{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:54:59Z","timestamp":1790805299529,"version":"4.1.0"},"reference-count":158,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T00:00:00Z","timestamp":1760659200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003196","name":"Italian Ministry of Health","doi-asserted-by":"publisher","award":["PNRR-POC-2023-12377653"],"award-info":[{"award-number":["PNRR-POC-2023-12377653"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"name":"The Italian Ministry of Education and Research"},{"name":"Dipartimenti di Eccellenza Program","award":["2023\u20132027"],"award-info":[{"award-number":["2023\u20132027"]}]},{"name":"Dept. of Pathophysiology and Transplantation"},{"DOI":"10.13039\/100012352","name":"University of Milan","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012352","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Hub Life Science- Diagnostica Avanzata","award":["PNC-E3-2022-23683266"],"award-info":[{"award-number":["PNC-E3-2022-23683266"]}]},{"DOI":"10.13039\/501100002862","name":"CUP","doi-asserted-by":"publisher","award":["C43C22001630001"],"award-info":[{"award-number":["C43C22001630001"]}],"id":[{"id":"10.13039\/501100002862","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Piano Nazionale Complementare Ecosistema Innovativo della Salute"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Spinal muscular atrophy (SMA) is a devastating neurodegenerative disorder caused by SMN1 gene mutations that lead to compromised production of survival motor neuron (SMN) protein. The inflammatory footprint of SMA extends beyond neural tissues, leading to significant inflammatory manifestations across multiple body systems. In motor neurons, several molecular pathways contribute to local degeneration: cytokine activation, purinergic signalling, NF-\u03baB pathway stimulation, complement cascade activation and glutamate excitotoxicity. These mechanisms, which involve reactive glial and immune cells, trigger neuroinflammation and non-cell-autonomous death processes that can affect non-neural organ systems.<\/jats:p>\n                  <jats:p>Biological samples from patients with SMA and model systems of SMA consistently exhibit altered immune patterns, elevated inflammatory markers, and immunocyte and glial dysfunction. Even revolutionary SMN-restoring therapy for SMA may not treat the potential inflammation-related aspects of the disease. Current research has identified both anti-inflammatory and pro-inflammatory effects and outcomes after SMN restoration, with unclear implications.<\/jats:p>\n                  <jats:p>Preclinical investigations targeting inflammation in SMA models have yielded promising findings, although optimal intervention timing requires refinement. Despite these encouraging results, translation to clinical practice remains unrealized. The field requires advanced investigative approaches, particularly single-cell RNA sequencing, to elucidate inflammatory molecular pathways, identify novel biomarkers for early detection and develop targeted anti-inflammatory agents that complement SMN-augmenting therapies.<\/jats:p>\n                  <jats:p>Understanding the inflammatory mechanisms associated with SMA is crucial for developing effective combination therapies addressing both SMN deficiency and inflammatory processes. Elucidating inflammation timing and mechanisms will inform optimal intervention windows and identify patients most likely to benefit from combined treatment approaches. Future research should prioritize personalized strategies targeting both SMN-dependent and inflammatory pathways.<\/jats:p>","DOI":"10.1093\/brain\/awaf392","type":"journal-article","created":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T12:35:43Z","timestamp":1760618143000},"page":"404-421","source":"Crossref","is-referenced-by-count":6,"title":["Decoding inflammatory pathways in spinal muscular atrophy: implications for next-generation therapies"],"prefix":"10.1093","volume":"149","author":[{"given":"Linda","family":"Ottoboni","sequence":"first","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milano 20122 ,","place":["Italy"]},{"name":"Neurology Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milano 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Bruno","sequence":"additional","affiliation":[{"name":"Center of Translational and Experimental Myology, IRCCS Istituto Giannina Gaslini , Genova ,","place":["Italy"]},{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health-DINOGMI, University of Genoa , Genova ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5425-969X","authenticated-orcid":false,"given":"Stefania","family":"Corti","sequence":"additional","affiliation":[{"name":"Dino Ferrari Center, Department of Pathophysiology and Transplantation (DEPT), University of Milan , Milano 20122 ,","place":["Italy"]},{"name":"Neuromuscular and Rare Diseases Unit, Department of Neurosciences, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico , Milano 20122 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,10,17]]},"reference":[{"key":"2026020710024931800_awaf392-B1","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1038\/s41582-020-00413-4","article-title":"Spinal muscular atrophy\u2014Insights and challenges in the treatment era","volume":"16","author":"Mercuri","year":"2020","journal-title":"Nat Rev Neurol"},{"key":"2026020710024931800_awaf392-B2","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"2026020710024931800_awaf392-B3","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/j.bbagrm.2016.12.008","article-title":"Diverse role of survival motor neuron protein","volume":"1860","author":"Singh","year":"2017","journal-title":"Biochim Biophys Acta Gene Regul Mech"},{"key":"2026020710024931800_awaf392-B4","doi-asserted-by":"crossref","first-page":"100878","DOI":"10.1016\/j.spen.2021.100878","article-title":"Spinal muscular atrophy","volume":"37","author":"Nicolau","year":"2021","journal-title":"Semin Pediatr Neurol"},{"key":"2026020710024931800_awaf392-B5","doi-asserted-by":"crossref","first-page":"6307","DOI":"10.1073\/pnas.96.11.6307","article-title":"A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy","volume":"96","author":"Lorson","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2026020710024931800_awaf392-B6","doi-asserted-by":"crossref","first-page":"1","DOI":"10.21926\/obm.genet.2302182","article-title":"Spinal muscular atrophy: An overview","volume":"7","author":"Ross","year":"2023","journal-title":"OBM Genet"},{"key":"2026020710024931800_awaf392-B7","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1186\/1750-1172-6-71","article-title":"Spinal muscular atrophy","volume":"6","author":"D\u2019Amico","year":"2011","journal-title":"Orphanet J Rare Dis"},{"key":"2026020710024931800_awaf392-B8","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1086\/338627","article-title":"Quantitative analyses of SMN1 and SMN2 based on real-time LightCycler PCR: Fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophy","volume":"70","author":"Feldk\u00f6tter","year":"2002","journal-title":"Am J Hum Genet"},{"key":"2026020710024931800_awaf392-B9","doi-asserted-by":"crossref","first-page":"2155","DOI":"10.1002\/acn3.51889","article-title":"Combination disease-modifying treatment in spinal muscular atrophy: A proposed classification","volume":"10","author":"Proud","year":"2023","journal-title":"Ann Clin Transl Neurol"},{"key":"2026020710024931800_awaf392-B10","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/S1474-4422(23)00419-2","article-title":"Challenges and opportunities in spinal muscular atrophy therapeutics","volume":"23","author":"Yeo","year":"2024","journal-title":"Lancet Neurol"},{"key":"2026020710024931800_awaf392-B11","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1016\/j.nmd.2022.06.005","article-title":"264th ENMC International Workshop: Multi-system involvement in spinal muscular atrophy Hoofddorp, The Netherlands, November 19th\u201321st 2021","volume":"32","author":"Detering","year":"2022","journal-title":"Neuromuscul Disord"},{"key":"2026020710024931800_awaf392-B12","doi-asserted-by":"crossref","first-page":"1375428","DOI":"10.3389\/fimmu.2024.1375428","article-title":"What could be the function of the spinal muscular atrophy-causing protein SMN in macrophages?","volume":"15","author":"Tapken","year":"2024","journal-title":"Front Immunol"},{"key":"2026020710024931800_awaf392-B13","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-\u03baB signaling","volume":"1864","author":"Kim","year":"2017","journal-title":"Biochim Biophys Acta Mol Cell Res"},{"key":"2026020710024931800_awaf392-B14","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":"2026020710024931800_awaf392-B15","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1038\/s41582-020-00411-6","article-title":"Spinal muscular atrophy\u2014Challenges in the therapeutic era","volume":"16","author":"Faravelli","year":"2020","journal-title":"Nat Rev Neurol"},{"key":"2026020710024931800_awaf392-B16","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1038\/nrneurol.2015.77","article-title":"Spinal muscular atrophy\u2014Recent therapeutic advances for an old challenge","volume":"11","author":"Faravelli","year":"2015","journal-title":"Nat Rev Neurol"},{"key":"2026020710024931800_awaf392-B17","doi-asserted-by":"crossref","first-page":"104870","DOI":"10.1016\/j.nbd.2020.104870","article-title":"Glial cells involvement in spinal muscular atrophy: Could SMA be a neuroinflammatory disease?","volume":"140","author":"Abati","year":"2020","journal-title":"Neurobiol Dis"},{"key":"2026020710024931800_awaf392-B18","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1016\/j.tins.2022.08.001","article-title":"How the immune system shapes neurodegenerative diseases","volume":"45","author":"Mason","year":"2022","journal-title":"Trends Neurosci"},{"key":"2026020710024931800_awaf392-B19","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":"2026020710024931800_awaf392-B20","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1038\/s41392-024-01743-1","article-title":"Microbiota\u2013gut\u2013brain axis and its therapeutic applications in neurodegenerative diseases","volume":"9","author":"Loh","year":"2024","journal-title":"Signal Transduct Target Ther"},{"key":"2026020710024931800_awaf392-B21","first-page":"100479","article-title":"Gut microbiota in muscular atrophy development, progression, and treatment: New therapeutic targets and opportunities","volume":"4","author":"Chen","year":"2023","journal-title":"Innovation (Camb)"},{"key":"2026020710024931800_awaf392-B22","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1016\/j.tins.2021.04.008","article-title":"Non-cell-autonomous pathogenic mechanisms in amyotrophic lateral sclerosis","volume":"44","author":"Van Harten","year":"2021","journal-title":"Trends Neurosci"},{"key":"2026020710024931800_awaf392-B23","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":"2026020710024931800_awaf392-B24","doi-asserted-by":"crossref","first-page":"884748","DOI":"10.3389\/fcell.2022.884748","article-title":"Single-cell and single-nuclei RNA sequencing as powerful tools to decipher cellular heterogeneity and dysregulation in neurodegenerative diseases","volume":"10","author":"Cuevas-Diaz Duran","year":"2022","journal-title":"Front Cell Dev Biol"},{"key":"2026020710024931800_awaf392-B25","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1016\/j.celrep.2020.01.010","article-title":"Microglia heterogeneity in the single-cell era","volume":"30","author":"Masuda","year":"2020","journal-title":"Cell Rep"},{"key":"2026020710024931800_awaf392-B26","volume-title":"Spinal muscular atrophy","author":"Sumner","year":"2017"},{"key":"2026020710024931800_awaf392-B27","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":"2026020710024931800_awaf392-B28","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":"2026020710024931800_awaf392-B29","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":"2026020710024931800_awaf392-B30","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":"2026020710024931800_awaf392-B31","doi-asserted-by":"crossref","first-page":"882","DOI":"10.4103\/0028-3886.293477","article-title":"Spinal muscular atrophy: Autopsy based neuropathological demonstration","volume":"68","author":"Thirunavukkarasu","year":"2020","journal-title":"Neurol India"},{"key":"2026020710024931800_awaf392-B32","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1111\/neup.12784","article-title":"Spinal muscular atrophy type 2 patient who survived 61 years: An autopsy case report","volume":"42","author":"Yamadera","year":"2022","journal-title":"Neuropathology"},{"key":"2026020710024931800_awaf392-B33","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1093\/hmg\/ddae190","article-title":"Motor pool selectivity of neuromuscular degeneration in type I spinal muscular atrophy is conserved between human and mouse","volume":"34","author":"Lee","year":"2025","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B34","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":"2026020710024931800_awaf392-B35","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":"2026020710024931800_awaf392-B36","doi-asserted-by":"crossref","first-page":"eadi7828","DOI":"10.1126\/scitranslmed.adi7828","article-title":"Neuroinflammation: An astrocyte perspective","volume":"15","author":"Lee","year":"2023","journal-title":"Sci Transl Med"},{"key":"2026020710024931800_awaf392-B37","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1038\/s41582-020-00435-y","article-title":"Neuroinflammation and microglial activation in Alzheimer disease: Where do we go from here?","volume":"17","author":"Leng","year":"2021","journal-title":"Nat Rev Neurol"},{"key":"2026020710024931800_awaf392-B38","doi-asserted-by":"crossref","first-page":"158","DOI":"10.3390\/brainsci14020158","article-title":"The diverse roles of reactive astrocytes in the pathogenesis of amyotrophic lateral sclerosis","volume":"14","author":"Yang","year":"2024","journal-title":"Brain Sci"},{"key":"2026020710024931800_awaf392-B39","doi-asserted-by":"crossref","first-page":"1047540","DOI":"10.3389\/fmmed.2023.1047540","article-title":"Why should we care about astrocytes in a motor neuron disease?","volume":"3","author":"Stoklund Dittlau","year":"2023","journal-title":"Front Mol Med"},{"key":"2026020710024931800_awaf392-B40","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.neures.2017.09.011","article-title":"The multi-dimensional roles of astrocytes in ALS","volume":"126","author":"Yamanaka","year":"2018","journal-title":"Neurosci Res"},{"key":"2026020710024931800_awaf392-B41","doi-asserted-by":"crossref","first-page":"100117","DOI":"10.1016\/j.neurop.2023.100117","article-title":"The role of astrocytes with genetic mutations linked to amyotrophic lateral sclerosis","volume":"3","author":"Brash-Arias","year":"2023","journal-title":"Neurol Perspect"},{"key":"2026020710024931800_awaf392-B42","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1097\/WCO.0000000000001294","article-title":"The genetics of amyotrophic lateral sclerosis","volume":"37","author":"Nijs","year":"2024","journal-title":"Curr Opin Neurol"},{"key":"2026020710024931800_awaf392-B43","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/B978-0-443-19102-2.00004-1","article-title":"Neuroglia in neurodegeneration: Amyotrophic lateral sclerosis and frontotemporal dementia","volume":"210","author":"De Marchi","year":"2025","journal-title":"Handb Clin Neurol"},{"key":"2026020710024931800_awaf392-B44","doi-asserted-by":"crossref","first-page":"41","DOI":"10.3390\/neurolint17030041","article-title":"Glial cells in spinal muscular atrophy: Speculations on non-cell-autonomous mechanisms and therapeutic implications","volume":"17","author":"Belan\u010di\u0107","year":"2025","journal-title":"Neurol Int"},{"key":"2026020710024931800_awaf392-B45","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1186\/1750-1326-4-20","article-title":"Calcium signaling in neurodegeneration","volume":"4","author":"Marambaud","year":"2009","journal-title":"Mol Neurodegener"},{"key":"2026020710024931800_awaf392-B46","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1038\/nrn2670","article-title":"Spinal muscular atrophy: Why do low levels of survival motor neuron protein make motor neurons sick?","volume":"10","author":"Burghes","year":"2009","journal-title":"Nat Rev Neurosci"},{"key":"2026020710024931800_awaf392-B47","doi-asserted-by":"crossref","first-page":"5579","DOI":"10.1038\/s41467-020-19177-y","article-title":"Systematic elucidation of neuron-astrocyte interaction in models of amyotrophic lateral sclerosis using multi-modal integrated bioinformatics workflow","volume":"11","author":"Mishra","year":"2020","journal-title":"Nat Commun"},{"key":"2026020710024931800_awaf392-B48","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1016\/j.neuroscience.2024.06.023","article-title":"Lactate: A new target for brain disorders","volume":"552","author":"Liu","year":"2024","journal-title":"Neuroscience"},{"key":"2026020710024931800_awaf392-B49","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1002\/glia.23114","article-title":"Wildtype motoneurons, ALS-linked SOD1 mutation and glutamate profoundly modify astrocyte metabolism and lactate shuttling","volume":"65","author":"Madji Hounoum","year":"2017","journal-title":"Glia"},{"key":"2026020710024931800_awaf392-B50","first-page":"801","article-title":"Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice","volume":"26","author":"Deguise","year":"2017","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B51","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":"2016","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B52","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":"2026020710024931800_awaf392-B53","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":"2026020710024931800_awaf392-B54","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":"2026020710024931800_awaf392-B55","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":"2026020710024931800_awaf392-B56","doi-asserted-by":"crossref","first-page":"1488951","DOI":"10.3389\/fphys.2024.1488951","article-title":"Spinal TNF-\u03b1 receptor 1 is differentially required for phrenic long-term facilitation (pLTF) over the course of motor neuron death in adult rats","volume":"15","author":"Lewis","year":"2024","journal-title":"Front Physiol"},{"key":"2026020710024931800_awaf392-B57","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":"2026020710024931800_awaf392-B58","doi-asserted-by":"crossref","first-page":"242","DOI":"10.3389\/fnagi.2017.00242","article-title":"The dual role of microglia in ALS: Mechanisms and therapeutic approaches","volume":"9","author":"Geloso","year":"2017","journal-title":"Front Aging Neurosci"},{"key":"2026020710024931800_awaf392-B59","doi-asserted-by":"crossref","first-page":"102840","DOI":"10.1016\/j.conb.2024.102840","article-title":"Astrocytic crosstalk with brain and immune cells in healthy and diseased conditions","volume":"84","author":"Lee","year":"2024","journal-title":"Curr Opin Neurobiol"},{"key":"2026020710024931800_awaf392-B60","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.immuni.2021.01.013","article-title":"Astrocyte-immune cell interactions in physiology and pathology","volume":"54","author":"Han","year":"2021","journal-title":"Immunity"},{"key":"2026020710024931800_awaf392-B61","doi-asserted-by":"crossref","first-page":"872134","DOI":"10.3389\/fnagi.2022.872134","article-title":"The emerging role of central and peripheral immune systems in neurodegenerative diseases","volume":"14","author":"Zang","year":"2022","journal-title":"Front Aging Neurosci"},{"key":"2026020710024931800_awaf392-B62","doi-asserted-by":"crossref","first-page":"1946","DOI":"10.1016\/j.ymthe.2021.04.020","article-title":"Extracellular vesicles in neuroinflammation: Pathogenesis, diagnosis, and therapy","volume":"29","author":"Ruan","year":"2021","journal-title":"Mol Ther"},{"key":"2026020710024931800_awaf392-B63","doi-asserted-by":"crossref","first-page":"5674","DOI":"10.1038\/s41598-025-90083-3","article-title":"Extracellular vesicles efficiently deliver survival motor neuron protein to cells in culture","volume":"15","author":"Ren\u00e9","year":"2025","journal-title":"Sci Rep"},{"key":"2026020710024931800_awaf392-B64","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/978-1-4939-9658-2_4","article-title":"Microglia reactivity: Heterogeneous pathological phenotypes","volume":"2034","author":"Hirbec","year":"2019","journal-title":"Methods Mol Biol"},{"key":"2026020710024931800_awaf392-B65","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":"2026020710024931800_awaf392-B66","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":"2026020710024931800_awaf392-B67","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":"2026020710024931800_awaf392-B68","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1038\/nn1715","article-title":"Control of microglial neurotoxicity by the fractalkine receptor","volume":"9","author":"Cardona","year":"2006","journal-title":"Nat Neurosci"},{"key":"2026020710024931800_awaf392-B69","doi-asserted-by":"crossref","first-page":"32713","DOI":"10.1074\/jbc.M111.254268","article-title":"CX3CR1 protein signaling modulates microglial activation and protects against plaque-independent cognitive deficits in a mouse model of Alzheimer disease","volume":"286","author":"Cho","year":"2011","journal-title":"J Biol Chem"},{"key":"2026020710024931800_awaf392-B70","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1002\/glia.23529","article-title":"Enforced microglial depletion and repopulation as a promising strategy for the treatment of neurological disorders","volume":"67","author":"Han","year":"2019","journal-title":"Glia"},{"key":"2026020710024931800_awaf392-B71","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1007\/s11064-022-03742-6","article-title":"Strategies for manipulating microglia to determine their role in the healthy and diseased brain","volume":"48","author":"Parajuli","year":"2023","journal-title":"Neurochem Res"},{"key":"2026020710024931800_awaf392-B72","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1002\/jnr.24114","article-title":"Myelin as an inflammatory mediator: Myelin interactions with complement, macrophages, and microglia in spinal cord injury","volume":"96","author":"Kopper","year":"2018","journal-title":"J Neurosci Res"},{"key":"2026020710024931800_awaf392-B73","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":"2026020710024931800_awaf392-B74","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1186\/s40478-023-01551-8","article-title":"Boosting neuregulin 1 type-III expression hastens SMA motor axon maturation","volume":"11","author":"Kong","year":"2023","journal-title":"Acta Neuropathol Commun"},{"key":"2026020710024931800_awaf392-B75","doi-asserted-by":"crossref","first-page":"6","DOI":"10.3390\/neurolint17010006","article-title":"The rise of pluripotent stem cell-derived glia models of neuroinflammation","volume":"17","author":"Kala","year":"2025","journal-title":"Neurol Int"},{"key":"2026020710024931800_awaf392-B76","doi-asserted-by":"crossref","first-page":"116","DOI":"10.3389\/fncel.2019.00116","article-title":"Emerging role of Schwann cells in neuropathic pain: Receptors, glial mediators and myelination","volume":"13","author":"Wei","year":"2019","journal-title":"Front Cell Neurosci"},{"key":"2026020710024931800_awaf392-B77","doi-asserted-by":"crossref","first-page":"1179011","DOI":"10.3389\/fnins.2023.1179011","article-title":"Notch signaling as a master regulator of adult neurogenesis","volume":"17","author":"Lampada","year":"2023","journal-title":"Front Neurosci"},{"key":"2026020710024931800_awaf392-B78","doi-asserted-by":"crossref","first-page":"103590","DOI":"10.1016\/j.mcn.2021.103590","article-title":"Macrophage roles in peripheral nervous system injury and pathology: Allies in neuromuscular junction recovery","volume":"111","author":"Rios","year":"2021","journal-title":"Mol Cell Neurosci"},{"key":"2026020710024931800_awaf392-B79","doi-asserted-by":"crossref","first-page":"101192","DOI":"10.1016\/j.isci.2020.101192","article-title":"Immunosuppressive functions of M2 macrophages derived from iPSCs of patients with ALS and healthy controls","volume":"23","author":"Zhao","year":"2020","journal-title":"iScience"},{"key":"2026020710024931800_awaf392-B80","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1177\/1073858416687249","article-title":"Neuroinflammation, mast cells, and glia: Dangerous liaisons","volume":"23","author":"Skaper","year":"2017","journal-title":"Neuroscientist"},{"key":"2026020710024931800_awaf392-B81","doi-asserted-by":"crossref","first-page":"785","DOI":"10.3390\/cells13090785","article-title":"Systemic LPS administration stimulates the activation of non-neuronal cells in an experimental model of spinal muscular atrophy","volume":"13","author":"Karafoulidou","year":"2024","journal-title":"Cells"},{"key":"2026020710024931800_awaf392-B82","doi-asserted-by":"crossref","first-page":"6964","DOI":"10.3390\/jcm11236964","article-title":"Research evidence of the role of the glymphatic system and its potential pharmacological modulation in neurodegenerative diseases","volume":"11","author":"Verghese","year":"2022","journal-title":"J Clin Med"},{"key":"2026020710024931800_awaf392-B83","doi-asserted-by":"crossref","first-page":"7311","DOI":"10.3390\/ijms25137311","article-title":"Beyond motor neurons in spinal muscular atrophy: A focus on neuromuscular junction","volume":"25","author":"Torri","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2026020710024931800_awaf392-B84","doi-asserted-by":"crossref","first-page":"3520","DOI":"10.3390\/ijms25063520","article-title":"Disruption of neuromuscular junction following spinal cord injury and motor neuron diseases","volume":"25","author":"Nemeth","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2026020710024931800_awaf392-B85","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1016\/j.autrev.2017.05.015","article-title":"Autophagy in neuroinflammatory diseases","volume":"16","author":"Muller","year":"2017","journal-title":"Autoimmun Rev"},{"key":"2026020710024931800_awaf392-B86","doi-asserted-by":"crossref","first-page":"739","DOI":"10.14336\/AD.2023.0718-1","article-title":"Autophagy in neuroinflammation: A focus on epigenetic regulation","volume":"15","author":"Chen","year":"2024","journal-title":"Aging Dis"},{"key":"2026020710024931800_awaf392-B87","doi-asserted-by":"crossref","first-page":"115664","DOI":"10.1016\/j.bcp.2023.115664","article-title":"Oxidative stress: Roles in skeletal muscle atrophy","volume":"214","author":"Zhang","year":"2023","journal-title":"Biochem Pharmacol"},{"key":"2026020710024931800_awaf392-B88","first-page":"1","article-title":"Fatigue in spinal muscular atrophy: A fundamental open issue","volume":"43","author":"Crisafulli","year":"2024","journal-title":"Acta Myol"},{"key":"2026020710024931800_awaf392-B89","doi-asserted-by":"crossref","first-page":"1844","DOI":"10.1093\/hmg\/ddr067","article-title":"Increased IGF-1 in muscle modulates the phenotype of severe SMA mice","volume":"20","author":"Bosch-Marc\u00e9","year":"2011","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B90","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1038\/labinvest.3700163","article-title":"Reduced expression of nicotinic AChRs in myotubes from spinal muscular atrophy I patients","volume":"84","author":"Arnold","year":"2004","journal-title":"Lab Invest"},{"key":"2026020710024931800_awaf392-B91","doi-asserted-by":"crossref","first-page":"4301","DOI":"10.1093\/hmg\/dds263","article-title":"Hypoxia is a modifier of SMN2 splicing and disease severity in a severe SMA mouse model","volume":"21","author":"Bebee","year":"2012","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B92","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s10571-024-01508-y","article-title":"Genetic variability in oxidative stress, inflammatory, and neurodevelopmental pathways: Impact on the susceptibility and course of spinal muscular atrophy","volume":"44","author":"Barbo","year":"2024","journal-title":"Cell Mol Neurobiol"},{"key":"2026020710024931800_awaf392-B93","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1001\/jamaneurol.2015.0178","article-title":"Impaired muscle mitochondrial biogenesis and myogenesis in spinal muscular atrophy","volume":"72","author":"Ripolone","year":"2015","journal-title":"JAMA Neurol"},{"key":"2026020710024931800_awaf392-B94","doi-asserted-by":"crossref","first-page":"120067","DOI":"10.1016\/j.lfs.2021.120067","article-title":"Enhanced pro-BDNF-p75NTR pathway activity in denervated skeletal muscle","volume":"286","author":"Aby","year":"2021","journal-title":"Life Sci"},{"key":"2026020710024931800_awaf392-B95","doi-asserted-by":"crossref","first-page":"10064","DOI":"10.4081\/ejtm.2022.10064","article-title":"Involvement of muscle satellite cell dysfunction in neuromuscular disorders: Expanding the portfolio of satellite cell-opathies","volume":"32","author":"Ganassi","year":"2022","journal-title":"Eur J Transl Myol"},{"key":"2026020710024931800_awaf392-B96","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1093\/hmg\/ddae162","article-title":"SMN depletion impairs skeletal muscle formation and maturation in a mouse model of SMA","volume":"34","author":"Liu","year":"2025","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B97","doi-asserted-by":"crossref","first-page":"1084068","DOI":"10.3389\/fcell.2023.1084068","article-title":"Single cell analysis reveals satellite cell heterogeneity for proinflammatory chemokine expression","volume":"11","author":"Andre","year":"2023","journal-title":"Front Cell Dev Biol"},{"key":"2026020710024931800_awaf392-B98","doi-asserted-by":"crossref","first-page":"e153430","DOI":"10.1172\/JCI153430","article-title":"Microvasculopathy in spinal muscular atrophy is driven by a reversible autonomous endothelial cell defect","volume":"132","author":"Zhou","year":"2022","journal-title":"J Clin Invest"},{"key":"2026020710024931800_awaf392-B99","first-page":"932","article-title":"SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of Spinal Muscular Atrophy","volume":"26","author":"Khairallah","year":"2017","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B100","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1016\/j.ebiom.2018.04.024","article-title":"Interventions targeting glucocorticoid-Kr\u00fcppel-like factor 15-branched-chain amino acid signaling improve disease phenotypes in spinal muscular atrophy mice","volume":"31","author":"Walter","year":"2018","journal-title":"EBioMedicine"},{"key":"2026020710024931800_awaf392-B101","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1002\/ana.24549","article-title":"Vascular defects and spinal cord hypoxia in spinal muscular atrophy","volume":"79","author":"Somers","year":"2016","journal-title":"Ann Neurol"},{"key":"2026020710024931800_awaf392-B102","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1111\/j.1365-2982.2012.01906.x","article-title":"Microbes and the gut-brain axis","volume":"24","author":"Bercik","year":"2012","journal-title":"Neurogastroenterol Motil"},{"key":"2026020710024931800_awaf392-B103","doi-asserted-by":"crossref","first-page":"101802","DOI":"10.1016\/j.smim.2023.101802","article-title":"The gut-brain vascular axis in neuroinflammation","volume":"69","author":"Carloni","year":"2023","journal-title":"Semin Immunol"},{"key":"2026020710024931800_awaf392-B104","doi-asserted-by":"crossref","first-page":"2956","DOI":"10.3390\/nu15132956","article-title":"Dietary implications of the bidirectional relationship between the gut microflora and inflammatory diseases with special emphasis on irritable bowel disease: Current and future perspective","volume":"15","author":"Aziz","year":"2023","journal-title":"Nutrients"},{"key":"2026020710024931800_awaf392-B105","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.pediatrneurol.2023.08.007","article-title":"Malnutrition in spinal muscular atrophy type I: Case report of a novel nutritional intervention with improved growth and function while receiving parallel gene splicing therapies","volume":"151","author":"Nabil","year":"2024","journal-title":"Pediatr Neurol"},{"key":"2026020710024931800_awaf392-B106","doi-asserted-by":"crossref","first-page":"6493","DOI":"10.1523\/JNEUROSCI.0206-11.2011","article-title":"The canonical nuclear factor-\u03baB pathway regulates cell survival in a developmental model of spinal cord motoneurons","volume":"31","author":"Mincheva","year":"2011","journal-title":"J Neurosci"},{"key":"2026020710024931800_awaf392-B107","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1016\/j.neuron.2014.01.013","article-title":"Microglia induce motor neuron death via the classical NF-\u03baB pathway in amyotrophic lateral sclerosis","volume":"81","author":"Frakes","year":"2014","journal-title":"Neuron"},{"key":"2026020710024931800_awaf392-B108","doi-asserted-by":"crossref","first-page":"5019","DOI":"10.1007\/s12035-017-0710-4","article-title":"Regulation of survival motor neuron protein by the nuclear factor-kappa B pathway in mouse spinal cord motoneurons","volume":"55","author":"Arumugam","year":"2018","journal-title":"Mol Neurobiol"},{"key":"2026020710024931800_awaf392-B109","doi-asserted-by":"crossref","first-page":"1054270","DOI":"10.3389\/fncel.2022.1054270","article-title":"Survival motor neuron protein and neurite degeneration are regulated by Gemin3 in spinal muscular atrophy motoneurons","volume":"16","author":"Miralles","year":"2022","journal-title":"Front Cell Neurosci"},{"key":"2026020710024931800_awaf392-B110","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1111\/j.1471-4159.2008.05668.x","article-title":"The complement cascade: Yin\u2013Yang in neuroinflammation\u2014neuro-protection and -degeneration","volume":"107","author":"Alexander","year":"2008","journal-title":"J Neurochem"},{"key":"2026020710024931800_awaf392-B111","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":"2026020710024931800_awaf392-B112","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1146\/annurev-neuro-061010-113810","article-title":"The complement system: An unexpected role in synaptic pruning during development and disease","volume":"35","author":"Stephan","year":"2012","journal-title":"Annu Rev Neurosci"},{"key":"2026020710024931800_awaf392-B113","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.3390\/cells9051108","article-title":"Regulation of microglial functions by purinergic mechanisms in the healthy and diseased CNS","volume":"9","author":"Illes","year":"2020","journal-title":"Cells"},{"key":"2026020710024931800_awaf392-B114","doi-asserted-by":"crossref","first-page":"257","DOI":"10.2174\/1871527315666160922104848","article-title":"Purinergic signalling and neurological diseases: An update","volume":"16","author":"Burnstock","year":"2017","journal-title":"CNS Neurol Disord Drug Targets"},{"key":"2026020710024931800_awaf392-B115","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1017\/S1740925X05000323","article-title":"Purinergic receptors activating rapid intracellular Ca2+ increases in microglia","volume":"2","author":"Light","year":"2006","journal-title":"Neuron Glia Biol"},{"key":"2026020710024931800_awaf392-B116","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1097\/WNR.0000000000000769","article-title":"Spinal microglial P2X4 receptor\u2013brain-derived neurotrophic factor signaling regulates nicotine withdrawal-induced hyperalgesia","volume":"28","author":"Zhang","year":"2017","journal-title":"Neuroreport"},{"key":"2026020710024931800_awaf392-B117","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1002\/glia.22745","article-title":"Rhoa\/ROCK pathway mediates p38 MAPK activation and morphological changes downstream of P2Y12\/13 receptors in spinal microglia in neuropathic pain","volume":"63","author":"Tatsumi","year":"2015","journal-title":"Glia"},{"key":"2026020710024931800_awaf392-B118","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.pneurobio.2013.10.004","article-title":"Autophagy and apoptosis dysfunction in neurodegenerative disorders","volume":"112","author":"Ghavami","year":"2014","journal-title":"Prog Neurobiol"},{"key":"2026020710024931800_awaf392-B119","doi-asserted-by":"crossref","first-page":"4886","DOI":"10.1007\/s12035-021-02472-0","article-title":"Exploring the role of autophagy dysfunction in neurodegenerative disorders","volume":"58","author":"Rana","year":"2021","journal-title":"Mol Neurobiol"},{"key":"2026020710024931800_awaf392-B120","doi-asserted-by":"crossref","first-page":"5963","DOI":"10.3390\/ijms21175963","article-title":"eATP\/P2X7R axis: An orchestrated pathway triggering inflammasome activation in muscle diseases","volume":"21","author":"Panicucci","year":"2020","journal-title":"Int J Mol Sci"},{"key":"2026020710024931800_awaf392-B121","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1298\/ptr.E10201","article-title":"Pain in spinal muscular atrophy: A questionnaire study","volume":"25","author":"Uchio","year":"2022","journal-title":"Phys Ther Res"},{"key":"2026020710024931800_awaf392-B122","doi-asserted-by":"crossref","first-page":"2798","DOI":"10.3390\/jcm13102798","article-title":"Chronic pain in patients with spinal muscular atrophy in Switzerland: A query to the spinal muscular atrophy registry","volume":"13","author":"Steiner","year":"2024","journal-title":"J Clin Med"},{"key":"2026020710024931800_awaf392-B123","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1042\/BCJ20210604","article-title":"Regulation of the release of damage-associated molecular patterns from necroptotic cells","volume":"479","author":"Nakano","year":"2022","journal-title":"Biochem J"},{"key":"2026020710024931800_awaf392-B124","doi-asserted-by":"crossref","first-page":"972029","DOI":"10.3389\/fncel.2022.972029","article-title":"Suppression of the necroptotic cell death pathways improves survival in Smn2B\/\u2212 mice","volume":"16","author":"Chehade","year":"2022","journal-title":"Front Cell Neurosci"},{"key":"2026020710024931800_awaf392-B125","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1038\/s41392-023-01486-5","article-title":"Role of neuroinflammation in neurodegeneration development","volume":"8","author":"Zhang","year":"2023","journal-title":"Signal Transduct Target Ther"},{"key":"2026020710024931800_awaf392-B126","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1038\/s41586-020-2625-x","article-title":"C9orf72 in myeloid cells suppresses STING-induced inflammation","volume":"585","author":"McCauley","year":"2020","journal-title":"Nature"},{"key":"2026020710024931800_awaf392-B127","doi-asserted-by":"crossref","first-page":"e81943","DOI":"10.7554\/eLife.81943","article-title":"Constitutively active STING causes neuroinflammation and degeneration of dopaminergic neurons in mice","volume":"11","author":"Szego","year":"2022","journal-title":"Elife"},{"key":"2026020710024931800_awaf392-B128","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1016\/j.cmet.2016.05.006","article-title":"CD38 dictates age-related NAD decline and mitochondrial dysfunction through an SIRT3-dependent mechanism","volume":"23","author":"Camacho-Pereira","year":"2016","journal-title":"Cell Metab"},{"key":"2026020710024931800_awaf392-B129","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1002\/jnr.24805","article-title":"Glycoproteins as diagnostic and prognostic biomarkers for neurodegenerative diseases: A glycoproteomic approach","volume":"99","author":"Xu","year":"2021","journal-title":"J Neurosci Res"},{"key":"2026020710024931800_awaf392-B130","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1038\/s41418-020-00664-0","article-title":"ALS motor neurons exhibit hallmark metabolic defects that are rescued by SIRT3 activation","volume":"28","author":"Hor","year":"2021","journal-title":"Cell Death Differ"},{"key":"2026020710024931800_awaf392-B131","doi-asserted-by":"crossref","first-page":"116938","DOI":"10.1016\/j.biopha.2024.116938","article-title":"Recent advances on signaling pathways and their inhibitors in spinal cord injury","volume":"176","author":"Ding","year":"2024","journal-title":"Biomed Pharmacother"},{"key":"2026020710024931800_awaf392-B132","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1016\/S0006-291X(02)02287-8","article-title":"Cytokines and related receptor-mediated signaling pathways","volume":"297","author":"Haddad","year":"2002","journal-title":"Biochem Biophys Res Commun"},{"key":"2026020710024931800_awaf392-B133","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1186\/s12915-024-01888-z","article-title":"Dysregulation of innate immune signaling in animal models of spinal muscular atrophy","volume":"22","author":"Garcia","year":"2024","journal-title":"BMC Biol"},{"key":"2026020710024931800_awaf392-B134","doi-asserted-by":"crossref","first-page":"130764","DOI":"10.1016\/j.bbagen.2025.130764","article-title":"Balancing RNA processing and innate immune response: Possible roles for SMN condensates in snRNP biogenesis","volume":"1869","author":"Maita","year":"2025","journal-title":"Biochim Biophys Acta Gen Subj"},{"key":"2026020710024931800_awaf392-B135","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1186\/s13023-021-01961-8","article-title":"Increased chitotriosidase 1 concentration following nusinersen treatment in spinal muscular atrophy","volume":"16","author":"Freigang","year":"2021","journal-title":"Orphanet J Rare Dis"},{"key":"2026020710024931800_awaf392-B136","doi-asserted-by":"crossref","first-page":"23491","DOI":"10.1038\/s41598-024-74338-z","article-title":"Profiling neuroinflammatory markers and response to nusinersen in paediatric spinal muscular atrophy","volume":"14","author":"Zhang","year":"2024","journal-title":"Sci Rep"},{"key":"2026020710024931800_awaf392-B137","doi-asserted-by":"crossref","first-page":"4329","DOI":"10.3390\/ijms22094329","article-title":"Nusinersen modulates proteomics profiles of cerebrospinal fluid in spinal muscular atrophy type 1 patients","volume":"22","author":"Bianchi","year":"2021","journal-title":"Int J Mol Sci"},{"key":"2026020710024931800_awaf392-B138","doi-asserted-by":"crossref","first-page":"982760","DOI":"10.3389\/fncel.2022.982760","article-title":"Identification of a cytokine profile in serum and cerebrospinal fluid of pediatric and adult spinal muscular atrophy patients and its modulation upon nusinersen treatment","volume":"16","author":"Bonanno","year":"2022","journal-title":"Front Cell Neurosci"},{"key":"2026020710024931800_awaf392-B139","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1093\/hmg\/ddab365","article-title":"Assessment of cerebral spinal fluid biomarkers and microRNA-mediated disease mechanisms in spinal muscular atrophy patient samples","volume":"31","author":"Welby","year":"2022","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B140","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 (Lond)"},{"key":"2026020710024931800_awaf392-B141","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.ejpn.2022.12.003","article-title":"Inflammatory markers in cerebrospinal fluid of paediatric spinal muscular atrophy patients receiving nusinersen treatment","volume":"42","author":"Scheijmans","year":"2023","journal-title":"Eur J Paediatr Neurol"},{"key":"2026020710024931800_awaf392-B142","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1007\/s00018-023-04885-7","article-title":"Multi-omics profiling of CSF from spinal muscular atrophy type 3 patients after nusinersen treatment: A 2-year follow-up multicenter retrospective study","volume":"80","author":"Faravelli","year":"2023","journal-title":"Cell Mol Life Sci"},{"key":"2026020710024931800_awaf392-B143","doi-asserted-by":"crossref","first-page":"3810","DOI":"10.3390\/ijms25073810","article-title":"Neurodegeneration biomarkers in adult spinal muscular atrophy (SMA) patients treated with nusinersen","volume":"25","author":"Andr\u00e9s-Benito","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2026020710024931800_awaf392-B144","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":"2026020710024931800_awaf392-B145","doi-asserted-by":"crossref","first-page":"3415","DOI":"10.1093\/hmg\/ddt191","article-title":"Celecoxib increases SMN and survival in a severe spinal muscular atrophy mouse model via p38 pathway activation","volume":"22","author":"Farooq","year":"2013","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B146","doi-asserted-by":"crossref","first-page":"308","DOI":"10.3389\/fnmol.2018.00308","article-title":"Pharmacological c-Jun NH2-terminal kinase (JNK) pathway inhibition reduces severity of spinal muscular atrophy disease in mice","volume":"11","author":"Schellino","year":"2018","journal-title":"Front Mol Neurosci"},{"key":"2026020710024931800_awaf392-B147","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1093\/hmg\/ddad192","article-title":"A transcriptomics-based drug repositioning approach to identify drugs with similar activities for the treatment of muscle pathologies in spinal muscular atrophy (SMA) models","volume":"33","author":"Hoolachan","year":"2024","journal-title":"Hum Mol Genet"},{"key":"2026020710024931800_awaf392-B148","doi-asserted-by":"crossref","first-page":"e2216814120","DOI":"10.1073\/pnas.2216814120","article-title":"Agonist of growth hormone-releasing hormone improves the disease features of spinal muscular atrophy mice","volume":"120","author":"Boido","year":"2023","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2026020710024931800_awaf392-B149","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.nmd.2022.04.003","article-title":"Longer-term follow-up of nusinersen efficacy and safety in adult patients with spinal muscular atrophy types 2 and 3","volume":"32","author":"Fainmesser","year":"2022","journal-title":"Neuromuscul Disord"},{"key":"2026020710024931800_awaf392-B150","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1186\/s12883-024-03543-0","article-title":"Spinal adhesive arachnoiditis in an adult patient with spinal muscular atrophy type 3 treated with intrathecal therapy","volume":"24","author":"Ubysz","year":"2024","journal-title":"BMC Neurol"},{"key":"2026020710024931800_awaf392-B151","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1089\/hum.2021.255","article-title":"Single-dose intrathecal dorsal root ganglia toxicity of onasemnogene abeparvovec in cynomolgus monkeys","volume":"33","author":"Tukov","year":"2022","journal-title":"Hum Gene Ther"},{"key":"2026020710024931800_awaf392-B152","doi-asserted-by":"crossref","first-page":"1109","DOI":"10.1007\/s40264-021-01107-6","article-title":"Clinical trial and postmarketing safety of onasemnogene abeparvovec therapy","volume":"44","author":"Day","year":"2021","journal-title":"Drug Saf"},{"key":"2026020710024931800_awaf392-B153","doi-asserted-by":"publisher","DOI":"10.1038\/s41434-025-00513-0","article-title":"Identifying novel response markers for spinal muscular atrophy revealed by targeted proteomics following gene therapy","author":"Pant","journal-title":"Gene Ther"},{"key":"2026020710024931800_awaf392-B154","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1089\/nat.2022.0067","article-title":"Systematic analysis of chemical modifications of phosphorothioate antisense oligonucleotides that modulate their innate immune response","volume":"33","author":"Pollak","year":"2023","journal-title":"Nucleic Acid Ther"},{"key":"2026020710024931800_awaf392-B155","doi-asserted-by":"crossref","first-page":"6082","DOI":"10.1093\/nar\/gkab451","article-title":"Sequence-dependent inhibition of cGAS and TLR9 DNA sensing by 2'-O-methyl gapmer oligonucleotides.","volume":"49","author":"Valentin","year":"2021","journal-title":"Nucleic Acids Res"},{"key":"2026020710024931800_awaf392-B156","first-page":"7052","article-title":"Rational design of antisense oligonucleotides modulating the activity of TLR7\/8 agonists.","volume":"48","author":"Alharbi","year":"2020","journal-title":"Nucleic Acids Res"},{"key":"2026020710024931800_awaf392-B157","doi-asserted-by":"crossref","first-page":"e143780","DOI":"10.1172\/JCI143780","article-title":"Overcoming innate immune barriers that impede AAV gene therapy vectors","volume":"131","author":"Muhuri","year":"2021","journal-title":"J Clin Invest"},{"key":"2026020710024931800_awaf392-B158","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1186\/s41687-024-00758-0","article-title":"Identification of the most relevant aspects of spinal muscular atrophy (SMA) with impact on the quality of life of SMA patients and their caregivers: The PROfuture project, a qualitative study","volume":"8","author":"de Lemus","year":"2024","journal-title":"J Patient Rep Outcomes"}],"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/brain\/advance-article-pdf\/doi\/10.1093\/brain\/awaf392\/64726883\/awaf392.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/149\/2\/404\/64726883\/awaf392.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/149\/2\/404\/64726883\/awaf392.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T15:03:11Z","timestamp":1770476591000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/article\/149\/2\/404\/8289697"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,17]]},"references-count":158,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,10,17]]},"published-print":{"date-parts":[[2026,2,7]]}},"URL":"https:\/\/doi.org\/10.1093\/brain\/awaf392","relation":{},"ISSN":["0006-8950","1460-2156"],"issn-type":[{"value":"0006-8950","type":"print"},{"value":"1460-2156","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,2]]},"published":{"date-parts":[[2025,10,17]]}}}