{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T22:58:10Z","timestamp":1792623490823,"version":"4.5.0"},"reference-count":287,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T00:00:00Z","timestamp":1741824000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurology International"],"abstract":"<jats:p>Spinal muscular atrophy (SMA) is a neuromuscular disorder caused by homozygous deletions or mutations in the SMN1 gene, leading to progressive motor neuron degeneration. While SMA has been classically viewed as a motor neuron-autonomous disease, increasing evidence indicates a significant role of glial cells\u2014astrocytes, microglia, oligodendrocytes, and Schwann cells\u2014in the disease pathophysiology. Astrocytic dysfunction contributes to motor neuron vulnerability through impaired calcium homeostasis, disrupted synaptic integrity, and neurotrophic factor deficits. Microglia, through reactive gliosis and complement-mediated synaptic stripping, exacerbate neurodegeneration and neuroinflammation. Oligodendrocytes exhibit impaired differentiation and metabolic support, while Schwann cells display abnormalities in myelination, extracellular matrix composition, and neuromuscular junction maintenance, further compromising motor function. Dysregulation of pathways such as NF-\u03baB, Notch, and JAK\/STAT, alongside the upregulation of complement proteins and microRNAs, reinforces the non-cell-autonomous nature of SMA. Despite the advances in SMN-restorative therapies, they do not fully mitigate glial dysfunction. Targeting glial pathology, including modulation of reactive astrogliosis, microglial polarization, and myelination deficits, represents a critical avenue for therapeutic intervention. This review comprehensively examines the multifaceted roles of glial cells in SMA and highlights emerging glia-targeted strategies to enhance treatment efficacy and improve patient outcomes.<\/jats:p>","DOI":"10.3390\/neurolint17030041","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T10:16:45Z","timestamp":1741861005000},"page":"41","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Glial Cells in Spinal Muscular Atrophy: Speculations on Non-Cell-Autonomous Mechanisms and Therapeutic Implications"],"prefix":"10.3390","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7848-6600","authenticated-orcid":false,"given":"Andrej","family":"Belan\u010di\u0107","sequence":"first","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6203-2858","authenticated-orcid":false,"given":"Tamara","family":"Jankovi\u0107","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-1255-8653","authenticated-orcid":false,"given":"Elvira Meni Maria","family":"Gkrinia","sequence":"additional","affiliation":[{"name":"Independent Researcher, 11741 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-2001-122X","authenticated-orcid":false,"given":"Iva","family":"Kristi\u0107","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jelena","family":"Raji\u010d Bumber","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentino","family":"Ra\u010dki","sequence":"additional","affiliation":[{"name":"Department of Neurology, Clinical Hospital Centre Rijeka, Kre\u0161imirova 42, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7129-1621","authenticated-orcid":false,"given":"Kristina","family":"Pilipovi\u0107","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6295-6580","authenticated-orcid":false,"given":"Dinko","family":"Vitezi\u0107","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6313-9232","authenticated-orcid":false,"given":"Jasenka","family":"Mr\u0161i\u0107-Pel\u010di\u0107","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Pharmacology and Toxicology, Faculty of Medicine, University of Rijeka, Bra\u0107e Branchetta 20, 51000 Rijeka, Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,3,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.pcl.2015.03.010","article-title":"Spinal Muscular Atrophies","volume":"62","author":"Darras","year":"2015","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1186\/s13023-017-0671-8","article-title":"Prevalence, Incidence and Carrier Frequency of 5q\u2013Linked Spinal Muscular Atrophy\u2014A Literature Review","volume":"12","author":"Verhaart","year":"2017","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Chen, T.-H. (2020). New and Developing Therapies in Spinal Muscular Atrophy: From Genotype to Phenotype to Treatment and Where Do We Stand?. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21093297"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/gt.2017.52","article-title":"The Clinical Landscape for SMA in a New Therapeutic Era","volume":"24","author":"Talbot","year":"2017","journal-title":"Gene Ther."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1186\/s43042-019-0044-z","article-title":"Genetic Pattern of SMN1, SMN2, and NAIP Genes in Prognosis of SMA Patients","volume":"21","author":"Hassan","year":"2020","journal-title":"Egypt. J. Med. Hum. Genet."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1016\/j.nmd.2017.05.011","article-title":"Spinal Muscular Atrophy: A Changing Phenotype beyond the Clinical Trials","volume":"27","author":"Tizzano","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1007\/s40271-020-00415-w","article-title":"\u201cThe Whole Game Is Changing and You\u2019ve Got Hope\u201d: Australian Perspectives on Treatment Decision Making in Spinal Muscular Atrophy","volume":"13","author":"Farrar","year":"2020","journal-title":"Patient-Patient-Centered Outcomes Res."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Abati, E., Citterio, G., Bresolin, N., Comi, G.P., and Corti, S. (2020). Glial Cells Involvement in Spinal Muscular Atrophy: Could SMA Be a Neuroinflammatory Disease?. Neurobiol. Dis., 140.","DOI":"10.1016\/j.nbd.2020.104870"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1242\/dmm.007245","article-title":"The Contribution of Mouse Models to Understanding the Pathogenesis of Spinal Muscular Atrophy","volume":"4","author":"Sleigh","year":"2011","journal-title":"Dis. Models Mech."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1038\/nn1988","article-title":"Glial Cells as Intrinsic Components of Non-Cell-Autonomous Neurodegenerative Disease","volume":"10","author":"Lobsiger","year":"2007","journal-title":"Nat. Neurosci."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1418","DOI":"10.1002\/glia.22522","article-title":"Spinal Muscular Atrophy Astrocytes Exhibit Abnormal Calcium Regulation and Reduced Growth Factor Production","volume":"61","author":"McGivern","year":"2013","journal-title":"Glia"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1083\/jcb.200304128","article-title":"Smn, the Spinal Muscular Atrophy\u2013Determining Gene Product, Modulates Axon Growth and Localization of \u03b2-Actin MRNA in Growth Cones of Motoneurons","volume":"163","author":"Rossoll","year":"2003","journal-title":"J. Cell Biol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1126\/science.1250127","article-title":"SMN2 Splicing Modifiers Improve Motor Function and Longevity in Mice with Spinal Muscular Atrophy","volume":"345","author":"Naryshkin","year":"2014","journal-title":"Science"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"3818","DOI":"10.1523\/JNEUROSCI.5775-11.2012","article-title":"Motor Neuron Rescue in Spinal Muscular Atrophy Mice Demonstrates That Sensory-Motor Defects Are a Consequence, Not a Cause, of Motor Neuron Dysfunction","volume":"32","author":"Gogliotti","year":"2012","journal-title":"J. Neurosci."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1038\/nature10485","article-title":"Peripheral SMN Restoration Is Essential for Long-Term Rescue of a Severe Spinal Muscular Atrophy Mouse Model","volume":"478","author":"Hua","year":"2011","journal-title":"Nature"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Lee, A.J.-H., Awano, T., Park, G.-H., and Monani, U.R. (2012). Limited Phenotypic Effects of Selectively Augmenting the SMN Protein in the Neurons of a Mouse Model of Severe Spinal Muscular Atrophy. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0046353"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1093\/hmg\/ddm379","article-title":"Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect","volume":"17","author":"Gavrilina","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1093\/hmg\/ddi467","article-title":"Distinct and overlapping alterations in motor and sensory neurons in a mouse model of spinal muscular atrophy","volume":"15","author":"Jablonka","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.neuron.2010.12.032","article-title":"Early Functional Impairment of Sensory-Motor Connectivity in a Mouse Model of Spinal Muscular Atrophy","volume":"69","author":"Mentis","year":"2011","journal-title":"Neuron"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1146\/annurev.neuro.24.1.677","article-title":"Neurotrophins: Roles in Neuronal Development and Function","volume":"24","author":"Huang","year":"2001","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"10122","DOI":"10.3390\/ijms140510122","article-title":"TrkB Receptor Signalling: Implications in Neurodegenerative, Psychiatric and Proliferative Disorders","volume":"14","author":"Gupta","year":"2013","journal-title":"Int. J. Mol. Sci."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Ramsey, A., and Huang, E.J. (2023). Plastin 3 Rescues BDNF-TrkB Signaling in Spinal Muscular Atrophy. J. Cell Biol., 222.","DOI":"10.1083\/jcb.202301036"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"8703","DOI":"10.1523\/JNEUROSCI.0204-12.2012","article-title":"Survival Motor Neuron Protein in Motor Neurons Determines Synaptic Integrity in Spinal Muscular Atrophy","volume":"32","author":"Martinez","year":"2012","journal-title":"J. Neurosci. Off. J. Soc. Neurosci."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"8578","DOI":"10.1523\/JNEUROSCI.1578-17.2017","article-title":"Effects of Astroglia on Motor Neurons in Spinal Muscular Atrophy","volume":"37","author":"Verheijen","year":"2017","journal-title":"J. Neurosci."},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"Storelli, E., Cassina, N., Rasini, E., Marino, F., and Cosentino, M. (2019). Do Th17 Lymphocytes and IL-17 Contribute to Parkinson\u2019s Disease? A Systematic Review of Available Evidence. Front. Neurol., 10.","DOI":"10.3389\/fneur.2019.00013"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"3409","DOI":"10.1093\/hmg\/ddx230","article-title":"Astrocyte-produced miR-146a as a mediator of motor neuron loss in spinal muscular atrophy","volume":"26","author":"Sison","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"ref_27","first-page":"282","article-title":"Oligodendrocyte Development and CNS Myelination Are Unaffected in a Mouse Model of Severe Spinal Muscular Atrophy","volume":"26","author":"Cummings","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1002\/glia.24177","article-title":"Survival Motor Neuron Protein Deficiency Alters Microglia Reactivity","volume":"70","author":"Khayrullina","year":"2022","journal-title":"Glia"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"12005","DOI":"10.1523\/JNEUROSCI.2208-10.2010","article-title":"Reduced Survival of Motor Neuron (SMN) Protein in Motor Neuronal Progenitors Functions Cell Autonomously to Cause Spinal Muscular Atrophy in Model Mice Expressing the Human Centromeric (SMN2) Gene","volume":"30","author":"Park","year":"2010","journal-title":"J. Neurosci."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1002\/glia.24153","article-title":"Viral Mediated Knockdown of GATA6 in SMA IPSC -Derived Astrocytes Prevents Motor Neuron Loss and Microglial Activation","volume":"70","author":"Allison","year":"2022","journal-title":"Glia"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1016\/j.jphs.2020.09.001","article-title":"Survival Motor Neuron Protein Regulates Oxidative Stress and Inflammatory Response in Microglia of the Spinal Cord in Spinal Muscular Atrophy","volume":"144","author":"Ando","year":"2020","journal-title":"J. Pharmacol. Sci."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1291","DOI":"10.1042\/BST20140107","article-title":"Glia in the Pathogenesis of Neurodegenerative Diseases","volume":"42","author":"Verkhratsky","year":"2014","journal-title":"Biochem. Soc. Trans."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1002\/glia.22389","article-title":"The \u201cQuad-Partite\u201d Synapse: Microglia-Synapse Interactions in the Developing and Mature CNS","volume":"61","author":"Schafer","year":"2013","journal-title":"Glia"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/j.tins.2008.09.003","article-title":"Neuroglia: The 150 Years After","volume":"31","author":"Kettenmann","year":"2008","journal-title":"Trends Neurosci."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1126\/science.aat0473","article-title":"Glia as Architects of Central Nervous System Formation and Function","volume":"362","author":"Allen","year":"2018","journal-title":"Science"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1002\/cne.21974","article-title":"Equal Numbers of Neuronal and Nonneuronal Cells Make the Human Brain an Isometrically Scaled-up Primate Brain","volume":"513","author":"Azevedo","year":"2009","journal-title":"J. Comp. Neurol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3865","DOI":"10.1002\/cne.24040","article-title":"The Search for True Numbers of Neurons and Glial Cells in the Human Brain: A Review of 150 Years of Cell Counting","volume":"524","author":"Bahney","year":"2016","journal-title":"J. Comp. Neurol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.1038\/nm.4397","article-title":"Microglia Emerge as Central Players in Brain Disease","volume":"23","author":"Salter","year":"2017","journal-title":"Nat. Med."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1152\/physrev.00036.2013","article-title":"Glial Cells as Progenitors and Stem Cells: New Roles in the Healthy and Diseased Brain","volume":"94","author":"Dimou","year":"2014","journal-title":"Physiol. Rev."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1007\/BF00689556","article-title":"Werdnig-Hoffmann Disease: Proposal of a Pathogenetic Mechanism","volume":"41","author":"Chou","year":"1978","journal-title":"Acta Neuropathol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/j.nbd.2009.10.005","article-title":"Inflammation in ALS and SMA: Sorting out the Good from the Evil","volume":"37","author":"Papadimitriou","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Romero, D.M., Zaidi, D., Cifuentes-Diaz, C., Maillard, C., Grannec, G., Selloum, M., Birling, M.-C., Bahi-Buisson, N., and Francis, F. (2023). A Human Dynein Heavy Chain Mutation Impacts Cortical Progenitor Cells Causing Developmental Defects, Reduced Brain Size and Altered Brain Architecture. Neurobiol. Dis., 180.","DOI":"10.1016\/j.nbd.2023.106085"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2512","DOI":"10.1007\/s11064-021-03296-z","article-title":"Radial Glial Cells: New Views on Old Questions","volume":"46","author":"Arellano","year":"2021","journal-title":"Neurochem. Res."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1111\/tra.12769","article-title":"Local Gene Regulation in Radial Glia: Lessons from across the Nervous System","volume":"21","author":"Silver","year":"2020","journal-title":"Traffic"},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Miranda-Negr\u00f3n, Y., and Garc\u00eda-Arrar\u00e1s, J.E. (2022). Radial Glia and Radial Glia-like Cells: Their Role in Neurogenesis and Regeneration. Front. Neurosci., 16.","DOI":"10.3389\/fnins.2022.1006037"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1002\/cne.901450105","article-title":"Mode of Cell Migration to the Superficial Layers of Fetal Monkey Neocortex","volume":"145","author":"Rakic","year":"1972","journal-title":"J. Comp. Neurol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/S0361-9230(99)00065-9","article-title":"The History of Radial Glia","volume":"49","author":"Bentivoglio","year":"1999","journal-title":"Brain Res. Bull."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1159\/000338108","article-title":"Activation of MAPK\/PI3K\/SMAD Pathways by TGF-\u03b21 Controls Differentiation of Radial Glia into Astrocytes in Vitro","volume":"34","author":"Stipursky","year":"2012","journal-title":"Dev. Neurosci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1002\/glia.10250","article-title":"Radial Glia Diversity: A Matter of Cell Fate","volume":"43","author":"Kriegstein","year":"2003","journal-title":"Glia"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1042\/BST20130188","article-title":"Mutations in Cytoplasmic Dynein and Its Regulators Cause Malformations of Cortical Development and Neurodegenerative Diseases","volume":"41","author":"Lipka","year":"2013","journal-title":"Biochem. Soc. Trans."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1714","DOI":"10.1212\/WNL.0b013e3182556c05","article-title":"Mutations in the Tail Domain of DYNC1H1 Cause Dominant Spinal Muscular Atrophy","volume":"78","author":"Harms","year":"2012","journal-title":"Neurology"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1016\/j.tins.2013.08.001","article-title":"Cytoplasmic Dynein Heavy Chain: The Servant of Many Masters","volume":"36","author":"Schiavo","year":"2013","journal-title":"Trends Neurosci."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.nbd.2013.05.015","article-title":"Dynein Mutations Associated with Hereditary Motor Neuropathies Impair Mitochondrial Morphology and Function with Age","volume":"58","author":"Eschbach","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"4705","DOI":"10.1007\/s12035-024-04576-9","article-title":"Molecular Motors in Myelination and Their Misregulation in Disease","volume":"62","author":"Barbosa","year":"2024","journal-title":"Mol. Neurobiol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1038\/s10038-020-0803-1","article-title":"The Clinical-Phenotype Continuum in DYNC1H1-Related Disorders\u2014Genomic Profiling and Proposal for a Novel Classification","volume":"65","author":"Becker","year":"2020","journal-title":"J. Hum. Genet."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1016\/j.celrep.2014.07.052","article-title":"Bicaudal D Family Adaptor Proteins Control the Velocity of Dynein-Based Movements","volume":"8","author":"Schlager","year":"2014","journal-title":"Cell Rep."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1016\/j.ajhg.2013.04.011","article-title":"Mutations in BICD2, Which Encodes a Golgin and Important Motor Adaptor, Cause Congenital Autosomal-Dominant Spinal Muscular Atrophy","volume":"92","author":"Neveling","year":"2013","journal-title":"Am. J. Hum. Genet."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1016\/j.ajhg.2013.04.018","article-title":"Mutations in BICD2 Cause Dominant Congenital Spinal Muscular Atrophy and Hereditary Spastic Paraplegia","volume":"92","author":"Oates","year":"2013","journal-title":"Am. J. Hum. Genet."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/j.ajhg.2013.04.013","article-title":"Molecular Defects in the Motor Adaptor BICD2 Cause Proximal Spinal Muscular Atrophy with Autosomal-Dominant Inheritance","volume":"92","author":"Peeters","year":"2013","journal-title":"Am. J. Hum. Genet."},{"key":"ref_60","unstructured":"Ram\u00f3n, S. (1952). Histologie du Syst\u00e8me Nerveux de l\u2019Homme & des Vert\u00e9br\u00e9s, Consejo Superior de Investigaciones Cient\u00edficas, Instituto Ramon y Cajal."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1007\/s00401-009-0619-8","article-title":"Astrocytes: Biology and Pathology","volume":"119","author":"Sofroniew","year":"2009","journal-title":"Acta Neuropathol."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Tabata, H. (2015). Diverse Subtypes of Astrocytes and Their Development during Corticogenesis. Front. Neurosci., 9.","DOI":"10.3389\/fnins.2015.00114"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"6391","DOI":"10.1523\/JNEUROSCI.6221-11.2012","article-title":"Genomic Analysis of Reactive Astrogliosis","volume":"32","author":"Zamanian","year":"2012","journal-title":"J. Neurosci."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"2761","DOI":"10.1093\/brain\/awl165","article-title":"Essential Protective Roles of Reactive Astrocytes in Traumatic Brain Injury","volume":"129","author":"Myer","year":"2006","journal-title":"Brain"},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Michinaga, S., and Koyama, Y. (2021). Pathophysiological Responses and Roles of Astrocytes in Traumatic Brain Injury. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22126418"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"2143","DOI":"10.1523\/JNEUROSCI.3547-03.2004","article-title":"Reactive Astrocytes Protect Tissue and Preserve Function after Spinal Cord Injury","volume":"24","author":"Faulkner","year":"2004","journal-title":"J. Neurosci."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0301-0082(00)00067-8","article-title":"Glutamate uptake","volume":"65","author":"Danbolt","year":"2001","journal-title":"Prog. Neurobiol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1111\/bph.13125","article-title":"The Contribution of Astrocytes and Microglia to Traumatic Brain Injury","volume":"173","author":"Karve","year":"2015","journal-title":"Br. J. Pharmacol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12964-020-00549-2","article-title":"Dual Roles of Astrocytes in Plasticity and Reconstruction after Traumatic Brain Injury","volume":"18","author":"Zhou","year":"2020","journal-title":"Cell Commun. Signal. CCS"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/S0166-2236(98)01349-6","article-title":"Tripartite Synapses: Glia, the Unacknowledged Partner","volume":"22","author":"Araque","year":"1999","journal-title":"Trends Neurosci."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1038\/nrn3484","article-title":"Emerging roles of astrocytes in neural circuit development","volume":"14","author":"Clarke","year":"2013","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"2543","DOI":"10.1523\/JNEUROSCI.3534-15.2016","article-title":"Defects in Motoneuron\u2013Astrocyte Interactions in Spinal Muscular Atrophy","volume":"36","author":"Zhou","year":"2016","journal-title":"J. Neurosci."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1126\/science.1967852","article-title":"Glutamate Induces Calcium Waves in Cultured Astrocytes: Long-Range Glial Signaling","volume":"247","author":"Finkbeiner","year":"1990","journal-title":"Science"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"8629","DOI":"10.1073\/pnas.97.15.8629","article-title":"Physiological Astrocytic Calcium Levels Stimulate Glutamate Release to Modulate Adjacent Neurons","volume":"97","author":"Parpura","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1111\/j.1365-2990.1983.tb00124.x","article-title":"GLIAL BUNDLES in SPINAL NERVE ROOTS: A FORM of ISOMORPHIC GLIOSIS at the JUNCTION of the CENTRAL and PERIPHERAL NERVOUS SYSTEM","volume":"9","author":"Ghatak","year":"1983","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1038\/nature11314","article-title":"Oligodendroglia Metabolically Support Axons and Contribute to Neurodegeneration","volume":"487","author":"Lee","year":"2012","journal-title":"Nature"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"4094","DOI":"10.1093\/hmg\/ddv148","article-title":"Astrocytes Influence the Severity of Spinal Muscular Atrophy","volume":"24","author":"Rindt","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Leo, M., Schmitt, L.-I., Fleischer, M., Steffen, R., Osswald, C., Kleinschnitz, C., and Hagenacker, T. (2022). Induction of Survival of Motor Neuron (SMN) Protein Deficiency in Spinal Astrocytes by Small Interfering RNA as an in Vitro Model of Spinal Muscular Atrophy. Cells, 11.","DOI":"10.3390\/cells11030558"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1007\/s00401-023-02554-4","article-title":"Spinal Astrocyte Dysfunction Drives Motor Neuron Loss in Late-Onset Spinal Muscular Atrophy","volume":"145","author":"Schmitt","year":"2023","journal-title":"Acta Neuropathol."},{"key":"ref_80","doi-asserted-by":"crossref","unstructured":"Hassan, A., di Vito, R., Nuzzo, T., Vidali, M., Carlini, M.J., Yadav, S., Yang, H., D\u2019Amico, A., Kolici, X., and Valsecchi, V. (2024). Dysregulated Balance of D- and L-Amino Acids Modulating Glutamatergic Neurotransmission in Severe Spinal Muscular Atrophy. Neurobiol. Dis., 207.","DOI":"10.1101\/2024.10.22.619645"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1002\/glia.24340","article-title":"Diminished Motor Neuron Activity Driven by Abnormal Astrocytic EAAT1 Glutamate Transporter Activity in Spinal Muscular Atrophy Is Not Fully Restored after Lentiviral SMN Delivery","volume":"71","author":"Welby","year":"2023","journal-title":"Glia"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1002\/acn3.51645","article-title":"Glial Fibrillary Acidic Protein in Cerebrospinal Fluid of Patients with Spinal Muscular Atrophy","volume":"9","author":"Freigang","year":"2022","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"5309","DOI":"10.1523\/JNEUROSCI.3472-16.2017","article-title":"Decreased Motor Neuron Support by SMA Astrocytes due to Diminished MCP1 Secretion","volume":"37","author":"Martin","year":"2017","journal-title":"J. Neurosci."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1523\/JNEUROSCI.4434-08.2009","article-title":"Impaired Synaptic Vesicle Release and Immaturity of Neuromuscular Junctions in Spinal Muscular Atrophy Mice","volume":"29","author":"Kong","year":"2009","journal-title":"J. Neurosci."},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Ling, K.K.Y., Lin, M.-Y., Zingg, B., Feng, Z., and Ko, C.-P. (2010). Synaptic Defects in the Spinal and Neuromuscular Circuitry in a Mouse Model of Spinal Muscular Atrophy. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0015457"},{"key":"ref_86","first-page":"342","article-title":"The Astrocyte Odyssey","volume":"86","author":"Wang","year":"2008","journal-title":"Prog. Neurobiol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1093\/hmg\/ddv489","article-title":"SMN Deficiency Does Not Induce Oxidative Stress in SMA IPSC-Derived Astrocytes or Motor Neurons","volume":"25","author":"Patitucci","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1523\/JNEUROSCI.20-01-00240.2000","article-title":"AMPA Exposures Induce Mitochondrial Ca2+Overload and ROS Generation in Spinal Motor Neurons In Vitro","volume":"20","author":"Carriedo","year":"2000","journal-title":"J. Neurosci."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/S0022-510X(01)00626-8","article-title":"Functional Diversity of Recombinant Human AMPA Type Glutamate Receptors: Possible Implications for Selective Vulnerability of Motor Neurons","volume":"191","author":"Krampfl","year":"2001","journal-title":"J. Neurol. Sci."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1038\/s41593-018-0242-x","article-title":"Microglia in Neurodegeneration","volume":"21","author":"Hickman","year":"2018","journal-title":"Nat. Neurosci."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1038\/s41392-023-01588-0","article-title":"Microglia in Neurodegenerative Diseases: Mechanism and Potential Therapeutic Targets","volume":"8","author":"Gao","year":"2023","journal-title":"Signal Transduct. Target. Ther."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1929","DOI":"10.1113\/JP272134","article-title":"Microglia across the Lifespan: From Origin to Function in Brain Development, Plasticity and Cognition","volume":"595","author":"Tay","year":"2016","journal-title":"J. Physiol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1186\/s12974-021-02309-6","article-title":"The Semantics of Microglia Activation: Neuroinflammation, Homeostasis, and Stress","volume":"18","author":"Woodburn","year":"2021","journal-title":"J. Neuroinflamm."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"3087","DOI":"10.1016\/j.celrep.2019.11.013","article-title":"The Classical Complement Pathway Mediates Microglia-Dependent Remodeling of Spinal Motor Circuits during Development and in SMA","volume":"29","author":"Vukojicic","year":"2019","journal-title":"Cell Rep."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.neuron.2012.12.023","article-title":"Microglia: New Roles for the Synaptic Stripper","volume":"77","author":"Kettenmann","year":"2013","journal-title":"Neuron"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"3240","DOI":"10.1172\/JCI90606","article-title":"Microglia in Alzheimer\u2019s Disease","volume":"127","author":"Sarlus","year":"2017","journal-title":"J. Clin. Investig."},{"key":"ref_97","first-page":"182","article-title":"Infiltration of CD4+ Lymphocytes into the Brain Contributes to Neurodegeneration in a Mouse Model of Parkinson Disease","volume":"119","author":"Brochard","year":"2009","journal-title":"J. Clin. Investig."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1002\/acn3.423","article-title":"New Insights into SMA Pathogenesis: Immune Dysfunction and Neuroinflammation","volume":"4","author":"Deguise","year":"2017","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1093\/jnen\/nly033","article-title":"Glial Activation and Central Synapse Loss, but Not Motoneuron Degeneration, Are Prevented by the Sigma-1 Receptor Agonist PRE-084 in the Smn2B\/\u2212 Mouse Model of Spinal Muscular Atrophy","volume":"77","author":"Blasco","year":"2018","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1440-1789.2008.00910.x","article-title":"An Autopsy Case of Spinal Muscular Atrophy Type III (Kugelberg-Welander Disease)","volume":"29","author":"Kuru","year":"2009","journal-title":"Neuropathology"},{"key":"ref_101","doi-asserted-by":"crossref","unstructured":"Karafoulidou, E., Kesidou, E., Theotokis, P., Konstantinou, C., Nella, M.-K., Michailidou, I., Touloumi, O., Polyzoidou, E., Salamotas, I., and Einstein, O. (2024). Systemic LPS Administration Stimulates the Activation of Non-Neuronal Cells in an Experimental Model of Spinal Muscular Atrophy. Cells, 13.","DOI":"10.3390\/cells13090785"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1016\/j.bbamcr.2017.02.011","article-title":"SMN1 Functions as a Novel Inhibitor for TRAF6-Mediated NF-\u039aB Signaling","volume":"1864","author":"Kim","year":"2017","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Res."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"4080","DOI":"10.1093\/hmg\/ddw247","article-title":"Enhancing Survival Motor Neuron Expression Extends Lifespan and Attenuates Neurodegeneration in Mutant TDP-43 Mice","volume":"25","author":"Perera","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1097\/NEN.0000000000000074","article-title":"Mechanisms Involved in Spinal Cord Central Synapse Loss in a Mouse Model of Spinal Muscular Atrophy","volume":"73","author":"Tarabal","year":"2014","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1097\/NEN.0b013e31821cbd8b","article-title":"Defective Neuromuscular Junction Organization and Postnatal Myogenesis in Mice with Severe Spinal Muscular Atrophy","volume":"70","author":"Dachs","year":"2011","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_106","first-page":"4061","article-title":"A Severe Mouse Model of Spinal Muscular Atrophy Develops Early Systemic Inflammation","volume":"27","author":"Wan","year":"2018","journal-title":"Hum. Mol. Genet."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"1274","DOI":"10.1523\/JNEUROSCI.2140-20.2020","article-title":"Microglia Elimination Increases Neural Circuit Connectivity and Activity in Adult Mouse Cortex","volume":"41","author":"Liu","year":"2021","journal-title":"J. Neurosci."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/j.bbi.2016.08.018","article-title":"Neonatal Microglia: The Cornerstone of Brain Fate","volume":"59","author":"Pierre","year":"2017","journal-title":"Brain Behav. Immun."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"1596","DOI":"10.1016\/j.cell.2013.11.030","article-title":"Microglia Promote Learning-Dependent Synapse Formation through Brain-Derived Neurotrophic Factor","volume":"155","author":"Parkhurst","year":"2013","journal-title":"Cell"},{"key":"ref_110","first-page":"145","article-title":"Displacement of Synaptic Terminals from Regenerating Motoneurons by Microglial Cells","volume":"85","author":"Blinzinger","year":"1968","journal-title":"Cell Tissue Res."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1016\/j.brainresrev.2003.11.004","article-title":"The Facial Nerve Axotomy Model","volume":"44","author":"Moran","year":"2004","journal-title":"Brain Res. Rev."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1016\/j.cell.2007.10.036","article-title":"The Classical Complement Cascade Mediates CNS Synapse Elimination","volume":"131","author":"Stevens","year":"2007","journal-title":"Cell"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1038\/nature12776","article-title":"Astrocytes Mediate Synapse Elimination through MEGF10 and MERTK Pathways","volume":"504","author":"Chung","year":"2013","journal-title":"Nature"},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1456","DOI":"10.1126\/science.1202529","article-title":"Synaptic Pruning by Microglia Is Necessary for Normal Brain Development","volume":"333","author":"Paolicelli","year":"2011","journal-title":"Science"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1016\/j.molimm.2004.06.011","article-title":"Complement: A Unique Innate Immune Sensor for Danger Signals","volume":"41","author":"Gasque","year":"2004","journal-title":"Mol. Immunol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1016\/j.bbi.2024.04.018","article-title":"Complement C1q\/C3-CR3 Signaling Pathway Mediates Abnormal Microglial Phagocytosis of Synapses in a Mouse Model of Depression","volume":"119","author":"Han","year":"2024","journal-title":"Brain Behav. Immun."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1126\/science.aaz2288","article-title":"Microglia Mediate Forgetting via Complement-Dependent Synaptic Elimination","volume":"367","author":"Wang","year":"2020","journal-title":"Science"},{"key":"ref_118","first-page":"115","article-title":"Complement Receptor 3 (CD11b\/CD18) Is Implicated in the Elimination of \u03b2-Amyloid Peptides","volume":"25","author":"Laporte","year":"2010","journal-title":"Fundam. Clin. Pharmacol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1126\/science.aad8373","article-title":"Complement and Microglia Mediate Early Synapse Loss in Alzheimer Mouse Models","volume":"352","author":"Hong","year":"2016","journal-title":"Science"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/j.cell.2016.04.001","article-title":"Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation","volume":"165","author":"Lui","year":"2016","journal-title":"Cell"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"110105","DOI":"10.1016\/j.jpsychores.2020.110105","article-title":"Increased Serum Levels of Complement C1q in Major Depressive Disorder","volume":"133","author":"Yao","year":"2020","journal-title":"J. Psychosom. Res."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"19348","DOI":"10.1073\/pnas.1319280110","article-title":"Dysregulation of Synaptogenesis Genes Antecedes Motor Neuron Pathology in Spinal Muscular Atrophy","volume":"110","author":"Zhang","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1002\/glia.440070117","article-title":"Cytotoxicity of Microglia","volume":"7","author":"Banati","year":"1993","journal-title":"Glia"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.bbi.2013.02.005","article-title":"Characterization of Phenotype Markers and Neuronotoxic Potential of Polarised Primary Microglia in Vitro","volume":"32","author":"Chhor","year":"2013","journal-title":"Brain Behav. Immun."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/0166-2236(96)10049-7","article-title":"Microglia: A Sensor for Pathological Events in the CNS","volume":"19","author":"Kreutzberg","year":"1996","journal-title":"Trends Neurosci."},{"key":"ref_126","doi-asserted-by":"crossref","unstructured":"Jurga, A.M., Paleczna, M., and Kuter, K.Z. (2020). Overview of General and Discriminating Markers of Differential Microglia Phenotypes. Front. Cell. Neurosci., 14.","DOI":"10.3389\/fncel.2020.00198"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1002\/dneu.22905","article-title":"Microglia in Motor Neuron Disease: Signaling Evidence from Last 10 Years","volume":"82","author":"Wang","year":"2022","journal-title":"Dev. Neurobiol."},{"key":"ref_128","first-page":"78","article-title":"Human Microglia Regional Heterogeneity and Phenotypes Determined by Multiplexed Single-Cell Mass Cytometry","volume":"22","author":"Schlickeiser","year":"2018","journal-title":"Nat. Neurosci."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.molmed.2020.09.001","article-title":"Redefining Microglial Identity in Health and Disease at Single-Cell Resolution","volume":"27","author":"Provenzano","year":"2020","journal-title":"Trends Mol. Med."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"2098","DOI":"10.1038\/s41593-019-0532-y","article-title":"Mapping Microglia States in the Human Brain through the Integration of High-Dimensional Techniques","volume":"22","author":"Sankowski","year":"2019","journal-title":"Nat. Neurosci."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"656","DOI":"10.2174\/1381612822666151204000636","article-title":"Oligodendrocyte Development and Myelination in Neurodevelopment: Molecular Mechanisms in Health and Disease","volume":"22","author":"Barateiro","year":"2016","journal-title":"Curr. Pharm. Des."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.tins.2019.01.002","article-title":"Molecular Control of Oligodendrocyte Development","volume":"42","author":"Elbaz","year":"2019","journal-title":"Trends Neurosci."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.bbadis.2010.09.010","article-title":"Cells of the Oligodendroglial Lineage, Myelination, and Remyelination","volume":"1812","author":"Miron","year":"2011","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Basis Dis."},{"key":"ref_134","doi-asserted-by":"crossref","unstructured":"Simons, M., and Nave, K.-A. (2019). Oligodendrocytes: Myelination and Axonal Support. Cold Spring Harb. Perspect. Biol., 8.","DOI":"10.1101\/cshperspect.a020479"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1093\/brain\/awf043","article-title":"Patients Lacking the Major CNS Myelin Protein, Proteolipid Protein 1, Develop Length-Dependent Axonal Degeneration in the Absence of Demyelination and Inflammation","volume":"125","author":"Garbern","year":"2002","journal-title":"Brain"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1038\/ng1095","article-title":"Disruption of Cnp1 Uncouples Oligodendroglial Functions in Axonal Support and Myelination","volume":"33","author":"Goebbels","year":"2003","journal-title":"Nat. Genet."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"6236","DOI":"10.1523\/JNEUROSCI.0567-10.2010","article-title":"Neurodegeneration in a Transgenic Mouse Model of Multiple System Atrophy Is Associated with Altered Expression of Oligodendroglial-Derived Neurotrophic Factors","volume":"30","author":"Ubhi","year":"2010","journal-title":"J. Neurosci."},{"key":"ref_138","doi-asserted-by":"crossref","unstructured":"B\u00e4umer, D., Lee, S., Nicholson, G., Davies, J.L., Parkinson, N.J., Murray, L.M., Gillingwater, T.H., Ansorge, O., Davies, K.E., and Talbot, K. (2009). Alternative Splicing Events Are a Late Feature of Pathology in a Mouse Model of Spinal Muscular Atrophy. PLoS Genet., 5.","DOI":"10.1371\/journal.pgen.1000773"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.bbamem.2012.01.024","article-title":"Gap Junction Communication in Myelinating Glia","volume":"1828","author":"Solsona","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1146\/annurev-neuro-110920-030610","article-title":"Beyond Wrapping: Canonical and Noncanonical Functions of Schwann Cells","volume":"45","author":"Taveggia","year":"2022","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_141","doi-asserted-by":"crossref","unstructured":"Bosch-Queralt, M., Fledrich, R., and Stassart, R.M. (2023). Schwann Cell Functions in Peripheral Nerve Development and Repair. Neurobiol. Dis., 176.","DOI":"10.1016\/j.nbd.2022.105952"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"1491","DOI":"10.1002\/glia.20753","article-title":"Neuregulin-1, a Key Axonal Signal That Drives Schwann Cell Growth and Differentiation","volume":"56","author":"Birchmeier","year":"2008","journal-title":"Glia"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1093\/hmg\/ddp562","article-title":"Ciliary Neurotrophic Factor-Induced Sprouting Preserves Motor Function in a Mouse Model of Mild Spinal Muscular Atrophy","volume":"19","author":"Simon","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1146\/annurev-neuro-100120-122621","article-title":"Ensheathment and Myelination of Axons: Evolution of Glial Functions","volume":"44","author":"Nave","year":"2021","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"4546","DOI":"10.1021\/pr500492j","article-title":"Label-Free Quantitative Proteomic Profiling Identifies Disruption of Ubiquitin Homeostasis as a Key Driver of Schwann Cell Defects in Spinal Muscular Atrophy","volume":"13","author":"Hunter","year":"2014","journal-title":"J. Proteome Res."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.brainresrev.2009.12.006","article-title":"NG2 Cells: Properties, Progeny and Origin","volume":"63","author":"Trotter","year":"2010","journal-title":"Brain Res. Rev."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.neuron.2013.01.006","article-title":"Oligodendrocyte Dynamics in the Healthy Adult CNS: Evidence for Myelin Remodeling","volume":"77","author":"Young","year":"2013","journal-title":"Neuron"},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1097\/WNR.0000000000001206","article-title":"Impairment of Oligodendrocyte Lineages in Spinal Muscular Atrophy Model Systems","volume":"30","author":"Ohuchi","year":"2019","journal-title":"Neuroreport"},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/S0896-6273(00)80515-2","article-title":"Notch Receptor Activation Inhibits Oligodendrocyte Differentiation","volume":"21","author":"Wang","year":"1998","journal-title":"Neuron"},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"11424","DOI":"10.3390\/ijms140611424","article-title":"Notch Signaling Pathway Is Activated in Motoneurons of Spinal Muscular Atrophy","volume":"14","author":"Garcera","year":"2013","journal-title":"Int. J. Mol. Sci."},{"key":"ref_151","doi-asserted-by":"crossref","unstructured":"Hauser, S., Schuster, S., Heuten, E., H\u00f6flinger, P., Admard, J., Schelling, Y., Velic, A., Macek, B., Ossowski, S., and Sch\u00f6ls, L. (2020). Comparative Transcriptional Profiling of Motor Neuron Disorder-Associated Genes in Various Human Cell Culture Models. Front. Cell Dev. Biol., 8.","DOI":"10.3389\/fcell.2020.544043"},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.1002\/glia.20893","article-title":"Early Ultrastructural Defects of Axons and Axon-Glia Junctions in Mice Lacking Expression of Cnp1","volume":"57","author":"Edgar","year":"2009","journal-title":"Glia"},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1126\/science.280.5369.1610","article-title":"Axonal Swellings and Degeneration in Mice Lacking the Major Proteolipid of Myelin","volume":"280","author":"Griffiths","year":"1998","journal-title":"Science"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1523\/JNEUROSCI.18-06-01953.1998","article-title":"Myelin-Associated Glycoprotein Is a Myelin Signal That Modulates the Caliber of Myelinated Axons","volume":"18","author":"Yin","year":"1998","journal-title":"J. Neurosci."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1038\/s41593-020-0689-4","article-title":"A Glycolytic Shift in Schwann Cells Supports Injured Axons","volume":"23","author":"Babetto","year":"2020","journal-title":"Nat. Neurosci."},{"key":"ref_156","doi-asserted-by":"crossref","unstructured":"Rodr\u00edguez Cruz, P.M., Cossins, J., Beeson, D., and Vincent, A. (2020). The Neuromuscular Junction in Health and Disease: Molecular Mechanisms Governing Synaptic Formation and Homeostasis. Front. Mol. Neurosci., 13.","DOI":"10.3389\/fnmol.2020.610964"},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"494","DOI":"10.14336\/AD.2020.0708","article-title":"Terminal Schwann Cell Aging: Implications for Age-Associated Neuromuscular Dysfunction","volume":"12","author":"Izeta","year":"2021","journal-title":"Aging Dis."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"e56935","DOI":"10.7554\/eLife.56935","article-title":"Specific Labeling of Synaptic Schwann Cells Reveals Unique Cellular and Molecular Features","volume":"9","author":"Castro","year":"2020","journal-title":"eLife"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"11870","DOI":"10.1523\/JNEUROSCI.3165-10.2010","article-title":"Perisynaptic Glia Discriminate Patterns of Motor Nerve Activity and Influence Plasticity at the Neuromuscular Junction","volume":"30","author":"Todd","year":"2010","journal-title":"J. Neurosci."},{"key":"ref_160","doi-asserted-by":"crossref","unstructured":"Ko, C.-P., and Robitaille, R. (2015). Perisynaptic Schwann Cells at the Neuromuscular Synapse: Adaptable, Multitasking Glial Cells. Cold Spring Harb. Perspect. Biol., 7.","DOI":"10.1101\/cshperspect.a020503"},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1038\/nrn3821","article-title":"Neuromuscular Synaptogenesis: Coordinating Partners with Multiple Functions","volume":"15","author":"Darabid","year":"2014","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/S0896-6273(03)00682-2","article-title":"Glial Cells Maintain Synaptic Structure and Function and Promote Development of the Neuromuscular Junction in Vivo","volume":"40","author":"Reddy","year":"2003","journal-title":"Neuron"},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"10390","DOI":"10.1523\/JNEUROSCI.19-23-10390.1999","article-title":"Glial Cells Promote Muscle Reinnervation by Responding to Activity-Dependent Postsynaptic Signals","volume":"19","author":"Love","year":"1999","journal-title":"J. Neurosci."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/0896-6273(95)90247-3","article-title":"Nerve Sprouting in Muscle Is Induced and Guided by Processes Extended by Schwann Cells","volume":"14","author":"Son","year":"1995","journal-title":"Neuron"},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.15252\/emmm.201607257","article-title":"CXCL12\u03b1\/SDF-1 from Perisynaptic Schwann Cells Promotes Regeneration of Injured Motor Axon Terminals","volume":"9","author":"Negro","year":"2017","journal-title":"EMBO Mol. Med."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1002\/glia.24184","article-title":"Functional Adaptation of Glial Cells at Neuromuscular Junctions in Response to Injury","volume":"70","author":"Provost","year":"2022","journal-title":"Glia"},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.nbd.2012.08.019","article-title":"Defects in Neuromuscular Junction Remodelling in the Smn2B\/\u2212 Mouse Model of Spinal Muscular Atrophy","volume":"49","author":"Murray","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"ref_168","doi-asserted-by":"crossref","unstructured":"Rimer, M., Seaberg, B.L., Yen, P.-F., Lam, S., Hastings, R.L., Lee, Y.I., Thompson, W.J., Feng, Z., Metzger, F., and Paushkin, S. (2019). Nerve Sprouting Capacity in a Pharmacologically Induced Mouse Model of Spinal Muscular Atrophy. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-44222-2"},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1016\/j.ydbio.2011.05.667","article-title":"Muscles in a Mouse Model of Spinal Muscular Atrophy Show Profound Defects in Neuromuscular Development Even in the Absence of Failure in Neuromuscular Transmission or Loss of Motor Neurons","volume":"356","author":"Lee","year":"2011","journal-title":"Dev. Biol."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.mcn.2015.11.007","article-title":"Central and Peripheral Defects in Motor Units of the Diaphragm of Spinal Muscular Atrophy Mice","volume":"70","author":"Neve","year":"2016","journal-title":"Mol. Cell. Neurosci."},{"key":"ref_171","doi-asserted-by":"crossref","unstructured":"Voigt, A., Herholz, D., Fiesel, F.C., Kaur, K., M\u00fcller, D., Karsten, P., Weber, S.S., Kahle, P.J., Marquardt, T., and Schulz, J.B. (2010). TDP-43-Mediated Neuron Loss in Vivo Requires RNA-Binding Activity. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0012247"},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1111\/nan.12064","article-title":"The Craniosacral Progression of Muscle Development Influences the Emergence of Neuromuscular Junction Alterations in a Severe Murine Model for Spinal Muscular Atrophy","volume":"40","author":"Voigt","year":"2014","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"eabb6871","DOI":"10.1126\/scitranslmed.abb6871","article-title":"Impaired Prenatal Motor Axon Development Necessitates Early Therapeutic Intervention in Severe SMA","volume":"13","author":"Kong","year":"2021","journal-title":"Sci. Transl. Med."},{"key":"ref_174","first-page":"2853","article-title":"Restoration of SMN in Schwann Cells Reverses Myelination Defects and Improves Neuromuscular Function in Spinal Muscular Atrophy","volume":"25","author":"Hunter","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"2235","DOI":"10.1093\/hmg\/ddt612","article-title":"SMN-Dependent Intrinsic Defects in Schwann Cells in Mouse Models of Spinal Muscular Atrophy","volume":"23","author":"Hunter","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1093\/hmg\/ddp506","article-title":"Pre-Symptomatic Development of Lower Motor Neuron Connectivity in a Mouse Model of Severe Spinal Muscular Atrophy","volume":"19","author":"Murray","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1146\/annurev.cellbio.22.010305.104258","article-title":"What Mouse Mutants Teach Us about Extracellular Matrix Function","volume":"22","author":"Legate","year":"2006","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1007\/s12035-007-0026-x","article-title":"Laminins in Peripheral Nerve Development and Muscular Dystrophy","volume":"35","author":"Yu","year":"2007","journal-title":"Mol. Neurobiol."},{"key":"ref_179","doi-asserted-by":"crossref","first-page":"1498","DOI":"10.1002\/glia.20740","article-title":"Regulation of Schwann Cell Function by the Extracellular Matrix","volume":"56","author":"Chernousov","year":"2008","journal-title":"Glia"},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1083\/jcb.200109021","article-title":"Conditional Disruption of \u03b21 Integrin in Schwann Cells Impedes Interactions with Axons","volume":"156","author":"Feltri","year":"2002","journal-title":"J. Cell Biol."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1007\/s13311-016-0477-8","article-title":"Astrocytes: Integrative Regulators of Neuroinflammation in Stroke and Other Neurological Diseases","volume":"13","author":"Cekanaviciute","year":"2016","journal-title":"Neurotherapeutics"},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1186\/s40478-020-01101-6","article-title":"Mitochondrial Defects in the Respiratory Complex I Contribute to Impaired Translational Initiation via ROS and Energy Homeostasis in SMA Motor Neurons","volume":"8","author":"Thelen","year":"2020","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_183","doi-asserted-by":"crossref","unstructured":"Bonanno, S., Cavalcante, P., Salvi, E., Giagnorio, E., Malacarne, C., Cattaneo, M., Andreetta, F., Venerando, A., Pensato, V., and Gellera, C. (2022). Identification of a Cytokine Profile in Serum and Cerebrospinal Fluid of Pediatric and Adult Spinal Muscular Atrophy Patients and Its Modulation upon Nusinersen Treatment. Front. Cell. Neurosci., 16.","DOI":"10.3389\/fncel.2022.982760"},{"key":"ref_184","doi-asserted-by":"crossref","unstructured":"Ohuchi, K., Funato, M., Yoshino, Y., Ando, S., Inagaki, S., Sato, A., Kawase, C., Seki, J., Saito, T., and Nishio, H. (2019). Notch Signaling Mediates Astrocyte Abnormality in Spinal Muscular Atrophy Model Systems. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-39788-w"},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1038\/nm.3806","article-title":"A Small-Molecule Inhibitor of the NLRP3 Inflammasome for the Treatment of Inflammatory Diseases","volume":"21","author":"Coll","year":"2015","journal-title":"Nat. Med."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1177\/09727531211070532","article-title":"Role of JAK\/STAT in the Neuroinflammation and Its Association with Neurological Disorders","volume":"28","author":"Jain","year":"2021","journal-title":"Ann. Neurosci."},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1186\/s12974-020-02024-8","article-title":"The Good, the Bad, and the Opportunities of the Complement System in Neurodegenerative Disease","volume":"17","author":"Schartz","year":"2020","journal-title":"J. Neuroinflamm."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"4280","DOI":"10.1523\/JNEUROSCI.2728-12.2013","article-title":"Shift from Extracellular Signal-Regulated Kinase to AKT\/CAMP Response Element-Binding Protein Pathway Increases Survival-Motor-Neuron Expression in Spinal-Muscular-Atrophy-like Mice and Patient Cells","volume":"33","author":"Branchu","year":"2013","journal-title":"J. Neurosci."},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1007\/s00401-003-0743-9","article-title":"Neuropathological Analysis in Spinal Muscular Atrophy Type II","volume":"106","author":"Araki","year":"2003","journal-title":"Acta Neuropathol."},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1093\/hmg\/11.14.1605","article-title":"Survival Motor Neuron (SMN) Protein: Role in Neurite Outgrowth and Neuromuscular Maturation during Neuronal Differentiation and Development","volume":"11","author":"Fan","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"2935","DOI":"10.1007\/s00431-023-04883-8","article-title":"New Therapies for Spinal Muscular Atrophy: Where We Stand and What Is Next","volume":"182","author":"Antonaci","year":"2023","journal-title":"Eur. J. Pediatr."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1007\/s00401-015-1513-1","article-title":"Astrocytes: A Central Element in Neurological Diseases","volume":"131","author":"Pekny","year":"2015","journal-title":"Acta Neuropathol."},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1038\/s41573-022-00390-x","article-title":"Function and Therapeutic Value of Astrocytes in Neurological Diseases","volume":"21","author":"Lee","year":"2022","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_194","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1093\/hmg\/ddw051","article-title":"Mitochondrial Targeting of XJB-5-131 Attenuates or Improves Pathophysiology in HdhQ150 Animals with Well-Developed Disease Phenotypes","volume":"25","author":"Polyzos","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_195","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1016\/j.cmet.2019.03.004","article-title":"Metabolic Reprogramming in Astrocytes Distinguishes Region-Specific Neuronal Susceptibility in Huntington Mice","volume":"29","author":"Polyzos","year":"2019","journal-title":"Cell Metab."},{"key":"ref_196","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/1098-1136(200010)32:1<1::AID-GLIA10>3.0.CO;2-W","article-title":"Astrocyte Glutamate Transport: Review of Properties, Regulation, and Physiological Functions","volume":"32","author":"Anderson","year":"2000","journal-title":"Glia"},{"key":"ref_197","doi-asserted-by":"crossref","first-page":"10193","DOI":"10.1523\/JNEUROSCI.19-23-10193.1999","article-title":"Glutamate Induces Rapid Upregulation of Astrocyte Glutamate Transport and Cell-Surface Expression of GLAST","volume":"19","author":"Duan","year":"1999","journal-title":"J. Neurosci."},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"eabf1230","DOI":"10.1126\/science.abf1230","article-title":"Barcoded Viral Tracing of Single-Cell Interactions in Central Nervous System Inflammation","volume":"372","author":"Clark","year":"2021","journal-title":"Science"},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"3877","DOI":"10.1007\/s00018-018-2849-1","article-title":"The Role of Survival Motor Neuron Protein (SMN) in Protein Homeostasis","volume":"75","author":"Chaytow","year":"2018","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_200","doi-asserted-by":"crossref","unstructured":"Racki, V., Marcelic, M., Stimac, I., Petric, D., and Kucic, N. (2021). Effects of Haloperidol, Risperidone, and Aripiprazole on the Immunometabolic Properties of BV-2 Microglial Cells. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22094399"},{"key":"ref_201","doi-asserted-by":"crossref","unstructured":"Ku\u010di\u0107, N., Ra\u010dki, V., \u0160verko, R., Vidovi\u0107, T., Grahovac, I., and Mr\u0161i\u0107-Pel\u010di\u0107, J. (2021). Immunometabolic Modulatory Role of Naltrexone in BV-2 Microglia Cells. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22168429"},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1038\/nn.4338","article-title":"A Polarizing Question: Do M1 and M2 Microglia Exist?","volume":"19","author":"Ransohoff","year":"2016","journal-title":"Nat. Neurosci."},{"key":"ref_203","doi-asserted-by":"crossref","first-page":"101651","DOI":"10.1016\/j.smim.2022.101651","article-title":"Redefining Microglia States: Lessons and Limits of Human and Mouse Models to Study Microglia States in Neurodegenerative Diseases","volume":"60","author":"Therrien","year":"2022","journal-title":"Semin. Immunol."},{"key":"ref_204","doi-asserted-by":"crossref","unstructured":"Guo, S., Wang, H., and Yin, Y. (2022). Microglia Polarization from M1 to M2 in Neurodegenerative Diseases. Front. Aging Neurosci., 14.","DOI":"10.3389\/fnagi.2022.815347"},{"key":"ref_205","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.neuroscience.2019.12.005","article-title":"Hydroxytyrosol Inhibits LPS-Induced Neuroinflammatory Responses via Suppression of TLR-4-Mediated NF-\u039aB P65 Activation and ERK Signaling Pathway","volume":"426","author":"Zhang","year":"2019","journal-title":"Neuroscience"},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1038\/nrc.2016.145","article-title":"Notch as a Tumour Suppressor","volume":"17","author":"Nowell","year":"2017","journal-title":"Nat. Rev. Cancer"},{"key":"ref_207","doi-asserted-by":"crossref","unstructured":"Hosseini-Alghaderi, S., and Baron, M. (2020). Notch3 in Development, Health and Disease. Biomolecules, 10.","DOI":"10.3390\/biom10030485"},{"key":"ref_208","doi-asserted-by":"crossref","unstructured":"Cui, W., Sun, C., Ma, Y., Wang, S., Wang, X., and Zhang, Y. (2020). Inhibition of TLR4 Induces M2 Microglial Polarization and Provides Neuroprotection via the NLRP3 Inflammasome in Alzheimer\u2019s Disease. Front. Neurosci., 14.","DOI":"10.3389\/fnins.2020.00444"},{"key":"ref_209","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1016\/S0140-6736(23)01524-6","article-title":"Complement in Human Disease: Approved and Up-And-Coming Therapeutics","volume":"403","author":"West","year":"2024","journal-title":"Lancet"},{"key":"ref_210","doi-asserted-by":"crossref","unstructured":"Jia, J., Cheng, J., Wang, C., and Zhen, X. (2018). Sigma-1 Receptor-Modulated Neuroinflammation in Neurological Diseases. Front. Cell. Neurosci., 12.","DOI":"10.3389\/fncel.2018.00314"},{"key":"ref_211","first-page":"133","article-title":"Sigma-1 Receptors and Neurodegenerative Diseases: Towards a Hypothesis of Sigma-1 Receptors as Amplifiers of Neurodegeneration and Neuroprotection","volume":"Volume 964","author":"Smith","year":"2017","journal-title":"Sigma Receptors: Their Role in Disease and as Therapeutic Targets"},{"key":"ref_212","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1093\/brain\/awu107","article-title":"Pharmacological Stimulation of Sigma-1 Receptors Has Neurorestorative Effects in Experimental Parkinsonism","volume":"137","author":"Francardo","year":"2014","journal-title":"Brain"},{"key":"ref_213","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1124\/jpet.111.182816","article-title":"Afobazole Modulates Microglial Function via Activation of Both \u03c3-1 and \u03c3-2 Receptors","volume":"339","author":"Cuevas","year":"2011","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_214","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1111\/bph.13076","article-title":"The Effects of Sigma (\u03c31) Receptor-Selective Ligands on Muscarinic Receptor Antagonist-Induced Cognitive Deficits in Mice","volume":"172","author":"Malik","year":"2015","journal-title":"Br. J. Pharmacol."},{"key":"ref_215","doi-asserted-by":"crossref","first-page":"137247","DOI":"10.1016\/j.neulet.2023.137247","article-title":"Corrigendum to \u201c(+)-Pentazocine Reduces Oxidative Stress and Apoptosis in Microglia Following Hypoxia\/Reoxygenation Injury\u201d [Neurosci. Lett. 626 142\u2013148]","volume":"807","author":"Heiss","year":"2023","journal-title":"Neurosci. Lett."},{"key":"ref_216","doi-asserted-by":"crossref","unstructured":"Gaja-Capdevila, N., Hern\u00e1ndez, N., Navarro, X., and Herrando-Grabulosa, M. (2021). Sigma-1 Receptor Is a Pharmacological Target to Promote Neuroprotection in the SOD1G93A ALS Mice. Front. Pharmacol., 12.","DOI":"10.3389\/fphar.2021.780588"},{"key":"ref_217","first-page":"1336","article-title":"Sigma 1 Receptor as a Therapeutic Target for Amyotrophic Lateral Sclerosis","volume":"178","author":"Vela","year":"2020","journal-title":"Br. J. Pharmacol."},{"key":"ref_218","doi-asserted-by":"crossref","first-page":"7034143","DOI":"10.1155\/2017\/7034143","article-title":"Microglia in Health and Disease: A Double-Edged Sword","volume":"2017","author":"Santiago","year":"2017","journal-title":"Mediat. Inflamm."},{"key":"ref_219","first-page":"267","article-title":"Role of Neuroinflammation in Neurodegeneration Development","volume":"8","author":"Zhang","year":"2023","journal-title":"Nature"},{"key":"ref_220","first-page":"792","article-title":"An Early and Late Peak in Microglial Activation in Alzheimer\u2019s Disease Trajectory","volume":"140","author":"Fan","year":"2017","journal-title":"Brain A J. Neurol."},{"key":"ref_221","doi-asserted-by":"crossref","first-page":"10634","DOI":"10.1038\/s41467-024-54779-w","article-title":"Homeostatic Microglia Initially Seed and Activated Microglia Later Reshape Amyloid Plaques in Alzheimer\u2019s Disease","volume":"15","author":"Albertini","year":"2024","journal-title":"Nat. Commun."},{"key":"ref_222","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1038\/s41423-019-0223-3","article-title":"The Benefits of Neuroinflammation for the Repair of the Injured Central Nervous System","volume":"16","author":"Yong","year":"2019","journal-title":"Cell. Mol. Immunol."},{"key":"ref_223","doi-asserted-by":"crossref","first-page":"199","DOI":"10.4103\/1673-5374.265541","article-title":"Genetic Targeting of Astrocytes to Combat Neurodegenerative Disease","volume":"15","author":"Kery","year":"2020","journal-title":"Neural Regen. Res."},{"key":"ref_224","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1093\/brain\/awad358","article-title":"Microglia Sense Astrocyte Dysfunction and Prevent Disease Progression in an Alexander Disease Model","volume":"147","author":"Saito","year":"2023","journal-title":"Brain"},{"key":"ref_225","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1038\/s41590-022-01208-z","article-title":"Astrocyte-Targeted Gene Delivery of Interleukin 2 Specifically Increases Brain-Resident Regulatory T Cell Numbers and Protects against Pathological Neuroinflammation","volume":"23","author":"Yshii","year":"2022","journal-title":"Nat. Immunol."},{"key":"ref_226","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1186\/s12974-021-02355-0","article-title":"Microglia and Astrocyte Involvement in Neurodegeneration and Brain Cancer","volume":"18","author":"Vandenbark","year":"2021","journal-title":"J. Neuroinflamm."},{"key":"ref_227","doi-asserted-by":"crossref","first-page":"1794","DOI":"10.1016\/j.immuni.2023.06.016","article-title":"TREM2 Receptor Protects against Complement-Mediated Synaptic Loss by Binding to Complement C1q during Neurodegeneration","volume":"56","author":"Zhong","year":"2023","journal-title":"Immunity"},{"key":"ref_228","doi-asserted-by":"crossref","unstructured":"Morini, R., Bizzotto, M., Perrucci, F., Filipello, F., and Matteoli, M. (2021). Strategies and Tools for Studying Microglial-Mediated Synapse Elimination and Refinement. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.640937"},{"key":"ref_229","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1002\/dneu.22814","article-title":"Microglia Regulate Synaptic Development and Plasticity","volume":"81","author":"Andoh","year":"2021","journal-title":"Dev. Neurobiol."},{"key":"ref_230","doi-asserted-by":"crossref","first-page":"705","DOI":"10.4103\/1673-5374.322423","article-title":"Microglia Regulation of Synaptic Plasticity and Learning and Memory","volume":"17","author":"Cornell","year":"2022","journal-title":"Neural Regen. Res."},{"key":"ref_231","doi-asserted-by":"crossref","unstructured":"Matejuk, A., and Ransohoff, R.M. (2020). Crosstalk between Astrocytes and Microglia: An Overview. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.01416"},{"key":"ref_232","doi-asserted-by":"crossref","first-page":"112010","DOI":"10.1016\/j.celrep.2023.112010","article-title":"Time-Dependent and Selective Microglia-Mediated Removal of Spinal Synapses in Neuropathic Pain","volume":"42","author":"Yousefpour","year":"2023","journal-title":"Cell Rep."},{"key":"ref_233","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1523\/JNEUROSCI.4926-08.2009","article-title":"Astrocyte-Derived MCP-1 Mediates Neuroprotective Effects of Noradrenaline","volume":"29","author":"Madrigal","year":"2009","journal-title":"J. Neurosci."},{"key":"ref_234","first-page":"3078","article-title":"MicroRNA-146a Inhibits NF-\u039aB Activation and Pro-Inflammatory Cytokine Production by Regulating IRAK1 Expression in THP-1 Cells","volume":"18","author":"Zhou","year":"2019","journal-title":"Exp. Ther. Med."},{"key":"ref_235","doi-asserted-by":"crossref","first-page":"7","DOI":"10.3892\/ijmm.2022.5210","article-title":"Mechanisms and Application Strategies of MiRNA-146a Regulating Inflammation and Fibrosis at Molecular and Cellular Levels (Review)","volume":"51","author":"Liao","year":"2022","journal-title":"Int. J. Mol. Med."},{"key":"ref_236","doi-asserted-by":"crossref","unstructured":"Fan, W., Liang, C., Ou, M., Zou, T., Sun, F., Zhou, H., and Cui, L. (2020). MicroRNA-146a Is a Wide-Reaching Neuroinflammatory Regulator and Potential Treatment Target in Neurological Diseases. Front. Mol. Neurosci., 13.","DOI":"10.3389\/fnmol.2020.00090"},{"key":"ref_237","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/1756-8722-5-13","article-title":"The Emerging Role of MIR-146A in the Control of Hematopoiesis, Immune Function and Cancer","volume":"5","author":"Labbaye","year":"2012","journal-title":"J. Hematol. Oncol."},{"key":"ref_238","doi-asserted-by":"crossref","unstructured":"Dull, K., Fazekas, F., De\u00e1k, D., Kov\u00e1cs, D., P\u00f3liska, S., Szegedi, A., Zouboulis, C.C., and T\u00f6r\u0151csik, D. (2021). MiR-146a Modulates TLR1\/2 and 4 Induced Inflammation and Links It with Proliferation and Lipid Production via the Indirect Regulation of GNG7 in Human SZ95 Sebocytes. Sci. Rep., 11.","DOI":"10.1038\/s41598-021-00907-1"},{"key":"ref_239","doi-asserted-by":"crossref","unstructured":"Gilyazova, I., Asadullina, D., Kagirova, E., Sikka, R., Mustafin, A., Ivanova, E., Bakhtiyarova, K., Gilyazova, G., Gupta, S., and Khusnutdinova, E. (2023). MiRNA-146a-A Key Player in Immunity and Diseases. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms241612767"},{"key":"ref_240","doi-asserted-by":"crossref","unstructured":"Chongmelaxme, B., Yodsurang, V., Vichayachaipat, P., Srimatimanon, T., and Sanmaneechai, O. (2024). Gene-Based Therapy for the Treatment of Spinal Muscular Atrophy Types 1 and 2: A Systematic Review and Meta-Analysis. Gene Ther.","DOI":"10.1038\/s41434-024-00503-8"},{"key":"ref_241","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1038\/s43856-023-00256-2","article-title":"Nusinersen Mitigates Neuroinflammation in Severe Spinal Muscular Atrophy Patients","volume":"3","author":"Nuzzo","year":"2023","journal-title":"Commun. Med."},{"key":"ref_242","doi-asserted-by":"crossref","unstructured":"Arslan, D., Ergul-Ulger, Z., Goksen, S., Esendagli, G., Erdem-Ozdamar, S., Tan, E., and Bekircan-Kurt, C.E. (2024). Evaluation of Inflammatory and Glial Cell-Related Biomarkers in Adults with Spinal Muscular Atrophy under Nusinersen Treatment (P2-11.003). Neurology, 102.","DOI":"10.1212\/WNL.0000000000204956"},{"key":"ref_243","doi-asserted-by":"crossref","unstructured":"Zhang, Q., Hong, Y., Brusa, C., Scoto, M., Cornell, N., Patel, P., Baranello, G., Muntoni, F., and Zhou, H. (2024). Profiling Neuroinflammatory Markers and Response to Nusinersen in Paediatric Spinal Muscular Atrophy. Sci. Rep., 14.","DOI":"10.1038\/s41598-024-74338-z"},{"key":"ref_244","doi-asserted-by":"crossref","first-page":"4120","DOI":"10.1038\/s41467-024-48195-3","article-title":"Cell-Mediated Cytotoxicity within CSF and Brain Parenchyma in Spinal Muscular Atrophy Unaltered by Nusinersen Treatment","volume":"15","author":"Lu","year":"2024","journal-title":"Nat. Commun."},{"key":"ref_245","doi-asserted-by":"crossref","unstructured":"Hensel, N., Kubinski, S., and Claus, P. (2020). The Need for SMN-Independent Treatments of Spinal Muscular Atrophy (SMA) to Complement SMN-Enhancing Drugs. Front. Neurol., 11.","DOI":"10.3389\/fneur.2020.00045"},{"key":"ref_246","doi-asserted-by":"crossref","first-page":"S233","DOI":"10.1212\/WNL.47.6_Suppl_4.233S","article-title":"The Pharmacology and Mechanism of Action of Riluzole","volume":"47","author":"Doble","year":"1996","journal-title":"Neurology"},{"key":"ref_247","doi-asserted-by":"crossref","first-page":"7775","DOI":"10.3390\/molecules20057775","article-title":"Riluzole as a Neuroprotective Drug for Spinal Cord Injury: From Bench to Bedside","volume":"20","author":"Nagoshi","year":"2015","journal-title":"Molecules"},{"key":"ref_248","doi-asserted-by":"crossref","first-page":"e240","DOI":"10.1038\/cddis.2011.117","article-title":"A New Vicious Cycle Involving Glutamate Excitotoxicity, Oxidative Stress and Mitochondrial Dynamics","volume":"2","author":"Nguyen","year":"2011","journal-title":"Cell Death Dis."},{"key":"ref_249","doi-asserted-by":"crossref","unstructured":"Zilio, E., Piano, V., and Wirth, B. (2022). Mitochondrial Dysfunction in Spinal Muscular Atrophy. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms231810878"},{"key":"ref_250","doi-asserted-by":"crossref","first-page":"6557","DOI":"10.1523\/JNEUROSCI.1536-12.2013","article-title":"The Neuroprotective Drug Riluzole Acts via Small Conductance Ca2+-Activated K+Channels to Ameliorate Defects in Spinal Muscular Atrophy Models","volume":"33","author":"Dimitriadi","year":"2013","journal-title":"J. Neurosci."},{"key":"ref_251","doi-asserted-by":"crossref","first-page":"1601","DOI":"10.1001\/archneur.60.11.1601","article-title":"A Phase 1 Trial of Riluzole in Spinal Muscular Atrophy","volume":"60","author":"Russman","year":"2003","journal-title":"Arch. Neurol."},{"key":"ref_252","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.ejphar.2017.08.005","article-title":"Edaravone Is a Candidate Agent for Spinal Muscular Atrophy: In Vitro Analysis Using a Human Induced Pluripotent Stem Cells-Derived Disease Model","volume":"814","author":"Ando","year":"2017","journal-title":"Eur. J. Pharmacol."},{"key":"ref_253","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1080\/14656566.2017.1319937","article-title":"Clinical Efficacy of Edaravone for the Treatment of Amyotrophic Lateral Sclerosis","volume":"18","author":"Sawada","year":"2017","journal-title":"Expert Opin. Pharmacother."},{"key":"ref_254","doi-asserted-by":"crossref","first-page":"100346","DOI":"10.1016\/j.xcrm.2021.100346","article-title":"Spinal Muscular Atrophy: From Approved Therapies to Future Therapeutic Targets for Personalized Medicine","volume":"2","author":"Chaytow","year":"2021","journal-title":"Cell Rep. Med."},{"key":"ref_255","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1124\/pharmrev.120.000072","article-title":"Therapeutic Targeting of the Complement System: From Rare Diseases to Pandemics","volume":"73","author":"Garred","year":"2021","journal-title":"Pharmacol. Rev."},{"key":"ref_256","doi-asserted-by":"crossref","first-page":"107342","DOI":"10.1016\/j.neuropsychologia.2020.107342","article-title":"Neuropathology of a Remarkable Case of Memory Impairment Informs Human Memory","volume":"140","author":"Gao","year":"2020","journal-title":"Neuropsychologia"},{"key":"ref_257","unstructured":"Fallon, M., and Tadi, P. (2024, December 01). Histology, Schwann Cells. PubMed, Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK544316\/."},{"key":"ref_258","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1093\/brain\/123.2.222","article-title":"Charcot\u2013Marie\u2013Tooth Disease Type 1","volume":"123","author":"Kamholz","year":"2000","journal-title":"Brain"},{"key":"ref_259","doi-asserted-by":"crossref","unstructured":"O\u2019Carroll, S.J., Cook, W.H., and Young, D. (2021). AAV Targeting of Glial Cell Types in the Central and Peripheral Nervous System and Relevance to Human Gene Therapy. Front. Mol. Neurosci., 13.","DOI":"10.3389\/fnmol.2020.618020"},{"key":"ref_260","doi-asserted-by":"crossref","first-page":"193","DOI":"10.4103\/NRR.NRR-D-23-02055","article-title":"Regeneration Mechanisms and Therapeutic Strategies for Neuromuscular Junctions in Aging and Diseases","volume":"20","author":"Fujitani","year":"2024","journal-title":"Neural Regen. Res."},{"key":"ref_261","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1111\/j.1749-6632.2012.06841.x","article-title":"Neuromuscular Transmission Failure in Myasthenia Gravis: Decrement of Safety Factor and Susceptibility of Extraocular Muscles","volume":"1275","author":"Serra","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_262","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1007\/s00401-011-0848-5","article-title":"The Spinal Muscular Atrophy Mouse Model, SMA\u03947, Displays Altered Axonal Transport without Global Neurofilament Alterations","volume":"122","author":"Dale","year":"2011","journal-title":"Acta Neuropathol."},{"key":"ref_263","doi-asserted-by":"crossref","unstructured":"Boido, M., and Vercelli, A. (2016). Neuromuscular Junctions as Key Contributors and Therapeutic Targets in Spinal Muscular Atrophy. Front. Neuroanat., 10.","DOI":"10.3389\/fnana.2016.00006"},{"key":"ref_264","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1002\/mus.24057","article-title":"Targeting Therapy to the Neuromuscular Junction: Proof of Concept","volume":"49","author":"Kusner","year":"2014","journal-title":"Muscle Nerve"},{"key":"ref_265","unstructured":"Alberts, B. (2024, December 01). Lymphocytes and the Cellular Basis of Adaptive Immunity. Nih.gov, Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK26921\/."},{"key":"ref_266","doi-asserted-by":"crossref","first-page":"2052","DOI":"10.1016\/j.cell.2024.03.037","article-title":"Principles and Therapeutic Applications of Adaptive Immunity","volume":"187","author":"Chi","year":"2024","journal-title":"Cell"},{"key":"ref_267","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1186\/s12974-022-02605-9","article-title":"The Role of the Adaptive Immune System and T Cell Dysfunction in Neurodegenerative Diseases","volume":"19","author":"DeMaio","year":"2022","journal-title":"J. Neuroinflamm."},{"key":"ref_268","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.ccr.2010.06.014","article-title":"The Therapeutic Effect of Anti-HER2\/Neu Antibody Depends on Both Innate and Adaptive Immunity","volume":"18","author":"Park","year":"2010","journal-title":"Cancer Cell"},{"key":"ref_269","doi-asserted-by":"crossref","unstructured":"(2024). Glia Move to the Foreground. Nat. Neurosci., 27, 1427.","DOI":"10.1038\/s41593-024-01735-y"},{"key":"ref_270","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1038\/s41590-021-00913-5","article-title":"Microglia Use TAM Receptors to Detect and Engulf Amyloid Beta Plaques","volume":"22","author":"Huang","year":"2021","journal-title":"Nat. Immunol."},{"key":"ref_271","first-page":"1","article-title":"Role of \u03b1-Synuclein in Inducing Innate and Adaptive Immunity in Parkinson Disease","volume":"5","author":"Standaert","year":"2015","journal-title":"J. Park. Dis."},{"key":"ref_272","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1093\/brain\/awab446","article-title":"Single-Cell Sequencing of Human Midbrain Reveals Glial Activation and a Parkinson-Specific Neuronal State","volume":"145","author":"Landoulsi","year":"2022","journal-title":"Brain J. Neurol."},{"key":"ref_273","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1016\/j.tins.2015.04.003","article-title":"Glial Fibrillary Acidic Protein: From Intermediate Filament Assembly and Gliosis to Neurobiomarker","volume":"38","author":"Yang","year":"2015","journal-title":"Trends Neurosci."},{"key":"ref_274","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1111\/bpa.12601","article-title":"Alexander Disease: An Astrocytopathy That Produces a Leukodystrophy","volume":"28","author":"Sosunov","year":"2018","journal-title":"Brain Pathol."},{"key":"ref_275","doi-asserted-by":"crossref","first-page":"2057","DOI":"10.1242\/jcs.02339","article-title":"Alexander-Disease Mutation of GFAP Causes Filament Disorganization and Decreased Solubility of GFAP","volume":"118","author":"Hsiao","year":"2005","journal-title":"J. Cell Sci."},{"key":"ref_276","doi-asserted-by":"crossref","first-page":"111942","DOI":"10.1016\/j.celrep.2022.111942","article-title":"Mutations in the Transcriptional Regulator MeCP2 Severely Impact Key Cellular and Molecular Signatures of Human Astrocytes during Maturation","volume":"42","author":"Sun","year":"2023","journal-title":"Cell Rep."},{"key":"ref_277","doi-asserted-by":"crossref","unstructured":"D\u2019Antoni, S., Spatuzza, M., Bonaccorso, C.M., and Catania, M.V. (2024). Role of Fragile X Messenger Ribonucleoprotein 1 in the Pathophysiology of Brain Disorders: A Glia Perspective. Neurosci. Biobehav. Rev., 162.","DOI":"10.1016\/j.neubiorev.2024.105731"},{"key":"ref_278","doi-asserted-by":"crossref","first-page":"18764","DOI":"10.1523\/JNEUROSCI.2657-13.2013","article-title":"Oligodendrocyte Lineage Cells Contribute Unique Features to Rett Syndrome Neuropathology","volume":"33","author":"Nguyen","year":"2013","journal-title":"J. Neurosci."},{"key":"ref_279","doi-asserted-by":"crossref","unstructured":"Krasovska, V., and Doering, L.C. (2018). Regulation of IL-6 Secretion by Astrocytes via TLR4 in the Fragile X Mouse Model. Front. Mol. Neurosci., 11.","DOI":"10.3389\/fnmol.2018.00272"},{"key":"ref_280","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1016\/j.ijdevneu.2013.09.011","article-title":"Non-Neuronal Cell Responses Differ between Normal and down Syndrome Developing Brains","volume":"31","author":"Kanaumi","year":"2013","journal-title":"Int. J. Dev. Neurosci."},{"key":"ref_281","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.neurobiolaging.2018.11.009","article-title":"Striking Reduction in Neurons and Glial Cells in Anterior Thalamic Nuclei of Older Patients with down Syndrome","volume":"75","author":"Perry","year":"2019","journal-title":"Neurobiol. Aging"},{"key":"ref_282","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1111\/bpa.12600","article-title":"Astrocytes, an Active Player in Aicardi-Gouti\u00e8res Syndrome","volume":"28","author":"Sase","year":"2018","journal-title":"Brain Pathol."},{"key":"ref_283","doi-asserted-by":"crossref","unstructured":"Mishra, S., Kanchan, S., Divakar, A., Jha, G., Sharma, D., Kapoor, R., and Kumar Rath, S. (2023). Alcohol Induces ER Stress and Apoptosis by Inducing Oxidative Stress and Disruption of Calcium Homeostasis in Glial Cells. Food Chem. Toxicol. Int. J. Publ. Br. Ind. Biol. Res. Assoc., 182.","DOI":"10.1016\/j.fct.2023.114192"},{"key":"ref_284","doi-asserted-by":"crossref","first-page":"100135","DOI":"10.1016\/j.addicn.2023.100135","article-title":"Glial Dysfunction in Substance Use Disorders. New Insights from PET and MR Imaging","volume":"9","author":"Saba","year":"2023","journal-title":"Addict. Neurosci."},{"key":"ref_285","doi-asserted-by":"crossref","first-page":"7965","DOI":"10.1523\/JNEUROSCI.0284-20.2020","article-title":"Alcohol Increases Exosome Release from Microglia to Promote Complement C1q-Induced Cellular Death of Proopiomelanocortin Neurons in the Hypothalamus in a Rat Model of Fetal Alcohol Spectrum Disorders","volume":"40","author":"Mukherjee","year":"2020","journal-title":"J. Neurosci."},{"key":"ref_286","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.it.2015.12.007","article-title":"The Role of Adaptive Immunity in the Efficacy of Targeted Cancer Therapies","volume":"37","author":"Xu","year":"2016","journal-title":"Trends Immunol."},{"key":"ref_287","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1038\/s41577-020-0285-6","article-title":"Defining Trained Immunity and Its Role in Health and Disease","volume":"20","author":"Netea","year":"2020","journal-title":"Nat. Rev. Immunol."}],"container-title":["Neurology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2035-8377\/17\/3\/41\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T16:53:08Z","timestamp":1760028788000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2035-8377\/17\/3\/41"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,13]]},"references-count":287,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,3]]}},"alternative-id":["neurolint17030041"],"URL":"https:\/\/doi.org\/10.3390\/neurolint17030041","relation":{},"ISSN":["2035-8377"],"issn-type":[{"value":"2035-8377","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,13]]}}}