{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T05:26:58Z","timestamp":1786771618557,"version":"build-2736575974"},"reference-count":111,"publisher":"Elsevier BV","issue":"10","license":[{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2016,10,1]],"date-time":"2016-10-01T00:00:00Z","timestamp":1475280000000},"content-version":"vor","delay-in-days":366,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010446","name":"Batten Disease Family Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010446","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011102","name":"Seventh Framework Programme","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011102","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011103","name":"Sixth Framework Programme","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011103","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011516","name":"Beyond Batten Disease Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011516","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005468","name":"Batten Disease Support and Research Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005468","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"published-print":{"date-parts":[[2015,10]]},"DOI":"10.1016\/j.bbadis.2015.05.014","type":"journal-article","created":{"date-parts":[[2015,5,28]],"date-time":"2015-05-28T22:29:50Z","timestamp":1432852190000},"page":"2256-2261","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":76,"special_numbering":"PB","title":["Towards a new understanding of NCL pathogenesis"],"prefix":"10.1016","volume":"1852","author":[{"given":"Jonathan D.","family":"Cooper","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta A.","family":"Tarczyluk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hemanth R.","family":"Nelvagal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbadis.2015.05.014_bb0005","doi-asserted-by":"crossref","first-page":"1807","DOI":"10.1016\/j.bbadis.2012.11.014","article-title":"Human pathology in NCL","volume":"1832","author":"Anderson","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0010","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1016\/j.ajhg.2011.04.004","article-title":"Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6","volume":"88","author":"Arsov","year":"2011","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0015","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.ymgme.2005.11.005","article-title":"A mutation in the cathepsin D gene (CTSD) in American Bulldogs with neuronal ceroid lipofuscinosis","volume":"87","author":"Awano","year":"2006","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0020","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/j.ymgme.2006.02.016","article-title":"A frame shift mutation in canine TPP1 (the ortholog of human CLN2) in a juvenile Dachshund with neuronal ceroid lipofuscinosis","volume":"89","author":"Awano","year":"2006","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0025","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.bbadis.2015.05.014_bb0030","doi-asserted-by":"crossref","first-page":"1842","DOI":"10.1016\/j.bbadis.2013.01.009","article-title":"Use of model organisms for the study of neuronal ceroid lipofuscinosis","volume":"1832","author":"Bond","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0035","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1007\/s00401-011-0825-z","article-title":"Alzheimer's pathogenesis: is there neuron-to-neuron propagation?","volume":"121","author":"Braak","year":"2011","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0040","doi-asserted-by":"crossref","first-page":"2646","DOI":"10.1093\/hmg\/dds089","article-title":"Mutation of the parkinsonism gene ATP13A2 causes neuronal ceroid-lipofuscinosis","volume":"21","author":"Bras","year":"2012","journal-title":"Hum. Mol. Genet."},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0045","first-page":"26","article-title":"Defining the onset and progression of neuropathological changes in mouse models of LINCL. NCL2014 poster presentation P10","volume":"74","author":"Brooks","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0050","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1136\/jmg.35.9.717","article-title":"Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6","volume":"35","author":"Broom","year":"1998","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0055","doi-asserted-by":"crossref","first-page":"e95023","DOI":"10.1371\/journal.pone.0095023","article-title":"Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL)","volume":"9","author":"Burkovetskaya","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0060","doi-asserted-by":"crossref","first-page":"2242","DOI":"10.1016\/j.bbadis.2015.04.027","article-title":"Cell biology of the NCL proteins: what they do and don't do","volume":"1852","author":"C\u00e1rcel-Trullols","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0065","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1038\/mt.2008.9","article-title":"Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late Infantile Neuronal Ceroid Lipofuscinosis","volume":"16","author":"Chang","year":"2008","journal-title":"Mol. Ther."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0070","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.bbadis.2015.05.014_bb0075","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":"10.1016\/j.bbadis.2015.05.014_bb0080","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.bbadis.2015.05.014_bb0085","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.bbadis.2006.08.002","article-title":"Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis","volume":"1762","author":"Cooper","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0090","first-page":"15","article-title":"Assessing the contribution of glial dysfunction to NCL pathogenesis. NCL2014 oral presentation P12","volume":"74","author":"Cooper","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0095","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/bs.mcb.2014.11.010","article-title":"Quantifying storage material accumulation in tissue sections","volume":"126","author":"Cooper","year":"2015","journal-title":"Methods Cell Biol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0100","doi-asserted-by":"crossref","first-page":"2709","DOI":"10.1093\/hmg\/11.22.2709","article-title":"Cln3(Deltaex7\/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.bbadis.2015.05.014_bb0105","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.nbd.2014.01.003","article-title":"Gene disruption of Mfsd8 in mice provides the first animal model for CLN7 disease","volume":"65","author":"Damme","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0110","article-title":"Large Animal Models. Chapter 18 in the Neuronal Ceroid Lipofuscinoses (Batten Disease)","author":"Dr\u00f6gem\u00fcller","year":"2011"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0115","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbadis.2015.04.022","article-title":"The neuronal ceroid lipofuscinoses: Opportunities from model systems","author":"Faller","year":"2015","journal-title":"Biochim Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0120","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.jgg.2012.07.014","article-title":"Genetically modified pig models for human diseases","volume":"40","author":"Fan","year":"2013","journal-title":"J. Genet. Genomics"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0125","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1007\/s13353-013-0182-9","article-title":"Genetically modified pigs to model human diseases","volume":"55","author":"Flisikowska","year":"2014","journal-title":"J. Appl. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0130","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1038\/nrn3901","article-title":"The connectomics of brain disorders","volume":"16","author":"Fornito","year":"2015","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0135","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/j.nbd.2007.09.006","article-title":"A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3","volume":"29","author":"Frugier","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0140","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1111\/j.1469-8749.2011.04170.x","article-title":"Progressive conduction defects and cardiac death in late infantile neuronal ceroid lipofuscinosis","volume":"54","author":"Fukumura","year":"2012","journal-title":"Dev. Med. Child Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0145","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1086\/338190","article-title":"Mutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouse","volume":"70","author":"Gao","year":"2006","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0150","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1111\/nan.12014","article-title":"Mechanisms underlying synaptic vulnerability and degeneration in neurodegenerative disease","volume":"39","author":"Gillingwater","year":"2013","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0155","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1016\/j.nbd.2004.03.005","article-title":"Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of Infantile Neuronal Ceroid Lipofuscinosis (INCL)","volume":"16","author":"Griffey","year":"2004","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0160","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":"10.1016\/j.bbadis.2015.05.014_bb0165","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.1093\/brain\/awt020","article-title":"Immune cells mediate neuronal damage in a mouse model of Infantile Neuronal Ceroid Lipofuscinosis","volume":"136","author":"Groh","year":"2013","journal-title":"Brain"},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0170","first-page":"15","article-title":"Sialoadhesin-positive microglia\/macrophages promote neuroinflammation and axonal perturbation in the retinotectal system of two mouse models of neuronal ceroid lipofuscinosis. NCL2014 oral presentation P15","volume":"74","author":"Groh","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0175","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."},{"issue":"Suppl. A","key":"10.1016\/j.bbadis.2015.05.014_bb0180","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1053\/ejpn.2000.0465","article-title":"Cardiac pathology in neuronal ceroid lipofuscinoses\u2014a clinicopathologic correlation in three patients","volume":"5","author":"Hofman","year":"2001","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0185","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.ymgme.2012.05.009","article-title":"Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis","volume":"107","author":"Hu","year":"2012","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0190","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.bbrc.2004.12.038","article-title":"A mutation in the CLN8 gene in English Setter dogs with neuronal ceroid-lipofuscinosis","volume":"327","author":"Katz","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0195","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":"10.1016\/j.bbadis.2015.05.014_bb0200","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."},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0205","first-page":"15","article-title":"Therapeutic targeting of neuroinflammatory pathways in juvenile Batten disease. NCL2014 oral presentation P14","volume":"74","author":"Kielian","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0210","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":"10.1016\/j.bbadis.2015.05.014_bb0215","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1097\/NEN.0b013e318238fc28","article-title":"Morphological and functional correlates of synaptic pathology in the cathepsin D knock-out mouse model of congenital neuronal ceroid-lipofuscinosis","volume":"70","author":"Koch","year":"2011","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0220","doi-asserted-by":"crossref","first-page":"6898","DOI":"10.1523\/JNEUROSCI.20-18-06898.2000","article-title":"Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons","volume":"20","author":"Koike","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0225","doi-asserted-by":"crossref","first-page":"1866","DOI":"10.1016\/j.bbadis.2013.01.019","article-title":"Cell biology and function of neuronal ceroid lipofuscinosis-related proteins","volume":"1832","author":"Kollmann","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0230","doi-asserted-by":"crossref","first-page":"2893","DOI":"10.1093\/hmg\/ddh312","article-title":"A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early aging","volume":"13","author":"Kopra","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0235","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/j.neuropharm.2010.10.010","article-title":"Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease","volume":"60","author":"Kov\u00e1cs","year":"2011","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0240","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/j.neuropharm.2012.05.040","article-title":"Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease","volume":"3","author":"Kov\u00e1cs","year":"2012","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0245","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1016\/j.nbd.2012.04.018","article-title":"Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice","volume":"47","author":"Kuronen","year":"2012","journal-title":"Neurobiol. Dis."},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0250","first-page":"24","article-title":"Evidence for impaired glial biology in infantile NCL. NCL2014 poster presentation P1","volume":"74","author":"Lange","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0255","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/S1474-4422(14)70142-5","article-title":"Oral cysteamine bitartrate and N-acetylcysteine for patients with infantile neuronal ceroid lipofuscinosis: a pilot study","volume":"13","author":"Levin","year":"2014","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0260","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.nbd.2006.09.005","article-title":"IgG entry and deposition are components of the neuroimmune response Batten Disease","volume":"25","author":"Lim","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0265","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.ymgme.2009.12.002","article-title":"Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis","volume":"99","author":"Lu","year":"2010","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0270","doi-asserted-by":"crossref","DOI":"10.1016\/j.ymgme.2015.05.005","article-title":"Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis","author":"Lu","year":"2015","journal-title":"Mol. Genet. Metab"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0275","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.expneurol.2009.01.022","article-title":"Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse","volume":"217","author":"Macauley","year":"2009","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0280","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1002\/ana.23545","article-title":"Synergistic effects of CNS-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis","volume":"71","author":"Macauley","year":"2012","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0285","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.ygeno.2005.06.005","article-title":"A mutation in canine CLN5 causes neuronal ceroid lipofuscinosis in Border collie dogs","volume":"86","author":"Melville","year":"2005","journal-title":"Genomics"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0290","doi-asserted-by":"crossref","first-page":"e40509","DOI":"10.1371\/journal.pone.0040509","article-title":"Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification","volume":"7","author":"Meng","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0295","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1038\/mt.2013.267","article-title":"Effective intravenous therapy for neurodegenerative disease with a therapeutic enzyme and a peptide that mediates delivery to the brain","volume":"22","author":"Meng","year":"2014","journal-title":"Mol. Ther."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0300","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.bbadis.2015.05.014_bb0305","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.bbadis.2015.05.014_bb0310","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1016\/j.bbadis.2015.05.011","article-title":"Genetics of the Neuronal Ceroid Lipofuscinoses (Batten disease)","volume":"1852","author":"Mole","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"issue":"11","key":"10.1016\/j.bbadis.2015.05.014_bb0315","doi-asserted-by":"crossref","first-page":"e78694","DOI":"10.1371\/journal.pone.0078694","article-title":"A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease","volume":"8","author":"Morgan","year":"2013","journal-title":"PLoS One"},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0320","first-page":"16","article-title":"Defining of the nature and progression of spinal cord and brainstem pathology in Ppt1\u2212\/\u2212 mice. NCL2014 oral presentation P17","volume":"74","author":"Nelvagal","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0325","doi-asserted-by":"crossref","first-page":"2292","DOI":"10.1016\/j.bbadis.2015.04.026","article-title":"Experimental therapies in the neuronal ceroid lipofuscinoses","volume":"1852","author":"Neverman","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0330","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.ajhg.2011.07.003","article-title":"Mutations in DNAJC5, encoding cysteine-string protein alpha, cause autosomal-dominant adult-onset neuronal ceroid lipofuscinosis","volume":"89","author":"Noskov\u00e1","year":"2011","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0335","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1212\/WNL.0b013e31821435bd","article-title":"Cardiac involvement in juvenile neuronal ceroid lipofuscinosis (Batten disease)","volume":"76","author":"Ostergaard","year":"2011","journal-title":"Neurology"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0340","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.nbd.2005.01.025","article-title":"Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)","volume":"20","author":"Oswald","year":"2005","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0345","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.nbd.2008.06.004","article-title":"Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis","volume":"32","author":"Oswald","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0350","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/S0167-4943(02)00011-0","article-title":"The origin of fluorescence in the neuronal ceroid lipofuscinoses (Batten disease) and neuron cultures from affected sheep for studies of neurodegeneration","volume":"34","author":"Palmer","year":"2002","journal-title":"Arch. Gerontol. Geriatr."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0355","doi-asserted-by":"crossref","first-page":"1882","DOI":"10.1016\/j.bbadis.2013.05.014","article-title":"NCL disease mechanisms","volume":"1832","author":"Palmer","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0360","doi-asserted-by":"crossref","first-page":"2279","DOI":"10.1016\/j.bbadis.2015.06.013","article-title":"Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network","volume":"1852","author":"Palmer","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0365","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":"10.1016\/j.bbadis.2015.05.014_bb0370","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1523\/JNEUROSCI.2676-05.2006","article-title":"Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis","volume":"26","author":"Passini","year":"2006","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0375","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.brainres.2004.07.030","article-title":"Late onset neurodegeneration in the Cln3\u2212\/\u2212 mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation","volume":"1023","author":"Pontikis","year":"2004","journal-title":"Brain Res."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0380","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1016\/j.nbd.2005.05.018","article-title":"Thalamocortical neuron loss and localized astrocytosis in the Cln3\u0394ex7\/8 knock-in mouse model of Batten disease","volume":"20","author":"Pontikis","year":"2005","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0385","doi-asserted-by":"crossref","first-page":"2262","DOI":"10.1016\/j.bbadis.2015.05.007","article-title":"Human NCL Neuropathology","volume":"1852","author":"Radke","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0390","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1016\/j.neuron.2011.12.040","article-title":"A network diffusion model of disease progression in dementia","volume":"73","author":"Raj","year":"2012","journal-title":"Neuron"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0395","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1038\/13868","article-title":"The neuronal ceroid lipofuscinoses in human EPMR and mnd mutant mice are associated with mutations in CLN8","volume":"23","author":"Ranta","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0400","doi-asserted-by":"crossref","first-page":"1608","DOI":"10.1038\/nn.3526","article-title":"Neuroprotection and lifespan extension in Ppt1(\u2212\/\u2212) mice by NtBuHA: therapeutic implications for INCL","volume":"16","author":"Sarkar","year":"2013","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0405","doi-asserted-by":"crossref","first-page":"1801","DOI":"10.1016\/j.bbadis.2013.04.008","article-title":"NCL diseases \u2014 clinical perspectives","volume":"1832","author":"Schulz","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0410","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.nbd.2011.12.009","article-title":"Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism","volume":"46","author":"Schmiedt","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0415","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":"10.1016\/j.bbadis.2015.05.014_bb0420","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.brainresbull.2012.03.003","article-title":"Mouse models of neuronal ceroid lipofuscinoses: useful pre-clinical tools to delineate disease pathophysiology and validate therapeutics","volume":"88","author":"Shacka","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0425","doi-asserted-by":"crossref","DOI":"10.2174\/1566524014666141010154913","article-title":"Neuronal Ceroid Lipofuscinosis: The Increasing Spectrum of an Old Disease","author":"Simonati","year":"2014","journal-title":"Curr. Mol. Med"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0430","doi-asserted-by":"crossref","first-page":"9117","DOI":"10.1523\/JNEUROSCI.2729-04.2004","article-title":"A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration","volume":"24","author":"Sleat","year":"2004","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0435","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1016\/j.ajhg.2012.04.021","article-title":"Strikingly different clinicopathological phenotypes determined by progranulin-mutation dosage","volume":"90","author":"Smith","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0440","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1093\/hmg\/dds558","article-title":"Cathepsin F mutations cause type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis","volume":"22","author":"Smith","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0445","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1046\/j.1471-4159.1999.0730700.x","article-title":"Biochemical characterization of a lysosomal protease deficient in classical late infantile neuronal ceroid lipofuscinosis (LINCL) and development of an enzyme-based assay for diagnosis and exclusion of LINCL in human specimens and animal models","volume":"73","author":"Sohar","year":"1999","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0450","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/sj.mt.6300049","article-title":"Enhanced survival of the LINCL mouse following CLN2 gene transfer using the rh.10 rhesus macaque-derived adeno-associated virus vector","volume":"15","author":"Sondhi","year":"2007","journal-title":"Mol. Ther."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0455","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.expneurol.2008.04.022","article-title":"Survival advantage of neonatal CNS gene transfer for late infantile neuronal ceroid lipofuscinosis","volume":"213","author":"Sondhi","year":"2008","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0460","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.ajhg.2012.05.023","article-title":"A homozygous mutation in KCTD7 links neuronal ceroid lipofuscinosis to the ubiquitin\u2013proteasome system","volume":"91","author":"Staropoli","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0465","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/j.stem.2009.05.022","article-title":"Neuroprotection of host cells by human central nervous system-stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis","volume":"5","author":"Tamaki","year":"2009","journal-title":"Cell Stem Cell"},{"issue":"Suppl. A","key":"10.1016\/j.bbadis.2015.05.014_bb0470","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1053\/ejpn.2000.0432","article-title":"Neuronal ceroid lipofuscinosis in Australian Merino sheep: a new animal model","volume":"5","author":"Tammen","year":"2001","journal-title":"Eur. J. Paediatr. Neurol."},{"issue":"Suppl. II","key":"10.1016\/j.bbadis.2015.05.014_bb0475","first-page":"30","article-title":"Combination therapies for juvenile Batten disease. NCL2014 poster presentation P26","volume":"74","author":"Tarczyluk","year":"2014","journal-title":"Medicina"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0480","doi-asserted-by":"crossref","first-page":"e35493","DOI":"10.1371\/journal.pone.0035493","article-title":"Autophagy, proteasomal degradation of mutant Cln6 and reactive gliosis are involved in the neuropathology of nclf mice","volume":"7","author":"Thelen","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0485","first-page":"350","article-title":"An autopsy case of juvenile neuronal ceroid-lipofuscinosis with dilated cardiomyopathy","volume":"40","author":"Tomiyasu","year":"2000","journal-title":"Rinsho Shinkeigaku"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0490","doi-asserted-by":"crossref","first-page":"349","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.bbadis.2015.05.014_bb0495","doi-asserted-by":"crossref","first-page":"15831","DOI":"10.1074\/jbc.271.26.15831","article-title":"Lysosomal targeting of palmitoyl-protein thioesterase","volume":"271","author":"Verkruyse","year":"1996","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0500","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1016\/j.nbd.2009.02.001","article-title":"Progressive thalamocortical neuron loss in Cln5 deficient mice: distinct effects in Finnish variant late infantile NCL","volume":"34","author":"von Schantz","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0505","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/j.ymgme.2014.09.004","article-title":"Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis","volume":"114","author":"Vuillemenot","year":"2015","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0510","doi-asserted-by":"crossref","first-page":"1827","DOI":"10.1016\/j.bbadis.2013.03.017","article-title":"Genetic basis and phenotypic correlations of the neuronal ceroid lipofusinoses","volume":"1832","author":"Warrier","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0515","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":"10.1016\/j.bbadis.2015.05.014_bb0520","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.brainres.2009.02.009","article-title":"Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"1266","author":"Weimer","year":"2009","journal-title":"Brain Res."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0525","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1212\/WNL.0b013e31825f0547","article-title":"New nomenclature and classification scheme for the neuronal ceroid lipofuscinoses","volume":"79","author":"Williams","year":"2012","journal-title":"Neurology"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0530","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1016\/j.bbadis.2006.07.001","article-title":"Diagnosis of the neuronal ceroid lipofuscinoses: an update","volume":"1762","author":"Williams","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2015.05.014_bb0535","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":"10.1016\/j.bbadis.2015.05.014_bb0540","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1111\/jnc.12385","article-title":"Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro-inflammatory phenotype","volume":"127","author":"Xiong","year":"2013","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0545","first-page":"288","article-title":"Neurodegenerative disease: neurodegenerative networking","volume":"13","author":"Yates","year":"2012","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.bbadis.2015.05.014_bb0550","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1038\/86554","article-title":"Lysosomal ceroid depletion by drugs: therapeutic implications for a hereditary neurodegenerative disease of childhood","volume":"7","author":"Zhang","year":"2001","journal-title":"Nat. Med."},{"issue":"5","key":"10.1016\/j.bbadis.2015.05.014_bb9000","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1007\/s10545-011-9446-x","article-title":"Combination small molecule PPT1 mimetic and CNS-directed gene therapy as a treatment for infantile neuronal ceroid lipofuscinosis","volume":"35","author":"Roberts","year":"2012","journal-title":"J. Inherit. Metab. Dis."}],"container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S092544391500157X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S092544391500157X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T04:37:12Z","timestamp":1786768632000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S092544391500157X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,10]]},"references-count":111,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2015,10]]}},"alternative-id":["S092544391500157X"],"URL":"https:\/\/doi.org\/10.1016\/j.bbadis.2015.05.014","relation":{},"ISSN":["0925-4439"],"issn-type":[{"value":"0925-4439","type":"print"}],"subject":[],"published":{"date-parts":[[2015,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Towards a new understanding of NCL pathogenesis","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbadis.2015.05.014","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}