{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:11:09Z","timestamp":1787062269986,"version":"build-2736575974"},"reference-count":91,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T00:00:00Z","timestamp":1671062400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["203151\/Z\/16\/Z"],"award-info":[{"award-number":["203151\/Z\/16\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000861","name":"Sparks","doi-asserted-by":"publisher","award":["14CAM03"],"award-info":[{"award-number":["14CAM03"]}],"id":[{"id":"10.13039\/501100000861","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","award":["IS-BRC-1215-20014"],"award-info":[{"award-number":["IS-BRC-1215-20014"]}],"id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,4,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Infantile Krabbe disease is a rapidly progressive and fatal disorder of myelin, caused by inherited deficiency of the lysosomal enzyme \u03b2-galactocerebrosidase. Affected children lose their motor skills and other faculties; uncontrolled seizures are a frequent terminal event. Overexpression of the sphingolipid metabolite psychosine is a pathogenic factor, but does not fully account for the pleiotropic manifestations and there is a clear need to investigate additional pathological mechanisms. We examined innate immunity, caspase-11 and associated inflammatory pathways in twitcher mice, an authentic model of Krabbe disease. Combined use of molecular tools, RNAscope in situ hybridization and immunohistochemical staining established that the expression of pro-inflammatory non-canonical caspase-11, canonical caspase-1, gasdermin D and cognate genes is induced in nervous tissue. Early onset and progressive upregulation of these genes accompany demyelination and gliosis and although the molecules are scant in healthy tissue, abundance of the respective translation products is greatly increased in diseased animals. Caspase-11 is found in reactive microglia\/macrophages as well as astrocytes but caspase-1 and gasdermin D are restricted to reactive microglia\/macrophages. The inflammasome signature is not unique to Krabbe disease; to varying degrees, this signature is also prominent in other lysosomal diseases, Sandhoff and Niemann-Pick Type-C1, and the lysolecithin toxin model of focal demyelination. Given the potent inflammatory response here identified in Krabbe disease and the other neurodegenerative disorders studied, a broad induction of inflammasomes is likely to be a dominant factor in the pathogenesis, and thus represents a platform for therapeutic exploration.<\/jats:p>","DOI":"10.1093\/hmg\/ddac299","type":"journal-article","created":{"date-parts":[[2022,12,15]],"date-time":"2022-12-15T17:02:33Z","timestamp":1671123753000},"page":"1361-1379","source":"Crossref","is-referenced-by-count":7,"title":["Upregulation of non-canonical and canonical inflammasome genes associates with pathological features in Krabbe disease and related disorders"],"prefix":"10.1093","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1884-2527","authenticated-orcid":false,"given":"Mar\u00eda B","family":"Cach\u00f3n-Gonz\u00e1lez","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Cambridge , Level 5, PO Box 157, Cambridge CB2 0QQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chao","family":"Zhao","sequence":"additional","affiliation":[{"name":"Wellcome Trust-Medical Research Council Cambridge Stem Cell Institute, University of Cambridge , Cambridge CB2 0AW , UK"},{"name":"Department of Clinical Neuro sciences, University of Cambridge , Cambridge CB2 2PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin J","family":"Franklin","sequence":"additional","affiliation":[{"name":"Wellcome Trust-Medical Research Council Cambridge Stem Cell Institute, University of Cambridge , Cambridge CB2 0AW , UK"},{"name":"Department of Clinical Neuro sciences, University of Cambridge , Cambridge CB2 2PY , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy M","family":"Cox","sequence":"additional","affiliation":[{"name":"University of Cambridge, Level 5, PO Box 157 Department of Medicine, , Cambridge CB2 0QQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,12,15]]},"reference":[{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1093\/brain\/39.1-2.74","article-title":"New familial, infantile form of diffuse brain-sclerosis","volume":"39","author":"Krabbe","year":"1916","journal-title":"Brain"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1073\/pnas.66.2.302","article-title":"Globoid cell leucodystrophy (Krabbe's disease): deficiency of galactocerebroside beta-galactosidase","volume":"66","author":"Suzuki","year":"1970","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.semcdb.2020.07.016","article-title":"Lipids in regulating oligodendrocyte structure and function","volume":"112","author":"Montani","year":"2021","journal-title":"Semin. Cell Dev. Biol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1007\/BF00293398","article-title":"Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation","volume":"88","author":"Taniike","year":"1994","journal-title":"Acta Neuropathol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1097\/00005072-199906000-00009","article-title":"An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy","volume":"58","author":"Taniike","year":"1999","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/S0022-2275(20)39839-4","article-title":"Krabbe disease: a galactosylsphingosine (psychosine) lipidosis","volume":"21","author":"Svennerholm","year":"1980","journal-title":"J. Lipid Res."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1126\/science.6719111","article-title":"Progressive accumulation of toxic metabolite in a genetic leukodystrophy","volume":"224","author":"Igisu","year":"1984","journal-title":"Science"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"20097","DOI":"10.1073\/pnas.1912108116","article-title":"Genetic ablation of acid ceramidase in Krabbe disease confirms the psychosine hypothesis and identifies a new therapeutic target","volume":"116","author":"Li","year":"2019","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2835","DOI":"10.1002\/ajmg.a.35624","article-title":"Death rates in the U.S. due to Krabbe disease and related leukodystrophy and lysosomal storage diseases","volume":"158A","author":"Barczykowski","year":"2012","journal-title":"Am. J. Med. Genet. A"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2208","DOI":"10.1038\/s41436-019-0480-7","article-title":"Clinical characteristics of 248 patients with Krabbe disease: quantitative natural history modeling based on published cases","volume":"21","author":"Komatsuzaki","year":"2019","journal-title":"Genet. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1159\/000112166","article-title":"Late-onset Krabbe disease (globoid cell leukodystrophy): clinical and biochemical features of 15 cases","volume":"13","author":"Kolodny","year":"1991","journal-title":"Dev. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1212\/WNL.0000000000004418","article-title":"Developmental outcomes of cord blood transplantation for Krabbe disease: a 15-year study","volume":"89","author":"Wright","year":"2017","journal-title":"Neurology"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"5356","DOI":"10.1038\/s41467-020-19179-w","article-title":"Brainstem development requires galactosylceramidase and is critical for pathogenesis in a model of Krabbe disease","volume":"11","author":"Weinstock","year":"2020","journal-title":"Nat. Commun."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.neuron.2020.03.031","article-title":"Macrophages expressing GALC improve peripheral Krabbe disease by a mechanism independent of cross-correction","volume":"107","author":"Weinstock","year":"2020","journal-title":"Neuron"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1002\/glia.22774","article-title":"Macrophages in spinal cord injury: phenotypic and functional change from exposure to myelin debris","volume":"63","author":"Wang","year":"2015","journal-title":"Glia"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1093\/brain\/awn109","article-title":"Debris clearance by microglia: an essential link between degeneration and regeneration","volume":"132","author":"Neumann","year":"2009","journal-title":"Brain"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1177\/09680519060120040701","article-title":"NALP3: a key player in caspase-1 activation","volume":"12","author":"Sutterwala","year":"2006","journal-title":"J. Endotoxin Res."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.bbi.2009.10.002","article-title":"Sustained hippocampal IL-1beta overexpression impairs contextual and spatial memory in transgenic mice","volume":"24","author":"Hein","year":"2010","journal-title":"Brain Behav. Immun."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/nri.2016.58","article-title":"Inflammasomes: mechanism of assembly, regulation and signalling","volume":"16","author":"Broz","year":"2016","journal-title":"Nat. Rev. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"20580","DOI":"10.1074\/jbc.271.34.20580","article-title":"Yuan J. identification and characterization of Ich-3, a member of the interleukin-1beta converting enzyme (ICE)\/Ced-3 family and an upstream regulator of ICE","volume":"271","author":"Wang","year":"1996","journal-title":"J. Biol. Chem."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1126\/science.1240988","article-title":"Cytoplasmic LPS activates caspase-11: implications in TLR4-independent endotoxic shock","volume":"341","author":"Hagar","year":"2013","journal-title":"Science"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1038\/nature13683","article-title":"Inflammatory caspases are innate immune receptors for intracellular LPS","volume":"514","author":"Shi","year":"2014","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2279","DOI":"10.1084\/jem.20180589","article-title":"Caspase-11 auto-proteolysis is crucial for noncanonical inflammasome activation","volume":"215","author":"Lee","year":"2018","journal-title":"J. Exp. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1038\/nature15514","article-title":"Cleavage of GSDMD by inflammatory caspases determines pyroptotic cell death","volume":"526","author":"Shi","year":"2015","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/nature18629","article-title":"Inflammasome-activated gasdermin D causes pyroptosis by forming membrane pores","volume":"535","author":"Liu","year":"2016","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"7858","DOI":"10.1073\/pnas.1607769113","article-title":"GsdmD p30 elicited by caspase-11 during pyroptosis forms pores in membranes","volume":"113","author":"Aglietti","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.molimm.2017.02.013","article-title":"Autophagy and inflammasomes","volume":"86","author":"Harris","year":"2017","journal-title":"Mol. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"188","DOI":"10.2119\/molmed.2017.00077","article-title":"Inflammasome and autophagy regulation \u2013 a two-way street","volume":"23","author":"Sun","year":"2017","journal-title":"Mol. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2020.591803","article-title":"Interplay between NLRP3 Inflammasome and autophagy","volume":"11","author":"Biasizzo","year":"2020","journal-title":"Front. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1111\/acel.12409","article-title":"Lysosomal storage and impaired autophagy lead to inflammasome activation in Gaucher macrophages","volume":"15","author":"Aflaki","year":"2016","journal-title":"Aging Cell"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201911185","article-title":"Haematopoietic stem cell gene therapy with IL-1Ra rescues cognitive loss in mucopolysaccharidosis IIIA","volume":"12","author":"Parker","year":"2020","journal-title":"EMBO Mol. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1046\/j.1471-4159.1996.66031118.x","article-title":"Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease","volume":"66","author":"Sakai","year":"1996","journal-title":"J. Neurochem."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1093\/brain\/103.3.695","article-title":"Hereditary leucodystrophy in the mouse: the new mutant twitcher","volume":"103","author":"Duchen","year":"1980","journal-title":"Brain"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.3390\/ijms22041506","article-title":"Regulation, activation and function of Caspase-11 during health and disease","volume":"22","author":"Agnew","year":"2021","journal-title":"Int. J. Mol. Sci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1038\/356768a0","article-title":"A novel heterodimeric cysteine protease is required for interleukin-1 beta processing in monocytes","volume":"356","author":"Thornberry","year":"1992","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1016\/j.ygeno.2004.06.005","article-title":"Caspase-1zeta, a new splice variant of the caspase-1 gene","volume":"84","author":"Feng","year":"2004","journal-title":"Genomics"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1038\/nature15541","article-title":"Caspase-11 cleaves gasdermin D for non-canonical inflammasome signalling","volume":"526","author":"Kayagaki","year":"2015","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1016\/j.chembiol.2017.03.009","article-title":"Pyroptosis and apoptosis pathways engage in bidirectional crosstalk in monocytes and macrophages","volume":"24","author":"Taabazuing","year":"2017","journal-title":"Cell Chem. Biol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2105316118","article-title":"Sulfatides are endogenous ligands for the TLR4-MD-2 complex","volume":"118","author":"Su","year":"2021","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1038\/nrn3638","article-title":"Inflammasomes in the CNS","volume":"15","author":"Walsh","year":"2014","journal-title":"Nat. Rev. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1038\/cdd.2015.16","article-title":"Neuronal NLRP1 inflammasome activation of Caspase-1 coordinately regulates inflammatory interleukin-1-beta production and axonal degeneration-associated Caspase-6 activation","volume":"22","author":"Kaushal","year":"2015","journal-title":"Cell Death Differ."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1083\/jcb.201708039","article-title":"Mechanism of Stx17 recruitment to autophagosomes via IRGM and mammalian Atg8 proteins","volume":"217","author":"Kumar","year":"2018","journal-title":"J. Cell Biol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"6046","DOI":"10.1073\/pnas.1321700111","article-title":"Guanylate binding proteins promote caspase-11-dependent pyroptosis in response to cytoplasmic LPS","volume":"111","author":"Pilla","year":"2014","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"787","DOI":"10.4049\/jimmunol.0901363","article-title":"Cutting edge: NF-kappaB activating pattern recognition and cytokine receptors license NLRP3 inflammasome activation by regulating NLRP3 expression","volume":"183","author":"Bauernfeind","year":"2009","journal-title":"J. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1042\/bj1070321","article-title":"N-acetyl-beta-glucosaminidases in human spleen","volume":"107","author":"Robinson","year":"1968","journal-title":"Biochem. J."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/ng1095-170","article-title":"Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism","volume":"11","author":"Sango","year":"1995","journal-title":"Nat. Genet."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1080\/15622975.2017.1379610","article-title":"Psychiatric and neurological symptoms in patients with Niemann-pick disease type C (NP-C): findings from the international NPC registry","volume":"20","author":"Bonnot","year":"2019","journal-title":"World J. Biol. Psychiatry"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/1750-1172-5-16","article-title":"Niemann-pick disease type C","volume":"5","author":"Vanier","year":"2010","journal-title":"Orphanet J. Rare Dis."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"10079","DOI":"10.1073\/pnas.1611956113","article-title":"Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2","volume":"113","author":"Li","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1038\/nm.1876","article-title":"Niemann-pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium","volume":"14","author":"Lloyd-Evans","year":"2008","journal-title":"Nat. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"5784","DOI":"10.1016\/S0021-9258(18)91082-3","article-title":"A genetic storage disorder in BALB\/C mice with a metabolic block in esterification of exogenous cholesterol","volume":"259","author":"Pentchev","year":"1984","journal-title":"J. Biol. Chem."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1002\/cne.10522","article-title":"Patterned Purkinje cell degeneration in mouse models of Niemann-pick type C disease","volume":"456","author":"Sarna","year":"2003","journal-title":"J. Comp. Neurol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1387","DOI":"10.1038\/nn1997","article-title":"Microglia: active sensor and versatile effector cells in the normal and pathologic brain","volume":"10","author":"Hanisch","year":"2007","journal-title":"Nat. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"14489","DOI":"10.1523\/JNEUROSCI.1256-12.2012","article-title":"Inflammatory mediators alter the astrocyte transcriptome and calcium signaling elicited by multiple G-protein-coupled receptors","volume":"32","author":"Hamby","year":"2012","journal-title":"J. Neurosci."},{"key":"2023040610570112600_","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":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"4947","DOI":"10.4049\/jimmunol.0803282","article-title":"Lipocalin 2 is required for pulmonary host defense against Klebsiella infection","volume":"182","author":"Chan","year":"2009","journal-title":"J. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"4069","DOI":"10.1073\/pnas.1218497110","article-title":"Reactive astrocytes secrete lcn2 to promote neuron death","volume":"110","author":"Bi","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/nature21029","article-title":"Neurotoxic reactive astrocytes are induced by activated microglia","volume":"541","author":"Liddelow","year":"2017","journal-title":"Nature"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2082","DOI":"10.1093\/hmg\/ddab159","article-title":"Expression of Ripk1 and DAM genes correlates with severity and progression of Krabbe disease","volume":"30","author":"Cach\u00f3n-Gonz\u00e1lez","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1242\/jcs.10.2.535","article-title":"The effect of injections of lysophosphatidyl choline into white matter of the adult mouse spinal cord","volume":"10","author":"Hall","year":"1972","journal-title":"J. Cell Sci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"3878","DOI":"10.1242\/jcs.169342","article-title":"Galactosylsphingosine (psychosine)-induced demyelination is attenuated by sphingosine 1-phosphate signalling","volume":"128","author":"O'Sullivan","year":"2015","journal-title":"J. Cell Sci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1052","DOI":"10.1126\/science.6382609","article-title":"Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse","volume":"225","author":"Yeager","year":"1984","journal-title":"Science"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"4259","DOI":"10.1073\/pnas.84.12.4259","article-title":"Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosides","volume":"84","author":"Ichioka","year":"1987","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1093\/jnen\/60.11.1062","article-title":"Expression of immune-related molecules is downregulated in twitcher mice following bone marrow transplantation","volume":"60","author":"Wu","year":"2001","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"9945","DOI":"10.1523\/JNEUROSCI.1802-11.2011","article-title":"Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2\/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy","volume":"31","author":"Reddy","year":"2011","journal-title":"J. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2709","DOI":"10.1093\/hmg\/10.23.2709","article-title":"Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: estrogen appears to be a critical factor","volume":"10","author":"Matsuda","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"14486","DOI":"10.1038\/s41598-021-93601-1","article-title":"Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor","volume":"11","author":"Babcock","year":"2021","journal-title":"Sci. Rep."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1812","DOI":"10.1111\/j.1462-5822.2006.00751.x","article-title":"Caspase-1-dependent pore formation during pyroptosis leads to osmotic lysis of infected host macrophages","volume":"8","author":"Fink","year":"2006","journal-title":"Cell. Microbiol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"3916","DOI":"10.1038\/s41467-018-06449-x","article-title":"Caspase-1 inhibition alleviates cognitive impairment and neuropathology in an Alzheimer's disease mouse model","volume":"9","author":"Flores","year":"2018","journal-title":"Nat. Commun."},{"key":"2023040610570112600_","first-page":"E6065","article-title":"Caspase-1 inhibition prevents glial inflammasome activation and pyroptosis in models of multiple sclerosis","volume":"115","author":"McKenzie","year":"2019","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"409","DOI":"10.3389\/fnmol.2017.00409","article-title":"Toll-like receptor 4 mediates methamphetamine-induced Neuroinflammation through Caspase-11 Signaling pathway in astrocytes","volume":"10","author":"Du","year":"2017","journal-title":"Front. Mol. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1083\/jcb.149.3.613","article-title":"Dual role of caspase-11 in mediating activation of caspase-1 and caspase-3 under pathological conditions","volume":"149","author":"Kang","year":"2000","journal-title":"J. Cell Biol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"3276","DOI":"10.1038\/s41467-020-16889-z","article-title":"Human GBP1 binds LPS to initiate assembly of a caspase-4 activating platform on cytosolic bacteria","volume":"11","author":"Santos","year":"2020","journal-title":"Nat. Commun."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/s41590-020-0697-2","article-title":"Guanylate-binding proteins convert cytosolic bacteria into caspase-4 signaling platforms","volume":"21","author":"Wandel","year":"2020","journal-title":"Nat. Immunol."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.15252\/embr.202050830","article-title":"Dynamin-related Irgm proteins modulate LPS-induced caspase-11 activation and septic shock","volume":"21","author":"Finethy","year":"2020","journal-title":"EMBO Rep."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"2653","DOI":"10.1016\/j.celrep.2015.11.036","article-title":"Nuclear retention of mRNA in mammalian tissues","volume":"13","author":"Bahar Halpern","year":"2015","journal-title":"Cell Rep."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1126\/science.1198817","article-title":"Mammalian genes are transcribed with widely different bursting kinetics","volume":"332","author":"Suter","year":"2011","journal-title":"Science"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1186\/1471-2164-8-340","article-title":"Comparison of the contributions of the nuclear and cytoplasmic compartments to global gene expression in human cells","volume":"8","author":"Barthelson","year":"2007","journal-title":"BMC Genomics"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201809386","article-title":"Glutathione peroxidase 8 negatively regulates caspase-4\/11 to protect against colitis","volume":"12","author":"Hsu","year":"2020","journal-title":"EMBO Mol. Med."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","DOI":"10.1111\/acel.12946","article-title":"IL-1\u03b1 cleavage by inflammatory caspases of the noncanonical inflammasome controls the senescence-associated secretory phenotype","volume":"18","author":"Wiggins","year":"2019","journal-title":"Aging Cell"},{"key":"2023040610570112600_","first-page":"cm1","article-title":"Interleukin-1 (IL-1) pathway","volume":"3","author":"Weber","year":"2010","journal-title":"Sci. Signal."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1089\/089771502320317096","article-title":"Improved recovery and delayed cytokine induction after closed head injury in mice with central overexpression of the secreted isoform of the interleukin-1 receptor antagonist","volume":"19","author":"Tehranian","year":"2002","journal-title":"J. Neurotrauma"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1002\/glia.20308","article-title":"Neuroprotective actions of endogenous interleukin-1 receptor antagonist (IL-1ra) are mediated by glia","volume":"53","author":"Pinteaux","year":"2006","journal-title":"Glia"},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1002\/jnr.20266","article-title":"Interleukin-1: a master regulator of neuroinflammation","volume":"78","author":"Basu","year":"2004","journal-title":"J. Neurosci. Res."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"3104","DOI":"10.1523\/JNEUROSCI.2346-19.2020","article-title":"Fingolimod rescues demyelination in a mouse model of Krabbe's disease","volume":"40","author":"B\u00e9chet","year":"2020","journal-title":"J. Neurosci."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"24939","DOI":"10.1038\/srep24939","article-title":"A Fingolimod modulates multiple neuroinflammatory markers in a mouse model of Alzheimer\u2019s disease","volume":"6","author":"Aytan","year":"2016","journal-title":"Sci. Rep."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.4103\/1673-5374.237108","article-title":"Neuroprotection by immunomodulatory agents in animal models of Parkinson's disease","volume":"13","author":"Martinez","year":"2018","journal-title":"Neural Regen. Res."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1073\/pnas.1014154108","article-title":"FTY720 (fingolimod) efficacy in an animal model of multiple sclerosis requires astrocyte sphingosine 1-phosphate receptor 1 (S1P1) modulation","volume":"108","author":"Choi","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","doi-asserted-by":"crossref","first-page":"10373","DOI":"10.1073\/pnas.0603765103","article-title":"Effective gene therapy in an authentic model of Tay-Sachs-related diseases","volume":"103","author":"Cach\u00f3n-Gonz\u00e1lez","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2023040610570112600_","first-page":"56322","article-title":"In vitro phagocytosis of myelin debris by bone marrow-derived macrophages","volume":"130","author":"Rolfe","year":"2017","journal-title":"J. Vis. Exp."},{"key":"2023040610570112600_","first-page":"50079","article-title":"Isolation and culture of mouse cortical astrocytes","volume":"71","author":"Schildge","year":"2013","journal-title":"J. Vis. Exp."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/hmg\/advance-article-pdf\/doi\/10.1093\/hmg\/ddac299\/48473226\/ddac299.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/hmg\/article-pdf\/32\/8\/1361\/49778313\/ddac299.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/hmg\/article-pdf\/32\/8\/1361\/49778313\/ddac299.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T04:56:21Z","timestamp":1728536181000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/32\/8\/1361\/6909000"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,15]]},"references-count":91,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,12,15]]},"published-print":{"date-parts":[[2023,4,6]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddac299","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,4,15]]},"published":{"date-parts":[[2022,12,15]]}}}