (2013) · Neurology · doi:10.1212/wnl.0b013e3182a6cc13 · Cited by 185 · see citing works
Microglial immune checkpoint mechanisms
Deczkowska, A., Amit, I., & Schwartz, M. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0145-x · Cited by 185 · see citing works
Broce, I., Karch, C. M. M., Wen, N., Fan, C. C., Wang, Y., Tan, C. H., Kouri, N., Ross, O. A., Höglinger, G. U., Müller, U., et al. (2018) · PLoS Medicine · doi:10.1371/journal.pmed.1002487 · Cited by 184 · see citing works
Expanded autologous regulatory T-lymphocyte infusions in ALS
Thonhoff, J. R., Beers, D. R., Zhao, W., Pleitez, M. Y., Simpson, E. P., Berry, J. D., Cudkowicz, M., & Appel, S. H. (2018) · Neurology Neuroimmunology & Neuroinflammation · doi:10.1212/nxi.0000000000000465 · Cited by 183 · see citing works
The microglial-motoneuron dialogue in ALS.
Appel, S. H., Zhao, W., Beers, D. R., & Henkel-Tigges, J. (2011) · Europe PMC (PubMed Central) · Cited by 179 · see citing works
Characterization of Gene Expression Phenotype in Amyotrophic Lateral Sclerosis Monocytes
Zhao, W., Beers, D. R., Hooten, K. G., Sieglaff, D. H., Zhang, A., Sundaram, S. K., Traini, C., Halsey, W. S., Hughes, A. M., Sathe, G. M., et al. (2017) · JAMA Neurology · doi:10.1001/jamaneurol.2017.0357 · Cited by 176 · see citing works
Association of Regulatory T-Cell Expansion With Progression of Amyotrophic Lateral Sclerosis
Sheean, R. K., McKay, F. C., Cretney, E., Bye, C. R., Perera, N. D., Tomas, D., Weston, R. A., Scheller, K. J., Djouma, E., Menon, P., et al. (2018) · JAMA Neurology · doi:10.1001/jamaneurol.2018.0035 · Cited by 176 · see citing works
Detection and cellular localization of enterovirus RNA sequences in spinal cord of patients with ALS
Berger, M. M., Kopp, N., Vital, C., Redl, B., Aymard, M., & Lina, B. (2000) · Neurology · doi:10.1212/wnl.54.1.20 · Cited by 174 · see citing works
Gleason, C. E., Ordureau, A., Gourlay, R., Arthur, J. S. C., & Cohen, P. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m111.267567 · Cited by 173 · see citing works
Systemic inflammatory response and neuromuscular involvement in amyotrophic lateral sclerosis
Lu, C., Allen, K., Oei, F., Leoni, E., Kühle, J., Tree, T. I. M., Fratta, P., Sharma, N., Sidle, K. C. L., Howard, R., et al. (2016) · Neurology Neuroimmunology & Neuroinflammation · doi:10.1212/nxi.0000000000000244 · Cited by 172 · see citing works
Garofalo, S., Cocozza, G., Porzia, A., Inghilleri, M., Raspa, M., Scavizzi, F., Aronica, E., Bernardini, G., Peng, L., Ransohoff, R. M., et al. (2020) · Nature Communications · doi:10.1038/s41467-020-15644-8 · Cited by 170 · see citing works
Birger, A., Ben‐Dor, I., Ottolenghi, M., Turetsky, T. T., Gil, Y., Sweetat, S., Perez, L., Belzer, V., Casden, N., Steiner, D., et al. (2019) · EBioMedicine · doi:10.1016/j.ebiom.2019.11.026 · Cited by 166 · see citing works
Uranishi, H., Tetsuka, T., Yamashita, M., Asamitsu, K., Shimizu, M., Itoh, M., & Okamoto, T. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m011176200 · Cited by 158 · see citing works
NF‐κB activation in astrocytes drives a stage‐specific beneficial neuroimmunological response in ALS
Alami, N. O., Schurr, C., Heuvel, F. O., Tang, L., Li, Q., Tasdogan, A., Kimbara, A., Nettekoven, M. H., Ottaviani, G., Raposo, C., et al. (2018) · The EMBO Journal · doi:10.15252/embj.201798697 · Cited by 157 · see citing works
Jean-Martin Charcot: The Father of Neurology
Kumar, D. R., Aslinia, F. M., Yale, S. H., & Mazza, J. J. (2010) · Clinical Medicine & Research · doi:10.3121/cmr.2009.883 · Cited by 156 · see citing works
Evidence for systemic immune system alterations in sporadic amyotrophic lateral sclerosis (sALS)
Zhang, R., Gascon, R., Miller, R. G., Gelinas, D. F., Mass, J., Hadlock, K. G., Jin, X., Reis, J., Narváez, A. B., & McGrath, M. S. (2004) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2004.10.009 · Cited by 155 · see citing works
The fecal microbiome of ALS patients
Brenner, D., Hiergeist, A., Adis, C. I., Mayer, B., Gessner, A., Ludolph, A. C., & Weishaupt, J. H. (2017) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2017.09.023 · Cited by 155 · 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
A prospective longitudinal study on the microbiota composition in amyotrophic lateral sclerosis
Di Gioia, D., Bozzi Cionci, N., Baffoni, L., Amoruso, A., Pane, M., Mogna, L., Gaggìa, F., Lucenti, M. A., Bersano, E., Cantello, R., et al. (2020) · BMC Medicine · doi:10.1186/s12916-020-01607-9 · Cited by 151 · see citing works
Self-citationElevated IL-6 and TNF-α levels in patients with ALS: Inflammation or hypoxia?
Moreau, C., Devos, D., Danel-Brunaud, V., Defebvre, L., Perez, T., Destée, A., Tonnel, A., Lassalle, P., & Just, N. (2005) · Neurology · doi:10.1212/01.wnl.0000188907.97339.76 · Cited by 146 · see citing works
Retroviruses and amyotrophic lateral sclerosis
Alfahad, T. B., & Nath, A. (2013) · Antiviral Research · doi:10.1016/j.antiviral.2013.05.006 · Cited by 144 · see citing works
Pompl, P. N., Ho, L., Bianchi, M., McManus, T. E., Qin, W., & Pasinetti, G. M. (2003) · The FASEB Journal · doi:10.1096/fj.02-0876fje · Cited by 144 · see citing works
Innate and adaptive immunity in amyotrophic lateral sclerosis: Evidence of complement activation
Sta, M., Sylva-Steenland, R. M. R., Casula, M., de Jong, J. M. B. V., Troost, D., Aronica, E., & Baas, F. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.01.002 · Cited by 143 · see citing works
Chronic exposure to TGFβ1 regulates myeloid cell inflammatory response in an IRF7-dependent manner
Cohen, M., Matcovitch, O., David, E., Barnett‐Itzhaki, Z., Keren‐Shaul, H., Blecher‐Gonen, R., Jaitin, D. A., Sica, A., Amit, I., & Schwartz, M. (2014) · The EMBO Journal · doi:10.15252/embj.201489293 · Cited by 142 · see citing works
Hematopoietic stem cell transplantation in patients with sporadic amyotrophic lateral sclerosis
Appel, S. H., Engelhardt, J. I., Henkel-Tigges, J., Siklós, L., Beers, D. R., Yen, A. A., Simpson, E. P., Luo, Y., Carrum, G., Heslop, H., et al. (2008) · Neurology · doi:10.1212/01.wnl.0000327668.43541.22 · Cited by 140 · see citing works
Astroglia In Neurological Diseases
Verkhratsky, A., Rodríguez, J. J., & Parpura, V. (2013) · Future Neurology · doi:10.2217/fnl.12.90 · Cited by 140 · see citing works
Mast cells and neutrophils mediate peripheral motor pathway degeneration in ALS
Trías, E., King, P. H., Si, Y., Kwon, Y., Varela, V., Ibarburu, S., Kovacs, M., Moura, I. C., Beckman, J. S., Hermine, O., et al. (2018) · JCI Insight · doi:10.1172/jci.insight.123249 · Cited by 139 · see citing works
Oeckl, P., Weydt, P., Steinacker, P., Anderl-Straub, S., Nordin, F., Volk, A. E., Diehl-Schmid, J., Andersen, P. M., Kornhuber, J., Danek, A., et al. (2019) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2018-318868 · Cited by 139 · see citing works
Zhao, W., Beers, D. R., Liao, B., Henkel-Tigges, J., & Appel, S. H. (2012) · Neurobiology of Disease · doi:10.1016/j.nbd.2012.07.008 · Cited by 137 · see citing works
Coque, E., Salsac, C., Espinosa-Carrasco, G., Varga, B., Degauque, N., Cadoux, M., Crabé, R., Virenque, A., Soulard, C., Fierle, J. K., et al. (2019) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1815961116 · Cited by 137 · see citing works
Schludi, M. H., Becker, L., Garrett, L., Gendron, T. F., Zhou, Q., Schreiber, F., Popper, B., Dimou, L., Strom, T. M., Winkelmann, J., et al. (2017) · Acta Neuropathologica · doi:10.1007/s00401-017-1711-0 · Cited by 131 · see citing works
Cerebrospinal fluid macrophage biomarkers in amyotrophic lateral sclerosis
Thompson, A. G., Gray, E., Thézénas, M., Charles, P. D., Evetts, S., Hu, M. T., Talbot, K., Fischer, R., Kessler, B. M., & Turner, M. R. (2018) · Annals of Neurology · doi:10.1002/ana.25143 · Cited by 130 · see citing works
Steinacker, P., Verde, F., Fang, L., Feneberg, E., Oeckl, P., Roeber, S., Anderl-Straub, S., Danek, A., Diehl-Schmid, J., Fassbender, K., et al. (2018) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2017-317138 · Cited by 129 · see citing works
Jin, M., Günther, R., Akgün, K., Hermann, A., & Ziemssen, T. (2020) · Scientific Reports · doi:10.1038/s41598-020-62756-8 · Cited by 128 · see citing works
The brain’s best friend: microglial neurotoxicity revisited
Hellwig, S., Heinrich, A., & Biber, K. (2013) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2013.00071 · Cited by 126 · see citing works
Graber, D. J., Hickey, W. F., & Harris, B. T. (2010) · Journal of Neuroinflammation · doi:10.1186/1742-2094-7-8 · Cited by 121 · see citing works
Manghera, M., Ferguson-Parry, J., Lin, R., & Douville, R. N. (2016) · Journal of Virology · doi:10.1128/jvi.01503-16 · Cited by 118 · see citing works
IFNβ secreted by microglia mediates clearance of myelin debris in CNS autoimmunity
Kocur, M., Schneider, R., Pulm, A.-K., Bauer, J., Kropp, S., Gliem, M., Ingwersen, J., Goebels, N., Alferink, J., Prozorovski, T., et al. (2015) · Acta Neuropathologica Communications · doi:10.1186/s40478-015-0192-4 · Cited by 117 · see citing works
Gravel, M., Béland, L.-C., Soucy, G., Abdelhamid, E., Rahimian, R., Gravel, C., & Kriz, J. (2016) · The Journal of Neuroscience · doi:10.1523/jneurosci.0854-15.2016 · Cited by 117 · see citing works
Self-citationAppel, S. H., Engelhardt, J. I., García, J., & Stefani, E. (1991) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.88.2.647 · Cited by 116 · see citing works
Lincecum, J. M., Vieira, F. G., Wang, M. Z., Thompson, K., Zutter, G. S. D., Kidd, J. D., Moreno, A., Sànchez, R., Carrion, I. J., Levine, B. A., et al. (2010) · Nature Genetics · doi:10.1038/ng.557 · Cited by 114 · see citing works
Inflammation Induces TDP-43 Mislocalization and Aggregation
Correia, A. S., Patel, P., Dutta, K., & Julien, J.-P. (2015) · PLOS ONE · doi:10.1371/journal.pone.0140248 · Cited by 114 · see citing works
Addressing heterogeneity in amyotrophic lateral sclerosis CLINICAL TRIALS
Goyal, N., Berry, J. D., Windebank, A. J., Staff, N. P., Maragakis, N. J., van den Berg, L. H., Genge, A. L., Miller, R. G., Baloh, R. H., Kern, R. Z., et al. (2020) · Muscle & Nerve · doi:10.1002/mus.26801 · Cited by 111 · see citing works
The Role of Intrinsic Pathway in Apoptosis Activation and Progression in Peyronie’s Disease
Loreto, C., La Rocca, G., Anzalone, R., Caltabiano, R., Vespasiani, G., Castorina, S., Ralph, D. J., Cellek, S., Musumeci, G., Giunta, S., et al. (2014) · BioMed Research International · doi:10.1155/2014/616149 · Cited by 108 · see citing works
Benyamin, B., He, J., Zhao, Q., Gratten, J., Garton, F. C., Leo, P. J., Liu, Z., Mangelsdorf, M., Al‐Chalabi, A., Anderson, L. K., et al. (2017) · Nature Communications · doi:10.1038/s41467-017-00471-1 · Cited by 107 · see citing works
Solomon, J. N., Lewis, C. B., Ajami, B., Corbel, S. Y., Rossi, F. M. V., & Krieger, C. (2006) · Glia · doi:10.1002/glia.20331 · Cited by 105 · see citing works
Kunis, G., Baruch, K., Miller, O., & Schwartz, M. (2015) · The Journal of Neuroscience · doi:10.1523/jneurosci.3644-14.2015 · Cited by 105 · see citing works
Humoral factors in ALS patients during disease progression
Ehrhart, J. C., de Smith, A. J., Kuzmin‐Nichols, N., Zesiewicz, T., Jahan, I., Shytle, R. D., Kim, S.-H., Sanberg, C. D., Vu, T., Gooch, C. L., et al. (2015) · Journal of Neuroinflammation · doi:10.1186/s12974-015-0350-4 · Cited by 104 · see citing works
Roberts, K., Zeineddine, R., Corcoran, L., Li, W., Campbell, I. L., & Yerbury, J. J. (2012) · Glia · doi:10.1002/glia.22444 · Cited by 102 · see citing works
Gerbino, V., Kaunga, E., Ye, J., Canzio, D., O’Keeffe, S., Rudnick, N. D., Guarnieri, P., Lutz, C. M., & Maniatis, T. (2020) · Neuron · doi:10.1016/j.neuron.2020.03.005 · Cited by 102 · see citing works
Lymphocytic infiltration in the spinal cord of patients with amyotrophic lateral sclerosis.
Troost, D., van den Oord, J. J., de Jong, J. M. B. V., & Swaab, D. F. (1989) · PubMed · Cited by 101 · see citing works
Kia, A., McAvoy, K. J., Krishnamurthy, K., Trotti, D., & Pasinelli, P. (2018) · Glia · doi:10.1002/glia.23298 · Cited by 100 · see citing works
Keller, A. F., Gravel, M., & Križ, J. (2010) · Experimental Neurology · doi:10.1016/j.expneurol.2010.12.010 · Cited by 99 · see citing works
Crohn’s Disease: an Immune Deficiency State
Marks, D. J., Rahman, F. Z., Sewell, G. W., & Segal, A. W. (2009) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-009-8133-2 · Cited by 97 · see citing works
Enteroviral Infection: The Forgotten Link to Amyotrophic Lateral Sclerosis?
Xue, Y. C., Feuer, R., Cashman, N. R., & Luo, H. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00063 · Cited by 97 · see citing works
Alshikho, M. J., Zürcher, N. R., Loggia, M. L., Cernasov, P., Reynolds, B., Pijanowski, O., Chonde, D. B., Izquierdo‐Garcia, D., Mainero, C., Catana, C., et al. (2018) · Annals of Neurology · doi:10.1002/ana.25251 · Cited by 97 · see citing works
Optineurin suppression causes neuronal cell death via <scp>NF</scp>‐κB pathway
Akizuki, M., Yamashita, H., Uemura, K., Maruyama, H., Kawakami, H., Ito, H., & Takahashi, R. (2013) · Journal of Neurochemistry · doi:10.1111/jnc.12326 · Cited by 96 · see citing works
Kang, J., & Rivest, S. (2007) · The Journal of Cell Biology · doi:10.1083/jcb.200705046 · Cited by 94 · see citing works
Markovinović, A., Cimbro, R., Ljutić, T., Križ, J., Rogelj, B., & Munitić, I. (2017) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2017.04.005 · Cited by 94 · see citing works
Lee, J. D., Kamaruzaman, N. A., Fung, J. N., Taylor, S. M., Turner, B. J., Atkin, J. D., Woodruff, T. M., & Noakes, P. G. (2013) · Journal of Neuroinflammation · doi:10.1186/1742-2094-10-119 · Cited by 93 · see citing works
Pain in Amyotrophic Lateral Sclerosis: A Neglected Aspect of Disease
Handy, C. R., Krudy, C. A., Boulis, N. M., & Federici, T. (2011) · Neurology Research International · doi:10.1155/2011/403808 · Cited by 92 · see citing works
Biomarkers in Neurodegenerative Diseases
Jeromin, A., & Bowser, R. (2017) · Advances in Neurobiology · doi:10.1007/978-3-319-57193-5_20 · Cited by 92 · see citing works
Disease origin and progression in amyotrophic lateral sclerosis: an immunology perspective
Malaspina, A., Puentes, F., & Amor, S. (2014) · International Immunology · doi:10.1093/intimm/dxu099 · Cited by 90 · see citing works
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Role of neuroinflammation in neurodegeneration development
Zhang, W., Xiao, D., Mao, Q., & Xia, H. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01486-5 · Cited by 1,668 · see citing works
“Many of the genetic mutations are associated with immune response, such as C9orf72, TBK1, CYLD, OPTN, PGRN, and so on.(85,272) Most of them were suggested to induce mitochondrial impairment, which further led to oxidative stress and inflammation.”
BackgroundHop, P. J., Zwamborn, R. A. J., Hannon, E. J., Shireby, G. L., Nabais, M. F., Walker, E., van Rheenen, W., van Vugt, J. J. F. A., Dekker, A. M., Westeneng, H., et al. (2022) · Science Translational Medicine · doi:10.1126/scitranslmed.abj0264 · Cited by 102 · see citing works
The Gut Microbiota-Immunity Axis in ALS: A Role in Deciphering Disease Heterogeneity?
Niccolai, E., Pilato, V. D., Nannini, G., Baldi, S., Russo, E., Zucchi, E., Martinelli, I., Menicatti, M., Bartolucci, G., Mandrioli, J., et al. (2021) · Biomedicines · doi:10.3390/biomedicines9070753 · Cited by 66 · see citing works
Immune mechanisms and shared immune targets in neurodegenerative diseases
Weiner, H. L. (2024) · Nature Reviews Neurology · doi:10.1038/s41582-024-01046-7 · Cited by 63 · see citing works
Garofalo, S., Cocozza, G., Bernardini, G., Savage, J. C., Raspa, M., Aronica, E., Tremblay, M., Ransohoff, R. M., Santoni, A., & Limatola, C. (2022) · Brain Behavior and Immunity · doi:10.1016/j.bbi.2022.06.004 · Cited by 59 · see citing works
Interplay between immunity and amyotrophic lateral sclerosis: Clinical impact
Marchi, F. D., Munitić, I., Amedei, A., Berry, J. D., Feldman, E. L., Aronica, E., Nardo, G., Weehaeghe, D. V., Niccolai, E., Prtenjača, N., et al. (2021) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2021.06.027 · Cited by 52 · see citing works
Self-citationKuntić, M., Hahad, O., Münzel, T. F., & Daiber, A. (2024) · Antioxidants · doi:10.3390/antiox13030266 · Cited by 50 · see citing works
Overlapping Neuroimmune Mechanisms and Therapeutic Targets in Neurodegenerative Disorders
Marchi, F. D., Munitić, I., Vidatic, L., Papić, E., Rački, V., Nimac, J., Jurak, I., Novotni, G., Rogelj, B., Vuletić, V., et al. (2023) · Biomedicines · doi:10.3390/biomedicines11102793 · Cited by 46 · see citing works
Self-citationInflammatory signature in amyotrophic lateral sclerosis predicting disease progression
Femiano, C., Bruno, A., Gilio, L., Buttari, F., Dolcetti, E., Galifi, G., Azzolini, F., Borrelli, A., Furlan, R., Finardi, A., et al. (2024) · Scientific Reports · doi:10.1038/s41598-024-67165-9 · Cited by 34 · see citing works
Gut microbiota immune cross-talk in amyotrophic lateral sclerosis
Kaul, M., Mukherjee, D., Weiner, H. L., & Cox, L. M. (2024) · Neurotherapeutics · doi:10.1016/j.neurot.2024.e00469 · Cited by 35 · see citing works
Boraschi, D., Italiani, P., Migliorini, P., & Bossù, P. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1128190 · Cited by 33 · see citing works
Jin, M., Akgün, K., Ziemssen, T., Kipp, M., Günther, R., & Hermann, A. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22158042 · Cited by 32 · see citing works
Emerging Trends in the Field of Inflammation and Proteinopathy in ALS/FTD Spectrum Disorder
Marchi, F. D., Franjkic, T., Schito, P., Russo, T., Nimac, J., Chami, A. A., Mele, A., Vidatic, L., Križ, J., Julien, J., et al. (2023) · Biomedicines · doi:10.3390/biomedicines11061599 · Cited by 28 · see citing works
Self-citationBoeri, L., Perottoni, S., Izzo, L., Giordano, C., & Albani, D. (2021) · Advanced Healthcare Materials · doi:10.1002/adhm.202002043 · Cited by 28 · see citing works
The blood biomarkers puzzle – A review of protein biomarkers in neurodegenerative diseases
Barro, C., & Zetterberg, H. (2021) · Journal of Neuroscience Methods · doi:10.1016/j.jneumeth.2021.109281 · Cited by 29 · see citing works
Trolese, M. C., Scarpa, C., Melfi, V., Fabbrizio, P., Sironi, F. M., Rossi, M., Bendotti, C., & Nardo, G. (2022) · Molecular Therapy · doi:10.1016/j.ymthe.2022.04.018 · Cited by 29 · see citing works
Neuroinflammatory Pathways in the ALS-FTD Continuum: A Focus on Genetic Variants
Marchi, F. D., Tondo, G., Corrado, L., Menegon, F., Aprile, D., Anselmi, M., D’Alfonso, S., Comi, C., & Mazzini, L. (2023) · Genes · doi:10.3390/genes14081658 · Cited by 28 · see citing works
Self-citationAlarcan, H., Chaumond, R., Emond, P., Bretagne, I. B., Lefèvre, A., Bakkouche, S. E., Veyrat‐Durebex, C., Vourc’h, P., Andres, C. R., Corcia, P., et al. (2021) · Biomolecules · doi:10.3390/biom11050691 · Cited by 26 · see citing works
How Inflammation Pathways Contribute to Cell Death in Neuro-Muscular Disorders
Salucci, S., Stella, A. B., Battistelli, M., Burattini, S., Bavelloni, A., Cocco, L., Gobbi, P., & Faenza, I. (2021) · Biomolecules · doi:10.3390/biom11081109 · Cited by 24 · see citing works
Zaccai, S., Nemirovsky, A., Lerner, L., Alfahel, L., Eremenko, E., Israelson, A., & Monsonego, A. (2024) · Journal of Neuroinflammation · doi:10.1186/s12974-023-03007-1 · Cited by 23 · see citing works
HPLC-MS/MS Oxylipin Analysis of Plasma from Amyotrophic Lateral Sclerosis Patients
Mastrogiovanni, M., Trostchansky, A., Naya, H., Dominguez, R., Marco, C., Povedano, M., López‐Vales, R., & Rubbo, H. (2022) · Biomedicines · doi:10.3390/biomedicines10030674 · Cited by 20 · see citing works
Luo, S., Pan, M., Fu, G., Meng, X., Zhao, Z., Yu, H., Yue, G.-X., & Lei, T. (2025) · Pharmacological Research · doi:10.1016/j.phrs.2025.107974 · Cited by 19 · see citing works
Aberrant NLRP3 Inflammasome Activation Ignites the Fire of Inflammation in Neuromuscular Diseases
Péladeau, C. L., & Sandhu, J. K. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22116068 · Cited by 20 · see citing works
Peripheral Immune Profiles Predict ALS Progression in an Age- and Sex-Dependent Manner
Murdock, B. J., Zhao, B., Pawlowski, K. D., Famie, J. P., Piecuch, C. E., Webber‐Davis, I. F., Teener, S. J., Feldman, E. L., Zhao, L., & Goutman, S. A. (2024) · Neurology Neuroimmunology & Neuroinflammation · doi:10.1212/nxi.0000000000200241 · Cited by 19 · see citing works
Clinical Safety and Preliminary Efficacy of Regulatory T Cells for ALS
Shneider, N. A., Nesta, A. V., Rifai, O. M., Yasek, J. E., Elyaman, W., Aziz‐Zaman, S., Lyu, M.-A., Lévy, S., Hoover, B. N., Vlad, G., et al. (2025) · NEJM Evidence · doi:10.1056/evidoa2400249 · Cited by 18 · see citing works
Ravnik‐Glavač, M., Goričar, K., Vogrinc, D., Koritnik, B., Lavrenčič, J. G., Glavač, D., & Dolžan, V. (2022) · Genes · doi:10.3390/genes13050757 · Cited by 18 · see citing works
Effects of exercise on neuroinflammation in age-related neurodegenerative disorders
Su, Y., & Su, Z. (2025) · European journal of medical research · doi:10.1186/s40001-025-03165-3 · Cited by 18 · see citing works
Ren, J., Zhang, Y., Pan, H., Shi, R., Zhu, H., Yang, R., Zhang, L., Chen, B., Zhu, T., Lu, X., et al. (2023) · International Immunopharmacology · doi:10.1016/j.intimp.2023.111405 · Cited by 17 · see citing works
Cennamo, G., Montorio, D., Ausiello, F. P., Magno, L., Iodice, R., Mazzucco, A., Iuzzolino, V. V., Senerchia, G., Morra, V. B., Nolano, M., et al. (2022) · Biomedicines · doi:10.3390/biomedicines10102390 · Cited by 17 · see citing works
Alarcan, H., Ojaimi, Y. A., Lanznaster, D., Escoffre, J., Corcia, P., Vourc’h, P., Andres, C. R., Veyrat‐Durebex, C., & Blasco, H. (2022) · Journal of Personalized Medicine · doi:10.3390/jpm12071071 · Cited by 16 · see citing works
Kim, H. J., Ban, J.-J., Kang, J., Im, H. W., Ko, S. H., Sung, J., Park, S., Park, J.-E., & Choi, S. J. (2024) · Brain Communications · doi:10.1093/braincomms/fcae428 · Cited by 15 · see citing works
Dysbiosis and Neurodegeneration in ALS: Unraveling the Gut–Brain Axis
Sharma, V. (2025) · NeuroMolecular Medicine · doi:10.1007/s12017-025-08870-0 · Cited by 14 · see citing works
Quantum Biology Research Meets Pathophysiology and Therapeutic Mechanisms: A Biomedical Perspective
Calvillo, L., Redaelli, V., Ludwig, N. G., Qaswal, A. B., Ghidoni, A., Faini, A., Rosa, D., Lombardi, C., Pengo, M. F., Bossolasco, P., et al. (2022) · Quantum Reports · doi:10.3390/quantum4020011 · Cited by 15 · see citing works
Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels
Prtenjača, N., Rob, M., Alam, M. S., Markovinović, A., Stuani, C., Buratti, E., & Munitić, I. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23126829 · Cited by 14 · see citing works
Barreto‐Núñez, R., Béland, L., Boutej, H., Picher‐Martel, V., Dupré, N., Barbeito, L., & Križ, J. (2024) · Glia · doi:10.1002/glia.24531 · Cited by 15 · see citing works
Self-citationPalumbo, M. L., Moroni, A. D., Quiroga, S., Castro, M. M., Burgueño, A. L., & Genaro, A. M. (2021) · Pharmacology Research & Perspectives · doi:10.1002/prp2.795 · Cited by 12 · see citing works
Tsang, V. S. K., Malaspina, A., & Henson, S. M. (2025) · Journal of Inflammation · doi:10.1186/s12950-025-00460-y · Cited by 13 · see citing works
The Cellular and Molecular Signature of ALS in Muscle
Anakor, E., Duddy, W. J., & Duguez, S. (2022) · Journal of Personalized Medicine · doi:10.3390/jpm12111868 · Cited by 13 · see citing works
C9orf72 repeat expansion creates the unstable folate-sensitive fragile site FRA9A
Mirceta, M., Schmidt, M. H. M., Shum, N., Prasolava, T., Meikle, B., Lanni, S., Mohiuddin, M., McKeever, P. M., Zhang, M., Liang, M., et al. (2024) · NAR Molecular Medicine · doi:10.1093/narmme/ugae019 · Cited by 13 · see citing works
Urinary biomarkers for amyotrophic lateral sclerosis: candidates, opportunities and considerations
Rogers, M., Schultz, D. W., Karnaros, V., & Shepheard, S. R. (2023) · Brain Communications · doi:10.1093/braincomms/fcad287 · Cited by 13 · see citing works
The CCL2-CCR2 axis drives neuromuscular denervation in amyotrophic lateral sclerosis
Nógrádi, B., Molnár, K., Kristóf, R., Horváth, O., Huang, Y., Ridgway, Z., Elicegui, A., Fuertes-Álvarez, S., Alonso‐Martín, S., Szebeni, G. J., et al. (2025) · Nature Communications · doi:10.1038/s41467-025-62351-3 · Cited by 12 · see citing works
Contingent intramuscular boosting of P2XR7 axis improves motor function in transgenic ALS mice
Fabbrizio, P., D’Agostino, J., Margotta, C., Mella, G., Panini, N., Pasetto, L., Sammali, E., Raggi, F., Sorarú, G., Bonetto, V., et al. (2021) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-021-04070-8 · Cited by 12 · see citing works
Rubino, V., Palatucci, A. T., Rosa, G. L., Giovazzino, A., Aruta, F., Damiano, S., Carriero, F., Santillo, M., Iodice, R., Mondola, P., et al. (2021) · Antioxidants · doi:10.3390/antiox10121940 · Cited by 12 · see citing works
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 · see citing works
Marchi, F. D., Collo, A., Scognamiglio, A., Cavaletto, M., Cionci, N. B., Biroli, G., Gioia, D. D., Riso, S., & Mazzini, L. (2021) · Nutrition · doi:10.1016/j.nut.2021.111525 · Cited by 11 · see citing works
Self-citationFrolov, A., Guzmán, M. A., Hayat, G. R., & Martin, J. R. (2024) · Cureus · doi:10.7759/cureus.56023 · Cited by 10 · see citing works
Mazzini, L., Marchi, F. D., Niccolai, E., Mandrioli, J., & Amedei, A. (2021) · Exon Publications eBooks · doi:10.36255/exonpublications.amyotrophiclateralsclerosis.microbiota.2021 · Cited by 10 · see citing works
Self-citationDistinct Plasma Immune Profile in ALS Implicates sTNFR-II in pAMPK/Leptin Homeostasis
Picher‐Martel, V., Boutej, H., Vézina, A., Cordeau, P., Kaneb, H., Julien, J., Genge, A. L., Dupré, N., & Križ, J. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24065065 · Cited by 10 · see citing works
The multifaceted role of the CXC chemokines and receptors signaling axes in ALS pathophysiology
Cognata, V. L., Morello, G., Guarnaccia, M., & Cavallaro, S. (2024) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2024.102587 · Cited by 9 · see citing works
Marchi, F. D., Lombardi, I., Bombaci, A., Diamanti, L., Olivero, M., Perciballi, E., Tornabene, D., Vulcano, E., Ferrari, D., & Mazzini, L. (2025) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2025.2508781 · Cited by 9 · see citing works
Self-citationBertoni, G., Ristori, S., & Monti, D. (2026) · Cells · doi:10.3390/cells15080657 · Cited by 9 · see citing works
Causal ALS genes impact the MHC class II antigen presentation pathway
Chi, B., Öztürk, M. M., Paraggio, C. L., Leonard, C. E., Sanita, M. E., Dastpak, M., O’Connell, J. D., Coady, J. A., Zhang, J., Gygi, S. P., et al. (2023) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2305756120 · Cited by 9 · see citing works
Imaging immunological processes from blood to brain in amyotrophic lateral sclerosis
Amor, S., Nutma, E., Marzin, M. C., & Puentes, F. (2021) · Clinical & Experimental Immunology · doi:10.1111/cei.13660 · Cited by 8 · see citing works
Wang, T., Li, R., Niu, P., Wei, Z., Xie, D., Huang, H., Pan, J., & Rong, C. (2025) · Journal of Functional Foods · doi:10.1016/j.jff.2025.106676 · Cited by 8 · see citing works
Polgár, T. F., Meszlényi, V., Nógrádi, B., Körmöczy, L., Spisák, K., Tripolszki, K., Széll, M., Obál, I., Engelhardt, J. I., Siklós, L., et al. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22189994 · Cited by 8 · see citing works
The Neonatal Microbiome: Implications for Amyotrophic Lateral Sclerosis and Other Neurodegenerations
Eisen, A., & Kiernan, M. C. (2025) · Brain Sciences · doi:10.3390/brainsci15020195 · Cited by 7 · see citing works
Pham, T. K., Verber, N. S., Turner, M. R., Malaspina, A., Collins, M. O., Mead, R. J., & Shaw, P. J. (2025) · Translational Neurodegeneration · doi:10.1186/s40035-025-00496-3 · Cited by 7 · see citing works
The dual role of microglia in neurodegenerative diseases: A review
Zhang, N., Xiong, N., He, Y., & Zhou, L. (2026) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2026.111887 · Cited by 8 · see citing works
Therapeutic Approaches to Amyotrophic Lateral Sclerosis from the Lab to the Clinic
Chavda, V. P., Patel, C. A., Modh, D. H., Ertaş, Y. N., Sonak, S. S., Munshi, N. K., Anand, K., Soni, A., & Pande, S. V. (2022) · Current Drug Metabolism · doi:10.2174/1389200223666220310113110 · Cited by 8 · see citing works
Peradinović, J., Mohovic, N., Bulic, K., Markovinović, A., Cimbro, R., & Munitić, I. (2023) · Biology · doi:10.3390/biology12020240 · Cited by 6 · see citing works
Hop, P. J., Zwamborn, R., Hannon, E., Shireby, G., Nabais, M. F., Walker, E., van Rheenen, W., van Vugt, J. V., Dekker, A. M., Westeneng, H., et al. (2021) · medRxiv · doi:10.1101/2021.03.12.21253115 · Cited by 9 · see citing works
Adenosine deaminase, not immune to a mechanistic rethink in central nervous system disorders?
Hall, B., George, J. G., & Allen, S. P. (2022) · PubMed · doi:10.14670/hh-18-404 · Cited by 6 · see citing works
Gold, N. D., Mallard, A. J., Hermann, J. C., Zeifman, R. J., Pagni, B. A., Bogenschutz, M. P., & Ross, S. (2023) · Journal of Palliative Medicine · doi:10.1089/jpm.2022.0604 · Cited by 6 · see citing works
Neuroimmune characterization of optineurin insufficiency mouse model during ageing
Mohovic, N., Peradinović, J., Markovinović, A., Cimbro, R., Minić, Ž., Dominović, M., Jakovac, H., Nimac, J., Rogelj, B., & Munitić, I. (2023) · Scientific Reports · doi:10.1038/s41598-023-38875-3 · Cited by 6 · see citing works
Self-citationAssociation of serum Spp1 levels with disease progression in ALS and SBMA
Ju, W., Ban, J.-J., Im, H.-R., Ko, S.-H., Seo, J., Min, Y., Hong, Y.-H., Choi, S.-J., & Sung, J. (2024) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.52087 · Cited by 8 · see citing works
Ratano, P., Cocozza, G., Pinchera, C., Busdraghi, L. M., Cantando, I., Martinello, K., Scioli, M., Rosito, M., Bezzi, P., Fucile, S., et al. (2024) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2023.1333745 · Cited by 5 · see citing works
Moțățăianu, A., Andone, S. R., Stoian, A., Bălașa, R., Huţanu, A., & Sărmășan, E. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25073782 · Cited by 5 · see citing works
CHCHD10P80L knock-in zebrafish display a mild ALS-like phenotype
Légaré, V. P., Harji, Z. A., Rampal, C. J., Antonická, H., Gurberg, T. J. N., Persia, O., Rodríguez, E. C., Shoubridge, E. A., & Armstrong, G. A. B. (2024) · Experimental Neurology · doi:10.1016/j.expneurol.2024.114945 · Cited by 5 · see citing works
Brain macrophages and pial fibroblasts promote inflammation in a hypomyelination model
Okuno, N., Yamamoto, S., Hamashima, T., Tung, D. S., Okita, N., Fujikawa, M., Kunisawa, T., Takakura, N., Fujimori, T., Mori, H., et al. (2025) · Acta Neuropathologica Communications · doi:10.1186/s40478-025-02063-3 · Cited by 4 · see citing works
Tofacitinib extends survival in a mouse model of ALS through NK cell-independent mechanisms
Baird, L. A., Teener, S. J., Webber‐Davis, I. F., Carter, A., Huang, F., Jang, D., Famie, J. P., Piecuch, C. E., Guo, K. X., Feldman, E. L., et al. (2025) · Frontiers in Immunology · doi:10.3389/fimmu.2025.1662197 · Cited by 4 · see citing works
Tamadon, A., Afshar, A., Mussin, N. M., Zhilisbayeva, K. R., Kurmanalina, M. A., Baspakova, A. M., Aringazina, R. A., Mahdipour, M., Tanideh, N., Khoradmehr, A., et al. (2025) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.5c00533 · Cited by 4 · see citing works
Sex-aware causal inference assessment of the immune system in complex neurodegenerative diseases
Lona‐Durazo, F., Ross, B., Pilon, M., Greicius, M. D., Dubé, M., Belloy, M. E., McLaughlin, R. L., & Taliun, S. A. G. (2025) · Brain · doi:10.1093/brain/awaf474 · Cited by 4 · see citing works
Spiteri, A. G., Steele, J. R., Lee, H.-C., Zhang, H., Sun, J., Schittenhelm, R. B., Yu, C., McLean, C. A., Masters, C. L., Goudey, B., et al. (2025) · Scientific Reports · doi:10.1038/s41598-025-11466-0 · Cited by 3 · see citing works
Xu, L., Bin, H., Zhou, Y., Liao, X., Chen, T., & He, H. (2025) · BMC Pharmacology and Toxicology · doi:10.1186/s40360-025-01024-9 · Cited by 3 · see citing works
Dual effects of TGF ‐β inhibitor in ALS ‐ inhibit contracture and neurodegeneration
Lee, D., Kwon, Y. N., Lee, K., Kim, S. J., & Sung, J. (2024) · Journal of Neurochemistry · doi:10.1111/jnc.16102 · Cited by 3 · see citing works
Hemizygous Granzyme A Mice Expressing the hSOD1G93A Transgene Show Slightly Extended Lifespan
Moreno‐Martínez, L., Santiago, L., de la Torre, M., Calvo, A. C., Pardo, J., & Osta, R. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms232113554 · Cited by 3 · see citing works
Peptide‐based Therapeutics for Inflammatory and Autoimmune Diseases
Chankeshwara, S. V., Czechtizky, W., & Su, W. (2025) · Peptide Science · doi:10.1002/9781119824701.ch10 · Cited by 3 · see citing works
Scholl, L.-S., Demleitner, A. F., Riedel, J., Adachi, S., Neuenroth, L., Meijs, C., Tzeplaeff, L., Gomes, L. C., Galhoz, A., Cordts, I., et al. (2025) · Acta Neuropathologica Communications · doi:10.1186/s40478-025-02109-6 · Cited by 2 · see citing works
Regulatory T cell α(1,3)-exofucosylation for treatment of neurodegenerative diseases
Gendelman, H. E., Hu, G., Yeapuri, P., Srivastava, S., Mosley, R. L., Mamilla, S., Intrator, W., Are, M. S., & Sackstein, R. (2025) · Journal of Leukocyte Biology · doi:10.1093/jleuko/qiaf154 · Cited by 2 · see citing works
Repurposing immunomodulatory drugs targeting microglia for amyotrophic lateral sclerosis
Maes, K. J. H., & Briedé, J. J. (2025) · Brain Research · doi:10.1016/j.brainres.2025.150032 · Cited by 3 · see citing works
Zhao, H., Liu, Y., & Chen, X. (2026) · Journal of Neuroinflammation · doi:10.1186/s12974-026-03709-2 · Cited by 2 · see citing works
Alarcan, H., Berthet, M., Suire, L., Colas, C., Gonzalez, L., Paget, C., Bretagne, I. B., Piver, É., Vourc’h, P., Andres, C. R., et al. (2022) · SLAS TECHNOLOGY · doi:10.1016/j.slast.2022.07.003 · Cited by 2 · see citing works
Karafoulidou, E., Kesidou, E., Theotokis, P., Konstantinou, C., Nella, M., Michailidou, I., Touloumi, O., Polyzoidou, E., Salamotas, I., Einstein, O., et al. (2024) · Cells · doi:10.3390/cells13090785 · Cited by 2 · see citing works
Yang, J.-L., Wu, J.-Y., Liu, J., & Zheng, G. (2024) · Frontiers in Immunology · doi:10.3389/fimmu.2024.1433929 · Cited by 2 · see citing works
A Step in the ALS Direction: Lessons from the Purinome
Volonté, C., Yerbury, J. J., & Sluyter, R. (2023) · Springer eBooks · doi:10.1007/978-3-031-26945-5_10 · Cited by 2 · see citing works
Insulin in Amyotrophic Lateral Sclerosis: Impairment, Tests and TreatmentPreprint
Rappoport, A. (2023) · Preprints.org · doi:10.20944/preprints202212.0297.v3 · Cited by 2 · see citing works
Muscle fatigue and exercise-related biomarkers in amyotrophic lateral sclerosis
Bianchi, F., Becattini, L., Chico, L., Ricci, G., & Siciliano, G. (2023) · Exploration of Neuroprotective Therapy · doi:10.37349/ent.2023.00045 · Cited by 1 · see citing works
Deecke, L., Ohlei, O., Goldeck, D., Homann, J., Toepfer, S., Demuth, I., Bertram, L., Pawelec, G., & Lill, C. M. (2025) · Cells · doi:10.3390/cells14040250 · Cited by 1 · see citing works
Tosi, M., Favero, F., Zuccalà, M., Visha, E., Caushi, F., Barizzone, N., Pomella, N., Follia, L., Corrado, L., Corà, D., et al. (2026) · Neurological Sciences · doi:10.1007/s10072-026-08813-y · Cited by 1 · see citing works
Mendon, A., Jain, S., Mishra, N., & Parab, S. B. (2026) · Revue Neurologique · doi:10.1016/j.neurol.2026.02.148 · Cited by 1 · see citing works
The Gut Microbiome in Amyotrophic Lateral Sclerosis: Emerging Mechanisms and Therapeutic Potential
Oriquat, G. A., Malathi, H., Maharana, L., Dhyani, A., Al-Hasnaawei, S., Singh‐Chauhan, A., Arora, V., Sharma, J., & Sadeghi-Samarjan, R. (2026) · Molecular Neurobiology · doi:10.1007/s12035-026-05826-8 · Cited by 1 · see citing works
ERVK activity in CD8+ T cell immune cell compartment in patients with ALS
Sharma, S., Cortes-Pérez, C. M., Bird, S., Curzio, D. D., Vandenakker, A., Schellenberg, K. L., & Douville, R. N. (2026) · Journal of Neuroinflammation · doi:10.1186/s12974-026-03871-7 · Cited by 1 · see citing works
Lymphatic dysfunction correlates with inflammation in a mouse model of amyotrophic lateral sclerosis
Narayanan, A., Seaberg, B. L., Buxton, A. W., Vernino, A. O., Williams, V. E., Matarazzo, A., Kekre, J., Subramanian, B., Wang, W. J., Rutkowski, J. M., et al. (2025) · Disease Models & Mechanisms · doi:10.1242/dmm.052148 · Cited by 1 · see citing works
Decoding Amyotrophic Lateral Sclerosis: A Systems Biology Approach
Lombard, J., & Hamper, M. (2022) · Journal of Restorative Medicine · doi:10.14200/jrm.2022.0001 · Cited by 1 · see citing works
Scholl, L.-S., Demleitner, A. F., Riedel, J., Adachi, S., Neuenroth, L., Meijs, C., Tzeplaeff, L., Gomes, L. C., Galhoz, A., Cordts, I., et al. (2025) · Research Square · doi:10.21203/rs.3.rs-6512597/v1 · Cited by 1 · see citing works
Three ALS genes regulate expression of the MHC class II antigen presentation pathwayPreprint
Chi, B., Öztürk, M. M., Paraggio, C. L., Leonard, C. E., Sanita, M. E., Dastpak, M., O’Connell, J. D., Coady, J. A., Zhang, J., Gygi, S. P., et al. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.05.11.489680
Insulin-Based Treatment for Amyotrophic Lateral SclerosisPreprint
Rappoport, A. (2022) · Preprints.org · doi:10.20944/preprints202212.0297.v2
Levamisole Derivatives As Immune Modulators for the Treatment of Amyotrophic Lateral Sclerosis (ALS)
Baugh, S. D. P., Morrice, J. R., Reitz, A. B., Shaw, C. A., & Gregory, C. Y. (2023) · Future Medicinal Chemistry · doi:10.4155/fmc-2023-0028
- Authors
- Béland, L.-C., Markovinovic, A., Jakovac, H., De Marchi, F., Bilic, E., Mazzini, L., Kriz, J., & Munitic, I.
- Affiliations
- Université Laval (CA)
- Laboratory for Molecular Immunology, Department of Biotechnology, University of Rijeka, 51000 Rijeka, Croatia
- University of Rijeka (HR)
- Department of Neurology, ALS Centre, University of Piemonte Orientale, “Maggiore della Carità” Hospital, 28100 Novara, Italy
- Department of Neurology, Clinical Hospital Centre Zagreb, 10000 Zagreb, Croatia
- Università degli Studi del Piemonte Orientale “Amedeo Avogadro” (IT)
- CERVO Research Centre, Laval University, Quebec City, Quebec G1J 2G3, Canada
- Venue
- Brain Communications
- Publisher
- Oxford University Press (OUP)
- Published
- 2020
- Volume
- 2
- Issue
- 2
- Pages
- fcaa124-fcaa124
- ISSN
- 2632-1297 (ISSN-L)
- PMC
- PMC7585698
- OpenAlex
- W3049334858
- Cited by
- 125
- References
- 282
- Citations by year
- 2026: 15 · 2025: 33 · 2024: 26 · 2023: 17 · 2022: 18 · 2021: 16
- FWCI
- 1.43
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Brain Communications (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Funders
- Croatian Science Foundation (UIP-2013-11-7459, IP-2018-01-8563)
- University of Rijeka (18-211-1369)
- Università del Piemonte Orientale
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Keywords
- amyotrophic lateral sclerosis, neuroinflammation, autoimmunity, immune dysregulation, T cell infiltration, glial activation, C9orf72, TBK1, optineurin, CYLD, neuroprotection, neurotoxicity, immunophenotyping, patient stratification, immunosuppressive therapy failure
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