{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T20:42:56Z","timestamp":1789764176199,"version":"4.0.1"},"reference-count":46,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,4]]},"DOI":"10.1093\/brain\/awt020","type":"journal-article","created":{"date-parts":[[2013,3,13]],"date-time":"2013-03-13T20:40:51Z","timestamp":1363207251000},"page":"1083-1101","source":"Crossref","is-referenced-by-count":55,"title":["Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis"],"prefix":"10.1093","volume":"136","author":[{"given":"Janos","family":"Groh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas G.","family":"K\u00fchl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chi Wang","family":"Ip","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hemanth R.","family":"Nelvagal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarmi","family":"Sri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Duckett","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam","family":"Mirza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Langmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf","family":"Martini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2013,3,12]]},"reference":[{"key":"key\n\t\t\t\t20170425173450_awt020-B1","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.nbd.2007.06.012","article-title":"Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction","volume":"28","author":"Ahtiainen","year":"2007","journal-title":"Neurobiol Dis"},{"key":"key\n\t\t\t\t20170425173450_awt020-B2","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1016\/j.neuron.2009.01.020","article-title":"Distinct roles of transcription factors brn3a and brn3b in controlling the development, morphology, and function of retinal ganglion cells","volume":"61","author":"Badea","year":"2009","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170425173450_awt020-B3","doi-asserted-by":"crossref","first-page":"2735","DOI":"10.1523\/JNEUROSCI.4443-07.2008","article-title":"Progressive ganglion cell degeneration precedes neuronal loss in a mouse model of glaucoma","volume":"28","author":"Buckingham","year":"2008","journal-title":"J Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B4","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/j.expneurol.2008.02.016","article-title":"Moving towards therapies for juvenile Batten disease?","volume":"211","author":"Cooper","year":"2008","journal-title":"Exp Neurol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B5","doi-asserted-by":"crossref","first-page":"1448","DOI":"10.1042\/BST0381448","article-title":"The neuronal ceroid lipofuscinoses: the same, but different?","volume":"38","author":"Cooper","year":"2010","journal-title":"Biochem Soc Trans"},{"key":"key\n\t\t\t\t20170425173450_awt020-B6","first-page":"285","article-title":"Ganglion cell distribution in the retina of the mouse","volume":"20","author":"Drager","year":"1981","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B7","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1016\/j.expneurol.2003.10.004","article-title":"Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man","volume":"185","author":"Fischer","year":"2004","journal-title":"Exp Neurol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B8","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/j.mcn.2007.10.012","article-title":"Monocyte chemoattractant protein-1 is a pathogenic component in a model for a hereditary peripheral neuropathy","volume":"37","author":"Fischer","year":"2008","journal-title":"Mol Cell Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B9","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1038\/nn.2511","article-title":"Microglial Cx3cr1 knockout prevents neuron loss in a mouse model of Alzheimer's disease","volume":"13","author":"Fuhrmann","year":"2010","journal-title":"Nat Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B10","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/0092-8674(91)90462-8","article-title":"CD8 is needed for development of cytotoxic T cells but not helper T cells","volume":"65","author":"Fung-Leung","year":"1991","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170425173450_awt020-B11","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.exer.2011.02.008","article-title":"Axotomy-induced retinal ganglion cell death in adult mice: quantitative and topographic time course analyses","volume":"92","author":"Galindo-Romero","year":"2011","journal-title":"Exp Eye Res"},{"key":"key\n\t\t\t\t20170425173450_awt020-B12","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1016\/j.cell.2010.02.016","article-title":"Mechanisms underlying inflammation in neurodegeneration","volume":"140","author":"Glass","year":"2010","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170425173450_awt020-B13","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.ymthe.2005.11.008","article-title":"CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis","volume":"13","author":"Griffey","year":"2006","journal-title":"Mol Ther"},{"key":"key\n\t\t\t\t20170425173450_awt020-B14","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1093\/brain\/awr283","article-title":"Colony-stimulating factor-1 mediates macrophage-related neural damage in a model for Charcot-Marie-Tooth disease type 1X","volume":"135","author":"Groh","year":"2012","journal-title":"Brain"},{"key":"key\n\t\t\t\t20170425173450_awt020-B15","doi-asserted-by":"crossref","first-page":"13566","DOI":"10.1073\/pnas.251485198","article-title":"Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice","volume":"98","author":"Gupta","year":"2001","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170425173450_awt020-B16","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1016\/j.bbadis.2006.06.010","article-title":"The neuronal ceroid-lipofuscinoses: from past to present","volume":"1762","author":"Haltia","year":"2006","journal-title":"Biochim Biophys Acta"},{"key":"key\n\t\t\t\t20170425173450_awt020-B17","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/j.mcn.2008.04.009","article-title":"Origin of CD11b+ macrophage-like cells in the CNS of PLP-overexpressing mice: low influx of haematogenous macrophages and unchanged blood-brain-barrier in the optic nerve","volume":"38","author":"Ip","year":"2008","journal-title":"Mol Cell Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B18","doi-asserted-by":"crossref","first-page":"8206","DOI":"10.1523\/JNEUROSCI.1921-06.2006","article-title":"Immune cells contribute to myelin degeneration and axonopathic changes in mice overexpressing proteolipid protein in oligodendrocytes","volume":"26","author":"Ip","year":"2006","journal-title":"J Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B19","doi-asserted-by":"crossref","first-page":"e42554","DOI":"10.1371\/journal.pone.0042554","article-title":"Neuroinflammation by cytotoxic T-lymphocytes impairs retrograde axonal transport in an oligodendrocyte mutant mouse","volume":"7","author":"Ip","year":"2012","journal-title":"PLoS One"},{"key":"key\n\t\t\t\t20170425173450_awt020-B20","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1016\/j.bbamcr.2008.11.004","article-title":"Neuronal ceroid lipofuscinoses","volume":"1793","author":"Jalanko","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"key\n\t\t\t\t20170425173450_awt020-B21","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.nbd.2006.09.001","article-title":"Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis","volume":"25","author":"Kielar","year":"2007","journal-title":"Neurobiol Dis"},{"key":"key\n\t\t\t\t20170425173450_awt020-B22","doi-asserted-by":"crossref","first-page":"4066","DOI":"10.1093\/hmg\/ddp355","article-title":"Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease","volume":"18","author":"Kielar","year":"2009","journal-title":"Hum Mol Genet"},{"key":"key\n\t\t\t\t20170425173450_awt020-B23","doi-asserted-by":"crossref","first-page":"3075","DOI":"10.1172\/JCI33482","article-title":"Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice","volume":"118","author":"Kim","year":"2008","journal-title":"J Clin Invest"},{"key":"key\n\t\t\t\t20170425173450_awt020-B24","doi-asserted-by":"crossref","first-page":"1390","DOI":"10.2353\/ajpath.2010.090694","article-title":"MCP-1\/CCL2 modifies axon properties in a PMP22-overexpressing mouse model for Charcot-Marie-Tooth 1A neuropathy","volume":"176","author":"Kohl","year":"2010","journal-title":"Am J Pathol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B25","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1007\/BF00710254","article-title":"Human forms of neuronal ceroid-lipofuscinosis (Batten disease): consensus on diagnostic criteria, Hamburg 1992","volume":"16","author":"Kohlschutter","year":"1993","journal-title":"J Inherit Metab Dis"},{"key":"key\n\t\t\t\t20170425173450_awt020-B26","doi-asserted-by":"crossref","first-page":"549","DOI":"10.2353\/ajpath.2010.090722","article-title":"Ectopic T-cell specificity and absence of perforin and granzyme B alleviate neural damage in oligodendrocyte mutant mice","volume":"176","author":"Kroner","year":"2010","journal-title":"Am J Pathol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B27","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1038\/nrneurol.2012.168","article-title":"Progressive multiple sclerosis: pathology and pathogenesis","volume":"8","author":"Lassmann","year":"2012","journal-title":"Nat Rev Neurol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B28","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.mcn.2007.08.002","article-title":"Clonal expansions of pathogenic CD8+ effector cells in the CNS of myelin mutant mice","volume":"36","author":"Leder","year":"2007","journal-title":"Mol Cell Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B29","doi-asserted-by":"crossref","first-page":"15575","DOI":"10.1523\/JNEUROSCI.3579-11.2011","article-title":"The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis","volume":"31","author":"Macauley","year":"2011","journal-title":"J Neurosci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B30","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/0092-8674(92)90030-G","article-title":"RAG-1-deficient mice have no mature B and T lymphocytes","volume":"68","author":"Mombaerts","year":"1992","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170425173450_awt020-B31","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1097\/nen.0b013e31815f3899","article-title":"Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroid-lipofuscinosis","volume":"67","author":"Partanen","year":"2008","journal-title":"J Neuropathol Exp Neurol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B32","doi-asserted-by":"crossref","first-page":"2201","DOI":"10.1016\/S0042-6989(00)00081-X","article-title":"Behavioral assessment of visual acuity in mice and rats","volume":"40","author":"Prusky","year":"2000","journal-title":"Vision Res"},{"key":"key\n\t\t\t\t20170425173450_awt020-B33","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1038\/353180a0","article-title":"Normal development and function of CD8+ cells but markedly decreased helper cell activity in mice lacking CD4","volume":"353","author":"Rahemtulla","year":"1991","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170425173450_awt020-B34","doi-asserted-by":"crossref","first-page":"3823","DOI":"10.1016\/j.febslet.2008.10.015","article-title":"RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosis","volume":"582","author":"Saha","year":"2008","journal-title":"FEBS Lett"},{"key":"key\n\t\t\t\t20170425173450_awt020-B35","doi-asserted-by":"crossref","first-page":"2233","DOI":"10.1093\/hmg\/dds038","article-title":"The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol","volume":"21","author":"Saha","year":"2012","journal-title":"Hum Mol Genet"},{"key":"key\n\t\t\t\t20170425173450_awt020-B36","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/0022-510X(73)90075-0","article-title":"Infantile type of so-called neuronal ceroid-lipofuscinosis. 1. A clinical study of 15 patients","volume":"18","author":"Santavuori","year":"1973","journal-title":"J Neurol Sci"},{"key":"key\n\t\t\t\t20170425173450_awt020-B37","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1111\/j.1469-8749.1974.tb04183.x","article-title":"Infantile type of so-called neuronal ceroid-lipofuscinosis","volume":"16","author":"Santavuori","year":"1974","journal-title":"Dev Med Child Neurol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B38","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1016\/j.neurobiolaging.2005.12.006","article-title":"You say lipofuscin, we say ceroid: defining autofluorescent storage material","volume":"27","author":"Seehafer","year":"2006","journal-title":"Neurobiol Aging"},{"key":"key\n\t\t\t\t20170425173450_awt020-B39","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.jneuroim.2010.08.024","article-title":"Immunosuppression alters disease severity in juvenile Batten disease mice","volume":"230","author":"Seehafer","year":"2011","journal-title":"J Neuroimmunol"},{"key":"key\n\t\t\t\t20170425173450_awt020-B40","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1126\/science.1074069","article-title":"Alzheimer's disease is a synaptic failure","volume":"298","author":"Selkoe","year":"2002","journal-title":"Science"},{"key":"key\n\t\t\t\t20170425173450_awt020-B41","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1056\/NEJM199801293380502","article-title":"Axonal transection in the lesions of multiple sclerosis","volume":"338","author":"Trapp","year":"1998","journal-title":"N Engl J Med"},{"key":"key\n\t\t\t\t20170425173450_awt020-B42","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1093\/hmg\/ddm324","article-title":"ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones","volume":"17","author":"Wei","year":"2008","journal-title":"Hum Mol Genet"},{"key":"key\n\t\t\t\t20170425173450_awt020-B43","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.nbd.2005.11.008","article-title":"Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons","volume":"22","author":"Weimer","year":"2006","journal-title":"Neurobiol Dis"},{"key":"key\n\t\t\t\t20170425173450_awt020-B44","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1042\/BST0381484","article-title":"Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis","volume":"38","author":"Wong","year":"2010","journal-title":"Biochem Soc Trans"},{"key":"key\n\t\t\t\t20170425173450_awt020-B45","doi-asserted-by":"crossref","first-page":"8425","DOI":"10.1073\/pnas.0400625101","article-title":"Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice","volume":"101","author":"Wu","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"key\n\t\t\t\t20170425173450_awt020-B46","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1093\/hmg\/ddi451","article-title":"Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL","volume":"15","author":"Zhang","year":"2006","journal-title":"Hum Mol Genet"}],"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/brain\/article-pdf\/136\/4\/1083\/13795747\/awt020.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,8,22]],"date-time":"2017-08-22T19:45:13Z","timestamp":1503431113000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/article-lookup\/doi\/10.1093\/brain\/awt020"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,3,12]]},"references-count":46,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2013,3,12]]},"published-print":{"date-parts":[[2013,4]]}},"URL":"https:\/\/doi.org\/10.1093\/brain\/awt020","relation":{},"ISSN":["1460-2156","0006-8950"],"issn-type":[{"value":"1460-2156","type":"electronic"},{"value":"0006-8950","type":"print"}],"subject":[],"published":{"date-parts":[[2013,3,12]]}}}