{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T14:34:26Z","timestamp":1792679666912,"version":"4.5.0"},"reference-count":36,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2014,5,10]],"date-time":"2014-05-10T00:00:00Z","timestamp":1399680000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2014,12]]},"DOI":"10.1186\/2051-5960-2-54","type":"journal-article","created":{"date-parts":[[2014,5,9]],"date-time":"2014-05-09T21:02:40Z","timestamp":1399669360000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography"],"prefix":"10.1186","volume":"2","author":[{"given":"Janos","family":"Groh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Stadler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathias","family":"Buttmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf","family":"Martini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2014,5,10]]},"reference":[{"issue":"3","key":"126_CR1","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1097\/ICU.0b013e32835f8bf8","volume":"24","author":"M Adhi","year":"2013","unstructured":"Adhi M, Duker JS: Optical coherence tomography\u2013current and future applications. Curr Opin Ophthalmol 2013, 24(3):213\u2013221. 10.1097\/ICU.0b013e32835f8bf8","journal-title":"Curr Opin Ophthalmol"},{"issue":"6","key":"126_CR2","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1097\/ICU.0000000000000000","volume":"24","author":"LM Simao","year":"2013","unstructured":"Simao LM: The contribution of optical coherence tomography in neurodegenerative diseases. Curr Opin Ophthalmol 2013, 24(6):521\u2013527. 10.1097\/ICU.0000000000000000","journal-title":"Curr Opin Ophthalmol"},{"issue":"12","key":"126_CR3","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1038\/ncpneuro0950","volume":"4","author":"EM Frohman","year":"2008","unstructured":"Frohman EM, Fujimoto JG, Frohman TC, Calabresi PA, Cutter G, Balcer LJ: Optical coherence tomography: a window into the mechanisms of multiple sclerosis. Nat Clin Pract Neurol 2008, 4(12):664\u2013675.","journal-title":"Nat Clin Pract Neurol"},{"issue":"11","key":"126_CR4","doi-asserted-by":"publisher","first-page":"1866","DOI":"10.1016\/j.bbadis.2013.01.019","volume":"1832","author":"K Kollmann","year":"2013","unstructured":"Kollmann K, Uusi-Rauva K, Scifo E, Tyynela J, Jalanko A, Braulke T: Cell biology and function of neuronal ceroid lipofuscinosis-related proteins. Biochim Biophys Acta 2013, 1832(11):1866\u20131881. 10.1016\/j.bbadis.2013.01.019","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"126_CR5","doi-asserted-by":"publisher","first-page":"1882","DOI":"10.1016\/j.bbadis.2013.05.014","volume":"1832","author":"DN Palmer","year":"2013","unstructured":"Palmer DN, Barry LA, Tyynela J, Cooper JD: NCL disease mechanisms. Biochim Biophys Acta 2013, 1832(11):1882\u20131893. 10.1016\/j.bbadis.2013.05.014","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"126_CR6","doi-asserted-by":"publisher","first-page":"1807","DOI":"10.1016\/j.bbadis.2012.11.014","volume":"1832","author":"GW Anderson","year":"2013","unstructured":"Anderson GW, Goebel HH, Simonati A: Human pathology in NCL. Biochim Biophys Acta 2013, 1832(11):1807\u20131826. 10.1016\/j.bbadis.2012.11.014","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"126_CR7","doi-asserted-by":"publisher","first-page":"1827","DOI":"10.1016\/j.bbadis.2013.03.017","volume":"1832","author":"V Warrier","year":"2013","unstructured":"Warrier V, Vieira M, Mole SE: Genetic basis and phenotypic correlations of the neuronal ceroid lipofusinoses. Biochim Biophys Acta 2013, 1832(11):1827\u20131830. 10.1016\/j.bbadis.2013.03.017","journal-title":"Biochim Biophys Acta"},{"issue":"10","key":"126_CR8","doi-asserted-by":"publisher","first-page":"850","DOI":"10.1016\/j.bbadis.2006.06.010","volume":"1762","author":"M Haltia","year":"2006","unstructured":"Haltia M: The neuronal ceroid-lipofuscinoses: from past to present. Biochim Biophys Acta 2006, 1762(10):850\u2013856. 10.1016\/j.bbadis.2006.06.010","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"126_CR9","doi-asserted-by":"publisher","first-page":"1795","DOI":"10.1016\/j.bbadis.2012.08.012","volume":"1832","author":"M Haltia","year":"2013","unstructured":"Haltia M, Goebel HH: The neuronal ceroid-lipofuscinoses: a historical introduction. Biochim Biophys Acta 2013, 1832(11):1795\u20131800. 10.1016\/j.bbadis.2012.08.012","journal-title":"Biochim Biophys Acta"},{"issue":"4","key":"126_CR10","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1016\/j.survophthal.2009.04.007","volume":"54","author":"S Bozorg","year":"2009","unstructured":"Bozorg S, Ramirez-Montealegre D, Chung M, Pearce DA: Juvenile neuronal ceroid lipofuscinosis (JNCL) and the eye. Surv Ophthalmol 2009, 54(4):463\u2013471. 10.1016\/j.survophthal.2009.04.007","journal-title":"Surv Ophthalmol"},{"issue":"1\u20132","key":"126_CR11","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1016\/j.ymgme.2004.06.019","volume":"83","author":"RG Weleber","year":"2004","unstructured":"Weleber RG, Gupta N, Trzupek KM, Wepner MS, Kurz DE, Milam AH: Electroretinographic and clinicopathologic correlations of retinal dysfunction in infantile neuronal ceroid lipofuscinosis (infantile Batten disease). Mol Genet Metab 2004, 83(1\u20132):128\u2013137.","journal-title":"Mol Genet Metab"},{"issue":"1","key":"126_CR12","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1016\/j.nbd.2004.08.013","volume":"18","author":"A Jalanko","year":"2005","unstructured":"Jalanko A, Vesa J, Manninen T, von Schantz C, Minye H, Fabritius AL, Salonen T, Rapola J, Gentile M, Kopra O, Peltonen L: Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons. Neurobiol Dis 2005, 18(1):226\u2013241. 10.1016\/j.nbd.2004.08.013","journal-title":"Neurobiol Dis"},{"issue":"24","key":"126_CR13","doi-asserted-by":"publisher","first-page":"13566","DOI":"10.1073\/pnas.251485198","volume":"98","author":"P Gupta","year":"2001","unstructured":"Gupta P, Soyombo AA, Atashband A, Wisniewski KE, Shelton JM, Richardson JA, Hammer RE, Hofmann SL: Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice. Proc Natl Acad Sci U S A 2001, 98(24):13566\u201313571. 10.1073\/pnas.251485198","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"22","key":"126_CR14","doi-asserted-by":"publisher","first-page":"2709","DOI":"10.1093\/hmg\/11.22.2709","volume":"11","author":"SL Cotman","year":"2002","unstructured":"Cotman SL, Vrbanac V, Lebel LA, Lee RL, Johnson KA, Donahue LR, Teed AM, Antonellis K, Bronson RT, Lerner TJ, MacDonald ME: Cln3(Deltaex7\/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth. Hum Mol Genet 2002, 11(22):2709\u20132721. 10.1093\/hmg\/11.22.2709","journal-title":"Hum Mol Genet"},{"issue":"4","key":"126_CR15","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1002\/(SICI)1097-4547(19990815)57:4<551::AID-JNR15>3.0.CO;2-R","volume":"57","author":"ML Katz","year":"1999","unstructured":"Katz ML, Shibuya H, Liu PC, Kaur S, Gao CL, Johnson GS: A mouse gene knockout model for juvenile ceroid-lipofuscinosis (Batten disease). J Neurosci Res 1999, 57(4):551\u2013556. 10.1002\/(SICI)1097-4547(19990815)57:4<551::AID-JNR15>3.0.CO;2-R","journal-title":"J Neurosci Res"},{"issue":"5","key":"126_CR16","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1006\/nbdi.1999.0267","volume":"6","author":"HM Mitchison","year":"1999","unstructured":"Mitchison HM, Bernard DJ, Greene ND, Cooper JD, Junaid MA, Pullarkat RK, de Vos N, Breuning MH, Owens JW, Mobley WC, Gardiner RM, Lake BD, Taschner PE, Nussbaum RL: Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. the Batten Mouse Model Consortium [corrected]. Neurobiol Dis 1999, 6(5):321\u2013334. 10.1006\/nbdi.1999.0267","journal-title":"Neurobiol Dis"},{"issue":"3","key":"126_CR17","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1016\/j.ymthe.2005.04.018","volume":"12","author":"M Griffey","year":"2005","unstructured":"Griffey M, Macauley SL, Ogilvie JM, Sands MS: AAV2-mediated ocular gene therapy for infantile neuronal ceroid lipofuscinosis. Mol Ther 2005, 12(3):413\u2013421. 10.1016\/j.ymthe.2005.04.018","journal-title":"Mol Ther"},{"issue":"Pt 4","key":"126_CR18","doi-asserted-by":"publisher","first-page":"1083","DOI":"10.1093\/brain\/awt020","volume":"136","author":"J Groh","year":"2013","unstructured":"Groh J, Kuhl TG, Ip CW, Nelvagal HR, Sri S, Duckett S, Mirza M, Langmann T, Cooper JD, Martini R: Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis. Brain 2013, 136(Pt 4):1083\u20131101.","journal-title":"Brain"},{"issue":"5","key":"126_CR19","doi-asserted-by":"publisher","first-page":"1139","DOI":"10.1002\/jnr.21008","volume":"84","author":"B Lei","year":"2006","unstructured":"Lei B, Tullis GE, Kirk MD, Zhang K, Katz ML: Ocular phenotype in a mouse gene knockout model for infantile neuronal ceroid lipofuscinosis. J Neurosci Res 2006, 84(5):1139\u20131149. 10.1002\/jnr.21008","journal-title":"J Neurosci Res"},{"issue":"4","key":"126_CR20","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1006\/mcne.2001.1099","volume":"19","author":"GM Seigel","year":"2002","unstructured":"Seigel GM, Lotery A, Kummer A, Bernard DJ, Greene ND, Turmaine M, Derksen T, Nussbaum RL, Davidson B, Wagner J, Mitchison HM: Retinal pathology and function in a Cln3 knockout mouse model of juvenile neuronal ceroid lipofuscinosis (batten disease). Mol Cell Neurosci 2002, 19(4):515\u2013527. 10.1006\/mcne.2001.1099","journal-title":"Mol Cell Neurosci"},{"issue":"6","key":"126_CR21","doi-asserted-by":"publisher","first-page":"e38310","DOI":"10.1371\/journal.pone.0038310","volume":"7","author":"JF Staropoli","year":"2012","unstructured":"Staropoli JF, Haliw L, Biswas S, Garrett L, Holter SM, Becker L, Skosyrski S, Da Silva-Buttkus P, Calzada-Wack J, Neff F, Rathkolb B, Rozman J, Schrewe A, Adler T, Puk O, Sun M, Favor J, Racz I, Bekeredjian R, Busch DH, Graw J, Klingenspor M, Klopstock T, Wolf E, Wurst W, Zimmer A, Lopez E, Harati H, Hill E, Krause DS, et al.: Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system. PLoS One 2012, 7(6):e38310. 10.1371\/journal.pone.0038310","journal-title":"PLoS One"},{"issue":"2","key":"126_CR22","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1016\/j.nbd.2005.11.008","volume":"22","author":"JM Weimer","year":"2006","unstructured":"Weimer JM, Custer AW, Benedict JW, Alexander NA, Kingsley E, Federoff HJ, Cooper JD, Pearce DA: Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons. Neurobiol Dis 2006, 22(2):284\u2013293. 10.1016\/j.nbd.2005.11.008","journal-title":"Neurobiol Dis"},{"issue":"2","key":"126_CR23","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1016\/j.nbd.2007.08.017","volume":"29","author":"ML Katz","year":"2008","unstructured":"Katz ML, Johnson GS, Tullis GE, Lei B: Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis. Neurobiol Dis 2008, 29(2):242\u2013253. 10.1016\/j.nbd.2007.08.017","journal-title":"Neurobiol Dis"},{"key":"126_CR24","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1007\/978-1-4614-3209-8_63","volume":"801","author":"C Volz","year":"2014","unstructured":"Volz C, Mirza M, Langmann T, Jagle H: Retinal function in aging homozygous cln3 (deltaex7\/8) knock-in mice. Adv Exp Med Biol 2014, 801: 495\u2013501. 10.1007\/978-1-4614-3209-8_63","journal-title":"Adv Exp Med Biol"},{"issue":"6","key":"126_CR25","doi-asserted-by":"publisher","first-page":"2921","DOI":"10.1167\/iovs.12-9662","volume":"53","author":"MJ Mattapallil","year":"2012","unstructured":"Mattapallil MJ, Wawrousek EF, Chan CC, Zhao H, Roychoudhury J, Ferguson TA, Caspi RR: The Rd8 mutation of the Crb1 gene is present in vendor lines of C57BL\/6\u00a0N mice and embryonic stem cells, and confounds ocular induced mutant phenotypes. Invest Ophthalmol Vis Sci 2012, 53(6):2921\u20132927. 10.1167\/iovs.12-9662","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"7","key":"126_CR26","doi-asserted-by":"publisher","first-page":"3432","DOI":"10.1167\/iovs.08-2970","volume":"50","author":"UE Wolf-Schnurrbusch","year":"2009","unstructured":"Wolf-Schnurrbusch UE, Ceklic L, Brinkmann CK, Iliev ME, Frey M, Rothenbuehler SP, Enzmann V, Wolf S: Macular thickness measurements in healthy eyes using six different optical coherence tomography instruments. Invest Ophthalmol Vis Sci 2009, 50(7):3432\u20133437. 10.1167\/iovs.08-2970","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"7","key":"126_CR27","doi-asserted-by":"publisher","first-page":"964","DOI":"10.1136\/bjo.2008.150482","volume":"93","author":"CK Leung","year":"2009","unstructured":"Leung CK, Lindsey JD, Chen L, Liu Q, Weinreb RN: Longitudinal profile of retinal ganglion cell damage assessed with blue-light confocal scanning laser ophthalmoscopy after ischaemic reperfusion injury. Br J Ophthalmol 2009, 93(7):964\u2013968. 10.1136\/bjo.2008.150482","journal-title":"Br J Ophthalmol"},{"issue":"10","key":"126_CR28","doi-asserted-by":"publisher","first-page":"e75963","DOI":"10.1371\/journal.pone.0075963","volume":"8","author":"M Mirza","year":"2013","unstructured":"Mirza M, Volz C, Karlstetter M, Langiu M, Somogyi A, Ruonala MO, Tamm ER, Jagle H, Langmann T: Progressive retinal degeneration and glial activation in the CLN6 (nclf) mouse model of neuronal ceroid lipofuscinosis: a beneficial effect of DHA and curcumin supplementation. PLoS One 2013, 8(10):e75963. 10.1371\/journal.pone.0075963","journal-title":"PLoS One"},{"issue":"5","key":"126_CR29","doi-asserted-by":"publisher","first-page":"657","DOI":"10.1097\/IAE.0b013e31819b0542","volume":"29","author":"DP Hainsworth","year":"2009","unstructured":"Hainsworth DP, Liu GT, Hamm CW, Katz ML: Funduscopic and angiographic appearance in the neuronal ceroid lipofuscinoses. Retina 2009, 29(5):657\u2013668. 10.1097\/IAE.0b013e31819b0542","journal-title":"Retina"},{"issue":"6","key":"126_CR30","doi-asserted-by":"publisher","first-page":"1448","DOI":"10.1042\/BST0381448","volume":"38","author":"JD Cooper","year":"2010","unstructured":"Cooper JD: The neuronal ceroid lipofuscinoses: the same, but different? Biochem Soc Trans 2010, 38(6):1448\u20131452. 10.1042\/BST0381448","journal-title":"Biochem Soc Trans"},{"issue":"9","key":"126_CR31","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1177\/0883073813494479","volume":"28","author":"TG K\u00fchl","year":"2013","unstructured":"K\u00fchl TG, Dihanich S, Wong AMS, Cooper JD: Regional brain atrophy in mouse models of neuronal ceroid lipofuscinosis: a new rostrocaudal perspective. J Child Neurol 2013, 28(9):1117\u20131122. 10.1177\/0883073813494479","journal-title":"J Child Neurol"},{"issue":"11","key":"126_CR32","doi-asserted-by":"publisher","first-page":"1608","DOI":"10.1038\/nn.3526","volume":"16","author":"C Sarkar","year":"2013","unstructured":"Sarkar C, Chandra G, Peng S, Zhang Z, Liu A, Mukherjee AB: Neuroprotection and lifespan extension in Ppt1(-\/-) mice by NtBuHA: therapeutic implications for INCL. Nat Neurosci 2013, 16(11):1608\u20131617. 10.1038\/nn.3526","journal-title":"Nat Neurosci"},{"issue":"11","key":"126_CR33","doi-asserted-by":"publisher","first-page":"1906","DOI":"10.1016\/j.bbadis.2013.05.026","volume":"1832","author":"JA Hawkins-Salsbury","year":"2013","unstructured":"Hawkins-Salsbury JA, Cooper JD, Sands MS: Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease). Biochim Biophys Acta 2013, 1832(11):1906\u20131909. 10.1016\/j.bbadis.2013.05.026","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"126_CR34","doi-asserted-by":"publisher","first-page":"e79188","DOI":"10.1371\/journal.pone.0079188","volume":"8","author":"K Lidster","year":"2013","unstructured":"Lidster K, Jackson SJ, Ahmed Z, Munro P, Coffey P, Giovannoni G, Baker MD, Baker D: Neuroprotection in a novel mouse model of multiple sclerosis. PLoS One 2013, 8(11):e79188. 10.1371\/journal.pone.0079188","journal-title":"PLoS One"},{"issue":"Pt 2","key":"126_CR35","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1093\/brain\/awr264","volume":"135","author":"SB Syc","year":"2012","unstructured":"Syc SB, Saidha S, Newsome SD, Ratchford JN, Levy M, Ford E, Crainiceanu CM, Durbin MK, Oakley JD, Meyer SA, Frohman EM, Calabresi PA: Optical coherence tomography segmentation reveals ganglion cell layer pathology after optic neuritis. Brain 2012, 135(Pt 2):521\u2013533.","journal-title":"Brain"},{"issue":"2","key":"126_CR36","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1016\/j.neulet.2007.02.090","volume":"420","author":"C Paquet","year":"2007","unstructured":"Paquet C, Boissonnot M, Roger F, Dighiero P, Gil R, Hugon J: Abnormal retinal thickness in patients with mild cognitive impairment and Alzheimer\u2019s disease. Neurosci Lett 2007, 420(2):97\u201399. 10.1016\/j.neulet.2007.02.090","journal-title":"Neurosci Lett"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/2051-5960-2-54.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/2051-5960-2-54\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/2051-5960-2-54","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/2051-5960-2-54.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,1,22]],"date-time":"2019-01-22T08:25:15Z","timestamp":1548145515000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/2051-5960-2-54"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,5,10]]},"references-count":36,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,12]]}},"alternative-id":["126"],"URL":"https:\/\/doi.org\/10.1186\/2051-5960-2-54","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,5,10]]},"assertion":[{"value":"17 April 2014","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 April 2014","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 May 2014","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"54"}}