{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T06:08:21Z","timestamp":1792044501601,"version":"4.4.0"},"reference-count":43,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2004,10,1]],"date-time":"2004-10-01T00:00:00Z","timestamp":1096588800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Research"],"published-print":{"date-parts":[[2004,10]]},"DOI":"10.1016\/j.brainres.2004.07.030","type":"journal-article","created":{"date-parts":[[2004,8,27]],"date-time":"2004-08-27T18:24:01Z","timestamp":1093631041000},"page":"231-242","source":"Crossref","is-referenced-by-count":149,"title":["Late onset neurodegeneration in the Cln3\u2212\/\u2212 mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation"],"prefix":"10.1016","volume":"1023","author":[{"given":"Charlie C.","family":"Pontikis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire V.","family":"Cella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisha","family":"Parihar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming J.","family":"Lim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shubhodeep","family":"Chakrabarti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah M.","family":"Mitchison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William C.","family":"Mobley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Payam","family":"Rezaie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Pearce","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.brainres.2004.07.030_bib1","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1002\/ar.1090940210","article-title":"Estimation of nuclear populations from microtome sections","volume":"94","author":"Abercrombie","year":"1946","journal-title":"Anat. Rec."},{"key":"10.1016\/j.brainres.2004.07.030_bib2","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.nbd.2004.02.010","article-title":"Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 (PPT1) null mutant mouse model of infantile neuronal ceroid lipofuscinosis","volume":"16","author":"Bible","year":"2004","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2004.07.030_bib3","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1007\/BF01273267","article-title":"Loss of pigment-laden stellate cells: a severe alteration of the isocortex in juvenile neuronal ceroid-lipofuscinosis","volume":"42","author":"Braak","year":"1978","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainres.2004.07.030_bib4","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/BF00684808","article-title":"Pigmentoarchitectonic pathology of the isocortex in juvenile neuronal ceroid-lipofuscinosis: axonal enlargements in layer IIIab and cell loss in layer V","volume":"46","author":"Braak","year":"1979","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainres.2004.07.030_bib5","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1111\/j.1365-2990.1979.tb00637.x","article-title":"Pigment-filled appendages of the small spiny neurons: a severe pathological change of the striatum in neuronal ceroid lipofuscinosis","volume":"5","author":"Braak","year":"1979","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.brainres.2004.07.030_bib6","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/S1096-7192(02)00201-9","article-title":"Functional categorization of gene expression changes in the cerebellum of a Cln3-knockout mouse model for Batten disease","volume":"78","author":"Brooks","year":"2003","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.brainres.2004.07.030_bib7","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1093\/hmg\/11.12.1421","article-title":"An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease","volume":"11","author":"Chattopadhyay","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainres.2004.07.030_bib8","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1212\/01.WNL.0000041913.97883.8B","article-title":"An autoantibody to GAD65 in sera of patients with juvenile neuronal ceroid lipofuscinoses","volume":"59","author":"Chattopadhyay","year":"2002","journal-title":"Neurology"},{"key":"10.1016\/j.brainres.2004.07.030_bib9","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1097\/00019052-200304000-00001","article-title":"Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis","volume":"16","author":"Cooper","year":"2003","journal-title":"Curr. Opin. Neurol."},{"key":"10.1016\/j.brainres.2004.07.030_bib10","doi-asserted-by":"crossref","first-page":"2556","DOI":"10.1523\/JNEUROSCI.19-07-02556.1999","article-title":"Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment","volume":"19","author":"Cooper","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainres.2004.07.030_bib11","doi-asserted-by":"crossref","first-page":"2709","DOI":"10.1093\/hmg\/11.22.2709","article-title":"Cln3\u0394ex7\/8 knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth","volume":"11","author":"Cotman","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainres.2004.07.030_bib12","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0006-8993(02)02753-1","article-title":"Calretinin and calbindin D-28k, but not parvalbumin protect against glutamate-induced delayed excitotoxicity in transfected N18-RE 105 neuroblastoma-retina hybrid cells","volume":"94","author":"D'Orlando","year":"2002","journal-title":"Brain Res."},{"key":"10.1016\/j.brainres.2004.07.030_bib13","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1002\/(SICI)1098-1063(1996)6:4<347::AID-HIPO1>3.0.CO;2-I","article-title":"Interneurons of the hippocampus","volume":"6","author":"Freund","year":"1996","journal-title":"Hippocampus"},{"key":"10.1016\/j.brainres.2004.07.030_bib14","first-page":"211","article-title":"Clinical features and molecular genetic basis of the neuronal ceroid lipofuscinoses","volume":"89","author":"Gardiner","year":"2002","journal-title":"Adv. Neurol."},{"key":"10.1016\/j.brainres.2004.07.030_bib15","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/0165-0173(94)00015-H","article-title":"Microglia: intrinsic immuneffector cell of the brain","volume":"20","author":"Gehrmann","year":"1995","journal-title":"Brain Res. Brain Res. Rev."},{"key":"10.1016\/j.brainres.2004.07.030_bib16","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1111\/j.1365-2818.1987.tb02837.x","article-title":"The efficiency of systematic sampling in stereology and its prediction","volume":"147","author":"Gundersen","year":"1987","journal-title":"J. Microsc."},{"key":"10.1016\/j.brainres.2004.07.030_bib17","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. U. S. A."},{"key":"10.1016\/j.brainres.2004.07.030_bib18","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/BF00697751","article-title":"Infantile type of so-called neuronal ceroid-lipofuscinosis. Histological and electron microscopic studies","volume":"26","author":"Haltia","year":"1973","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainres.2004.07.030_bib19","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/0022-510X(73)90076-2","article-title":"Infantile type of so-called neuronal ceroid-lipofuscinosis: 2. Morphological and biochemical studies","volume":"18","author":"Haltia","year":"1973","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.brainres.2004.07.030_bib20","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1111\/j.1750-3639.2000.tb00255.x","article-title":"Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis","volume":"10","author":"Herva","year":"2000","journal-title":"Brain Pathol."},{"key":"10.1016\/j.brainres.2004.07.030_bib21","first-page":"3877","article-title":"The neuronal ceroid lipofuscinosis","volume":"vol III","author":"Hofmann","year":"2001"},{"key":"10.1016\/j.brainres.2004.07.030_bib22","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1093\/brain\/awg089","article-title":"Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis","volume":"126","author":"Jeyakumar","year":"2003","journal-title":"Brain"},{"key":"10.1016\/j.brainres.2004.07.030_bib23","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1002\/(SICI)1097-4547(19990815)57:4<551::AID-JNR15>3.0.CO;2-R","article-title":"A mouse gene knockout model for juvenile ceroid-lipofuscinosis (Batten disease)","volume":"57","author":"Katz","year":"1999","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.brainres.2004.07.030_bib24","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/0166-2236(96)10049-7","article-title":"Microglia: a sensor for pathological events in the CNS","volume":"19","author":"Kreutzberg","year":"1996","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.brainres.2004.07.030_bib25","first-page":"1593","article-title":"Pathologic involvement of interneurons in mouse models of neuronal ceroid lipofuscinosis","volume":"25","author":"Lam","year":"1999","journal-title":"Abstr.-Soc. Neurosci."},{"key":"10.1016\/j.brainres.2004.07.030_bib26","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1006\/nbdi.1999.0267","article-title":"Targeted Disruption of the Cln3 gene provides a mouse model for Batten Disease. The Batten Mouse Model Consortium","volume":"6","author":"Mitchison","year":"1999","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainres.2004.07.030_bib27","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1111\/j.1750-3639.2004.tb00502.x","article-title":"Selectivity and types of cell death in the neuronal ceroid lipofuscinoses (NCLs)","volume":"14","author":"Mitchison","year":"2004","journal-title":"Brain Pathol."},{"key":"10.1016\/j.brainres.2004.07.030_bib28","doi-asserted-by":"crossref","first-page":"1902","DOI":"10.1073\/pnas.252784899","article-title":"Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB","volume":"100","author":"Ohmi","year":"2003","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"Suppl. A","key":"10.1016\/j.brainres.2004.07.030_bib29","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1053\/ejpn.2000.0450","article-title":"Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease","volume":"5","author":"Oswald","year":"2001","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"10.1016\/j.brainres.2004.07.030_bib30","series-title":"The Mouse Brain in Stereotaxic Coordinates","author":"Paxinos","year":"2001"},{"key":"10.1016\/j.brainres.2004.07.030_bib31","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/S0165-0173(99)00007-7","article-title":"Neuroglial activation repertoire in the injured brain: graded response, molecular mechanisms and cues to physiological function","volume":"30","author":"Raivich","year":"1999","journal-title":"Brain Res. Brain Res. Rev."},{"key":"10.1016\/j.brainres.2004.07.030_bib32","first-page":"S69","article-title":"The role of microglia in regeneration","author":"Streit","year":"1994","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"10.1016\/j.brainres.2004.07.030_bib33","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1177\/019262330002800104","article-title":"Microglial response to brain injury: a brief synopsis","volume":"28","author":"Streit","year":"2000","journal-title":"Toxicol. Pathol."},{"key":"10.1016\/j.brainres.2004.07.030_bib34","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1002\/glia.10154","article-title":"Microglia as neuroprotective, immunocompetent cells of the CNS","volume":"40","author":"Streit","year":"2002","journal-title":"Glia"},{"key":"10.1016\/j.brainres.2004.07.030_bib35","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1016\/0092-8674(95)90274-0","article-title":"Isolation of a novel gene underlying Batten disease, CLN3","volume":"82","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.brainres.2004.07.030_bib36","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00005072-199704000-00005","article-title":"Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry","volume":"56","author":"Tyynel\u00e4","year":"1997","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainres.2004.07.030_bib37","doi-asserted-by":"crossref","DOI":"10.1111\/j.1750-3639.2004.tb00077.x","article-title":"Specific patterns of storage deposition, neurodegeneration, and glial activation in the hippocampus of patients with neuronal ceroid-lipofuscinoses","volume":"14","author":"Tyynel\u00e4","year":"2004","journal-title":"Brain Pathol."},{"key":"10.1016\/j.brainres.2004.07.030_bib38","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1097\/00005072-199803000-00006","article-title":"Microglia and the development of spongiform change in Creutzfeldt\u2013Jakob disease","volume":"57","author":"von Eitzen","year":"1998","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainres.2004.07.030_bib39","doi-asserted-by":"crossref","first-page":"10954","DOI":"10.1073\/pnas.97.20.10954","article-title":"Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation","volume":"97","author":"Wada","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainres.2004.07.030_bib40","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/0006-8993(82)90513-3","article-title":"Deficiency of corpus callosum varies with strain and supplier of the mice","volume":"239","author":"Wahlsten","year":"1982","journal-title":"Brain Res."},{"key":"10.1016\/j.brainres.2004.07.030_bib41","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1002\/ajmg.1320570218","article-title":"Pathogenesis of brain dysfunction in Batten disease","volume":"57","author":"Walkley","year":"1995","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.brainres.2004.07.030_bib42","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1002\/ar.1092310411","article-title":"Unbiased stereological estimation of the total number of neurons in the subdivisions of the rat hippocampus using the optical fractionator","volume":"231","author":"West","year":"1991","journal-title":"Anat. Rec."},{"key":"10.1016\/j.brainres.2004.07.030_bib43","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1016\/S0166-2236(02)02192-6","article-title":"Knockout mice: simple solutions to the problems of genetic background and flanking genes","volume":"25","author":"Wolfer","year":"2002","journal-title":"Trends Neurosci."}],"container-title":["Brain Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899304011485?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899304011485?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,1,14]],"date-time":"2024-01-14T07:42:10Z","timestamp":1705218130000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0006899304011485"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,10]]},"references-count":43,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2004,10]]}},"alternative-id":["S0006899304011485"],"URL":"https:\/\/doi.org\/10.1016\/j.brainres.2004.07.030","relation":{},"ISSN":["0006-8993"],"issn-type":[{"value":"0006-8993","type":"print"}],"subject":[],"published":{"date-parts":[[2004,10]]}}}