{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T22:23:31Z","timestamp":1787696611239,"version":"build-2784847793"},"reference-count":69,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Mol Brain"],"published-print":{"date-parts":[[2014,12]]},"DOI":"10.1186\/1756-6606-7-7","type":"journal-article","created":{"date-parts":[[2014,1,28]],"date-time":"2014-01-28T20:01:28Z","timestamp":1390939288000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["Loss of GABAergic cortical neurons underlies the neuropathology of Lafora disease"],"prefix":"10.1186","volume":"7","author":[{"given":"Saida","family":"Ortolano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Vieitez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto Carlos","family":"Agis-Balboa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Spuch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2014,1,28]]},"reference":[{"key":"253_CR1","first-page":"47","volume":"95","author":"EM Chan","year":"2005","unstructured":"Chan EM, Andrade DM, Franceschetti S, Minassian B: Progressive myoclonus epilepsies: EPM1, EPM2A, EPM2B. Adv Neurol. 2005, 95: 47-57.","journal-title":"Adv Neurol"},{"key":"253_CR2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s10038-005-0321-1","volume":"51","author":"S Ganesh","year":"2006","unstructured":"Ganesh S, Puri R, Singh S, Mittal S, Dubey D: Recent advances in the molecular basis of Lafora\u2019s progressive myoclonus epilepsy. J Hum Genet. 2006, 51: 1-8. 10.1007\/s10038-005-0321-1.","journal-title":"J Hum Genet"},{"key":"253_CR3","doi-asserted-by":"publisher","first-page":"99","DOI":"10.2174\/187221412800604617","volume":"6","author":"C Spuch","year":"2012","unstructured":"Spuch C, Ortolano S, Navarro C: Lafora progressive myoclonus epilepsy: recent insights into cell degeneration. Recent Pat Endocr Metab Drug Discov. 2012, 6: 99-107. 10.2174\/187221412800604617.","journal-title":"Recent Pat Endocr Metab Drug Discov"},{"key":"253_CR4","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1001\/archneur.1968.00480010033002","volume":"19","author":"S Yokoi","year":"1968","unstructured":"Yokoi S, Austin J, Witmer F, Sakai M: Studies in myoclonus epilepsy (Lafora body form). Isolation and preliminary characterization of Lafora bodies in two cases. Arch Neurol. 1968, 19: 15-33. 10.1001\/archneur.1968.00480010033002.","journal-title":"Arch Neurol"},{"key":"253_CR5","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1038\/2470","volume":"20","author":"BA Minassian","year":"1998","unstructured":"Minassian BA, Lee JR, Herbrick JA, Huizenga J, Soder S, Mungall AJ, Dunham I, Gardner R, Fong CY, Carpenter S, Jardim L, Satishchandra P, Andermann E, Snead OC, Lopes-Cendes I, Tsui LC, Delgado-Escueta AV, Rouleau GA, Scherer SW: Mutations in a gene encoding a novel protein tyrosine phosphatase cause progressive myoclonus epilepsy. Nat Genet. 1998, 20: 171-174. 10.1038\/2470.","journal-title":"Nat Genet"},{"key":"253_CR6","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1093\/hmg\/8.2.345","volume":"8","author":"JM Serratosa","year":"1999","unstructured":"Serratosa JM, G\u00f3mez-Garre P, Gallardo ME, Anta B, de Bernab\u00e9 DB, Lindhout D, Augustijn PB, Tassinari CA, Malafosse RM, Topcu M, Grid D, Dravet C, Berkovic SF, de C\u00f3rdoba SR: A novel protein tyrosine phosphatase gene is mutated in progressive myoclonus epilepsy of the Lafora type (EPM2). Hum Mol Genet. 1999, 8: 345-352. 10.1093\/hmg\/8.2.345.","journal-title":"Hum Mol Genet"},{"key":"253_CR7","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1038\/ng1238","volume":"35","author":"EM Chan","year":"2003","unstructured":"Chan EM, Young EJ, Ianzano L, Munteanu I, Zhao X, Christopoulos CC, Avanzini G, Elia M, Ackerley CA, Jovic NJ, Bohlega S, Andermann E, Rouleau GA, Delgado-Escueta AV, Minassian BA, Scherer SW: Mutations in NHLRC1 cause progressive myoclonus epilepsy. Nat Genet. 2003, 35: 125-127. 10.1038\/ng1238.","journal-title":"Nat Genet"},{"key":"253_CR8","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1136\/jmg.40.9.671","volume":"40","author":"EM Chan","year":"2003","unstructured":"Chan EM, Bulman DE, Paterson AD, Turnbull J, Andermann E, Andermann F, Rouleau GA, Delgado-Escueta AV, Scherer SW, Minassian BA: Genetic mapping of a new Lafora progressive myoclonus epilepsy locus (EPM2B) on 6p22. J Med Genet. 2003, 40: 671-675. 10.1136\/jmg.40.9.671.","journal-title":"J Med Genet"},{"key":"253_CR9","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1212\/01.WNL.0000133215.65836.03","volume":"63","author":"EM Chan","year":"2004","unstructured":"Chan EM, Omer S, Ahmed M, Bridges LR, Bennett C, Scherer SW, Minassian BA: Progressive myoclonus epilepsy with polyglucosans (Lafora disease): evidence for a third locus. Neurology. 2004, 63: 565-567. 10.1212\/01.WNL.0000133215.65836.03.","journal-title":"Neurology"},{"key":"253_CR10","doi-asserted-by":"publisher","first-page":"2684","DOI":"10.1093\/brain\/aws205","volume":"135","author":"J Turnbull","year":"2012","unstructured":"Turnbull J, Girard JM, Lohi H, Chan EM, Wang P, Tiberia E, Omer S, Ahmed M, Bennett C, Chakrabarty A, Tyagi A, Liu Y, Pencea N, Zhao X, Scherer SW, Ackerley CA, Minassian BA: Early-onset Lafora body disease. Brain. 2012, 135: 2684-2698. 10.1093\/brain\/aws205.","journal-title":"Brain"},{"key":"253_CR11","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1002\/emmm.201100174","volume":"3","author":"J Valles-Ortega","year":"2011","unstructured":"Valles-Ortega J, Duran J, Garcia-Rocha M, Bosch C, Saez I, Pujadas L, Serafin A, Ca\u00f1as X, Soriano E, Delgado-Garc\u00eda JM, Gruart A, Guinovart JJ: Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease. EMBO Mol Med. 2011, 3: 667-681. 10.1002\/emmm.201100174.","journal-title":"EMBO Mol Med"},{"key":"253_CR12","doi-asserted-by":"publisher","first-page":"1251","DOI":"10.1093\/hmg\/11.11.1251","volume":"11","author":"S Ganesh","year":"2002","unstructured":"Ganesh S, Delgado-Escueta AV, Sakamoto T, Avila MR, Machado-Salas J, Hoshii Y, Akagi T, Gomi H, Suzuki T, Amano K, Agarwala KL, Hasegawa Y, Bai DS, Ishihara T, Hashikawa T, Itohara S, Cornford EM, Niki H, Yamakawa K: Targeted disruption of the Epm2a gene causes formation of lafora inclusion bodies, neurodegeneration, ataxia, myoclonus epilepsy and impaired behavioral response in mice. Hum Mol Genet. 2002, 11: 1251-1262. 10.1093\/hmg\/11.11.1251.","journal-title":"Hum Mol Genet"},{"key":"253_CR13","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1111\/j.1742-4658.2012.08549.x","volume":"280","author":"MS Gentry","year":"2013","unstructured":"Gentry MS, Rom\u00e1-Mateo C, Sanz P: Laforin, a protein with many faces: glucan phosphatase, adapter protein et alii. FEBS J. 2013, 280: 525-537. 10.1111\/j.1742-4658.2012.08549.x.","journal-title":"FEBS J"},{"key":"253_CR14","doi-asserted-by":"publisher","first-page":"19262","DOI":"10.1073\/pnas.0707952104","volume":"104","author":"VS Tagliabracci","year":"2007","unstructured":"Tagliabracci VS, Turnbull J, Wang W, Girard JM, Zhao X, Skurat AV, Delgado-Escueta AV, Minassian BA, Depaoli-Roach AA, Roach PJ: Laforin is a glycogen phosphatase, deficiency of which leads to elevated phosphorylation of glycogen in vivo. Proc Natl Acad Sci USA. 2007, 104: 19262-19266. 10.1073\/pnas.0707952104.","journal-title":"Proc Natl Acad Sci USA"},{"key":"253_CR15","doi-asserted-by":"publisher","first-page":"33816","DOI":"10.1074\/jbc.M807428200","volume":"283","author":"VS Tagliabracci","year":"2008","unstructured":"Tagliabracci VS, Girard JM, Segvich D, Meyer C, Turnbull J, Zhao X, Minassian BA, Depaoli-Roach AA, Roach PJ: Abnormal metabolism of glycogen phosphate as a cause for Lafora disease. J Biol Chem. 2008, 283: 33816-33825. 10.1074\/jbc.M807428200.","journal-title":"J Biol Chem"},{"key":"253_CR16","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1042\/BJ20111416","volume":"441","author":"PJ Roach","year":"2012","unstructured":"Roach PJ, Depaoli-Roach AA, Hurley TD, Tagliabracci VS: Glycogen and its metabolism: some new developments and old themes. Biochem J. 2012, 441: 763-787. 10.1042\/BJ20111416.","journal-title":"Biochem J"},{"key":"253_CR17","doi-asserted-by":"publisher","first-page":"2727","DOI":"10.1093\/hmg\/ddi306","volume":"14","author":"H Lohi","year":"2005","unstructured":"Lohi H, Ianzano L, Zhao XC, Chan EM, Turnbull J, Scherer SW, Ackerley CA, Minassian BA: Novel glycogen synthase kinase 3 and ubiquitination pathways in progressive myoclonus epilepsy. Hum Mol Genet. 2005, 14: 2727-2736. 10.1093\/hmg\/ddi306.","journal-title":"Hum Mol Genet"},{"key":"253_CR18","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1093\/hmg\/ddm339","volume":"17","author":"MC Solaz-Fuster","year":"2008","unstructured":"Solaz-Fuster MC, Gimeno-Alca\u00f1iz JV, Ros S, Fernandez-Sanchez ME, Garcia-Fojeda B, Criado Garcia O, Vilchez D, Dominguez J, Garcia-Rocha M, Sanchez-Piris M, Aguado C, Knecht E, Serratosa J, Guinovart JJ, Sanz P, Rodriguez de C\u00f3rdoba S: Regulation of glycogen synthesis by the laforin-malin complex is modulated by the AMP-activated protein kinase pathway. Hum Mol Genet. 2008, 17: 667-678.","journal-title":"Hum Mol Genet"},{"key":"253_CR19","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1093\/hmg\/ddh130","volume":"13","author":"EM Chan","year":"2004","unstructured":"Chan EM, Ackerley CA, Lohi H, Ianzano L, Cortez MA, Shannon P, Scherer SW, Minassian BA: Laforin preferentially binds the neurotoxic starch-like polyglucosans, which form in its absence in progressive myoclonus epilepsy. Hum Mol Genet. 2004, 13: 1117-1129. 10.1093\/hmg\/ddh130.","journal-title":"Hum Mol Genet"},{"key":"253_CR20","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1093\/hmg\/ddr590","volume":"21","author":"O Criado","year":"2012","unstructured":"Criado O, Aguado C, Gayarre J, Duran-Trio L, Garcia-Cabrero AM, Vernia S, San Mill\u00e1n B, Heredia M, Rom\u00e1-Mateo C, Mouron S, Juana-L\u00f3pez L, Dom\u00ednguez M, Navarro C, Serratosa JM, Sanchez M, Sanz P, Bovolenta P, Knecht E, Rodriguez de Cordoba S: Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy. Hum Mol Genet. 2012, 21: 1521-1533. 10.1093\/hmg\/ddr590.","journal-title":"Hum Mol Genet"},{"key":"253_CR21","doi-asserted-by":"publisher","first-page":"e1002037","DOI":"10.1371\/journal.pgen.1002037","volume":"7","author":"J Turnbull","year":"2011","unstructured":"Turnbull J, DePaoli-Roach AA, Zhao X, Cortez MA, Pencea N, Tiberia E, Piliguian M, Roach PJ, Wang P, Ackerley CA, Minassian BA: PTG depletion removes lafora bodies and rescues the fatal epilepsy of lafora disease. PLoS Genet. 2011, 7: e1002037-10.1371\/journal.pgen.1002037.","journal-title":"PLoS Genet"},{"key":"253_CR22","doi-asserted-by":"publisher","first-page":"549","DOI":"10.2165\/11319250-000000000-00000","volume":"24","author":"TS Monaghan","year":"2010","unstructured":"Monaghan TS, Delanty N: Lafora disease: epidemiology, pathophysiology and management. CNS Drugs. 2010, 24: 549-561. 10.2165\/11319250-000000000-00000.","journal-title":"CNS Drugs"},{"key":"253_CR23","first-page":"119","volume":"5","author":"DL Martin","year":"1998","unstructured":"Martin DL, Barke KE: Are GAD65 and GAD67 associated with specific pools of GABA in brain?. Perspect Dev Neurobiol. 1998, 5: 119-129.","journal-title":"Perspect Dev Neurobiol"},{"key":"253_CR24","doi-asserted-by":"publisher","first-page":"14563","DOI":"10.1074\/jbc.M412957200","volume":"280","author":"N Zampieri","year":"2005","unstructured":"Zampieri N, Xu CF, Neubert TA, Chao MV: Cleavage of p75 neurotrophin receptor by \u03b1-secretase and \u03b3-secretase requires specific receptor domains. J Biol Chem. 2005, 280: 14563-14571. 10.1074\/jbc.M412957200.","journal-title":"J Biol Chem"},{"key":"253_CR25","doi-asserted-by":"publisher","first-page":"849","DOI":"10.1016\/j.neuron.2005.05.029","volume":"46","author":"M Domeniconi","year":"2005","unstructured":"Domeniconi M, Zampieri N, Spencer T, Hilaire M, Mellado W, Chao MV, Filbin MT: MAG induces regulated intramembrane proteolysis of the p75 neurotrophin receptor to inhibit neurite outgrowth. Neuron. 2005, 46: 849-855. 10.1016\/j.neuron.2005.05.029.","journal-title":"Neuron"},{"key":"253_CR26","doi-asserted-by":"publisher","first-page":"3351","DOI":"10.1242\/jcs.055061","volume":"122","author":"M Vilar","year":"2009","unstructured":"Vilar M, Charalampopoulos I, Kenchappa RS, Reversi A, Klos-Applequist JM, Karaca E, Simi A, Spuch C, Choi S, Friedman WJ, Ericson J, Schiavo G, Carter BD, Ib\u00e1\u00f1ez CF: Ligand-independent signaling by disulfide-crosslinked dimers of the p75 neurotrophin receptor. J Cell Sci. 2009, 122: 3351-3357. 10.1242\/jcs.055061.","journal-title":"J Cell Sci"},{"key":"253_CR27","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1186\/1471-2202-12-39","volume":"12","author":"C Spuch","year":"2011","unstructured":"Spuch C, Carro E: The p75 neurotrophin receptor localization in blood-CSF barrier: expression in choroid plexus epithelium. BMC Neurosci. 2011, 12: 39-10.1186\/1471-2202-12-39.","journal-title":"BMC Neurosci"},{"key":"253_CR28","first-page":"1","volume":"6","author":"HL Busard","year":"1987","unstructured":"Busard HL, Renier WO, Gabreels FJ, Jaspar HH, Slooff JL, Janssen AJ, Van Haelst UJ: Lafora disease: a quantitative morphological and biochemical study of the cerebral cortex. Clin Neuropathol. 1987, 6: 1-6.","journal-title":"Clin Neuropathol"},{"key":"253_CR29","first-page":"459","volume-title":"The Synaptic Organization of the Brain","author":"R Douglas","year":"1998","unstructured":"Douglas R, Martin K: Neocortex. The Synaptic Organization of the Brain. Edited by: Shepherd GM. 1998, Oxford, UK: Oxford University Press, 459-509. 4","edition":"4"},{"key":"253_CR30","doi-asserted-by":"publisher","first-page":"2309","DOI":"10.1212\/01.WNL.0000147475.71345.AA","volume":"63","author":"L Canafoglia","year":"2004","unstructured":"Canafoglia L, Ciano C, Panzica F, Scaioli V, Zucca C, Agazzi P, Visani E, Avanzini G, Franceschetti S: Sensorimotor cortex excitability in Unverricht-Lundborg disease and Lafora body disease. Neurology. 2004, 63: 2309-2315. 10.1212\/01.WNL.0000147475.71345.AA.","journal-title":"Neurology"},{"key":"253_CR31","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1016\/j.eplepsyres.2010.01.006","volume":"89","author":"L Canafoglia","year":"2010","unstructured":"Canafoglia L, Ciano C, Visani E, Anversa P, Panzica F, Viri M, Gennaro E, Zara F, Madia F, Franceschetti S: Short and long interval cortical inhibition in patients with Unverricht-Lundborg and Lafora body disease. Epilepsy Res. 2010, 89: 232-237. 10.1016\/j.eplepsyres.2010.01.006.","journal-title":"Epilepsy Res"},{"key":"253_CR32","doi-asserted-by":"publisher","first-page":"8093","DOI":"10.1073\/pnas.110078997","volume":"97","author":"M Turmaine","year":"2000","unstructured":"Turmaine M, Raza A, Mahal A, Mangiarini L, Bates GP, Davis SW: Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington\u2019s disease. Proc Natl Acad Sci USA. 2000, 97: 8093-8097. 10.1073\/pnas.110078997.","journal-title":"Proc Natl Acad Sci USA"},{"key":"253_CR33","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1016\/j.expneurol.2012.04.008","volume":"236","author":"J Machado-Salas","year":"2012","unstructured":"Machado-Salas J, Avila-Costa MR, Guevara P, Guevara J, Dur\u00f3n RM, Bai D, Tanaka M, Yamakawa K, Delgado-Escueta AV: Ontogeny of Lafora bodies and neurocytoskeleton changes in laforin-deficient mice. Exp Neurol. 2012, 236: 131-140. 10.1016\/j.expneurol.2012.04.008.","journal-title":"Exp Neurol"},{"key":"253_CR34","first-page":"175","volume":"35","author":"H Tsuda","year":"1995","unstructured":"Tsuda H, Katsumi Y, Nakamura M, Ikeda A, Fukuyama H, Kimura J, Shibasaki H: Cerebral blood flow and metabolism in Lafora disease. Rinsho Shinkeigaku. 1995, 35: 175-179.","journal-title":"Rinsho Shinkeigaku"},{"key":"253_CR35","doi-asserted-by":"publisher","first-page":"22657","DOI":"10.1074\/jbc.M109.009688","volume":"284","author":"R Puri","year":"2009","unstructured":"Puri R, Suzuki T, Yamakawa K, Ganesh S: Hyperphosphorylation and aggregation of Tau in laforin-deficient mice, an animal model for lafora disease. J Biol Chem. 2009, 284: 22657-22663. 10.1074\/jbc.M109.009688.","journal-title":"J Biol Chem"},{"key":"253_CR36","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1093\/hmg\/ddr452","volume":"21","author":"R Puri","year":"2012","unstructured":"Puri R, Suzuki T, Yamakawa K, Ganesh S: Dysfunctions in endosomal-lysosomal and autophagy pathways underlie neuropathology in a Mouse model for Lafora disease. Hum Mol Genet. 2012, 21: 175-184. 10.1093\/hmg\/ddr452.","journal-title":"Hum Mol Genet"},{"key":"253_CR37","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1007\/s00401-009-0619-8","volume":"119","author":"MV Sofroniew","year":"2010","unstructured":"Sofroniew MV, Vinters HV: Astrocytes: biology and pathology. Acta Neuropathol. 2010, 119: 7-35. 10.1007\/s00401-009-0619-8.","journal-title":"Acta Neuropathol"},{"key":"253_CR38","doi-asserted-by":"publisher","first-page":"2867","DOI":"10.1093\/hmg\/ddq190","volume":"19","author":"C Aguado","year":"2010","unstructured":"Aguado C, Sarkar S, Korolchuk VI, Criado O, Vernia S, Boya P, Sanz P, de C\u00f3rdoba SR, Knecht E, Rubinsztein DC: Laforin, the most common protein mutated in Lafora disease, regulates autophagy. Hum Mol Genet. 2010, 19: 2867-2876. 10.1093\/hmg\/ddq190.","journal-title":"Hum Mol Genet"},{"key":"253_CR39","doi-asserted-by":"publisher","first-page":"628","DOI":"10.1038\/35023579","volume":"2","author":"LS Minamide","year":"2000","unstructured":"Minamide LS, Striegl AM, Boyle JA, Meberg PJ, Bamburg JR: Neurodegenerative stimuli induce persistent ADF\/cofilin-actin rods that disrupt distal neurite function. Nat Cell Biol. 2000, 2: 628-636. 10.1038\/35023579.","journal-title":"Nat Cell Biol"},{"key":"253_CR40","doi-asserted-by":"publisher","first-page":"1407","DOI":"10.1038\/nn1998","volume":"10","author":"D Vilchez","year":"2007","unstructured":"Vilchez D, Ros S, Cifuentes D, Pujadas L, Vall\u00e8s J, Garc\u00eda-Fojeda B, Criado-Garc\u00eda O, Fern\u00e1ndez-S\u00e1nchez E, Medra\u00f1o-Fern\u00e1ndez I, Dom\u00ednguez J, Garc\u00eda-Rocha M, Soriano E, Rodr\u00edguez de C\u00f3rdoba S, Guinovart JJ: Mechanism supressing glycogen synthesis in neurons and its demise progressive myoclobus epilepsy. Nat Neurosci. 2007, 10: 1407-1413. 10.1038\/nn1998.","journal-title":"Nat Neurosci"},{"key":"253_CR41","first-page":"77","volume":"1287","author":"MT Gottlieb","year":"1996","unstructured":"Gottlieb MT, Oren M: p53 in growth control and neoplasia. Biochim Biophys Acta. 1996, 1287: 77-102.","journal-title":"Biochim Biophys Acta"},{"key":"253_CR42","doi-asserted-by":"publisher","first-page":"1054","DOI":"10.1101\/gad.10.9.1054","volume":"10","author":"JL Ko","year":"1996","unstructured":"Ko JL, Prives C: p53: puzzle and paradigm. Genes Dev. 1996, 10: 1054-1072. 10.1101\/gad.10.9.1054.","journal-title":"Genes Dev"},{"key":"253_CR43","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1016\/S0169-328X(99)00202-8","volume":"72","author":"Y Tendler","year":"1999","unstructured":"Tendler Y, Weisinger G, Coleman R, Diamond E, Lischinsky S, Kerner H, Rotter V, Zinder O: Tissue-specific p53 expression in the nervous system. Brain Res Mol Brain Res. 1999, 72: 40-46. 10.1016\/S0169-328X(99)00202-8.","journal-title":"Brain Res Mol Brain Res"},{"key":"253_CR44","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.1002\/j.1460-2075.1995.tb07097.x","volume":"14","author":"O Eizenberg","year":"1995","unstructured":"Eizenberg O, Faber-Elman A, Gotlieb E, Oren M, Rotter V, Schwartz M: Direct involvement of p53 in programmed cell death of oligodendrocytes. EMBO J. 1995, 14: 1136-1144.","journal-title":"EMBO J"},{"key":"253_CR45","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1002\/dneu.20767","volume":"70","author":"LC Schecterson","year":"2010","unstructured":"Schecterson LC, Bothwell M: Neurotrophin receptors: old friends with new partners. Dev Neurobiol. 2010, 70: 332-338.","journal-title":"Dev Neurobiol"},{"key":"253_CR46","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1146\/annurev.ne.19.030196.001445","volume":"19","author":"GR Lewin","year":"1996","unstructured":"Lewin GR, Barde YA: Physiology of neurotrophins. Annu Rev Neurosci. 1996, 19: 289-317. 10.1146\/annurev.ne.19.030196.001445.","journal-title":"Annu Rev Neurosci"},{"key":"253_CR47","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1515\/REVNEURO.1997.8.1.1","volume":"8","author":"B Lu","year":"1997","unstructured":"Lu B, Figurov A: Role of neurotrophins in synapse development and plasticity. Rev Neurosci. 1997, 8: 1-12.","journal-title":"Rev Neurosci"},{"key":"253_CR48","doi-asserted-by":"publisher","first-page":"487","DOI":"10.1093\/cercor\/bhi128","volume":"16","author":"S Alcantara","year":"2006","unstructured":"Alcantara S, Pozas E, Iba\u00f1ez CF, Soriano E: BDNF-modulated spatial organization of Cajal-Retzius and GABAergic neurons in the marginal zone plays a role in the development of cortical organization. Cereb Cortex. 2006, 16: 487-499.","journal-title":"Cereb Cortex"},{"key":"253_CR49","doi-asserted-by":"publisher","first-page":"14318","DOI":"10.1523\/JNEUROSCI.0709-12.2012","volume":"32","author":"EG Waterhouse","year":"2012","unstructured":"Waterhouse EG, An JJ, Orefice LL, Baydyuk M, Liao GY, Zheng K, Lu B, Xu B: BDNF promotes differentiation and maturation of adult-born neurons through GABAergic transmission. J Neurosci. 2012, 32: 14318-14330. 10.1523\/JNEUROSCI.0709-12.2012.","journal-title":"J Neurosci"},{"key":"253_CR50","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/S0166-2236(00)01682-9","volume":"19","author":"DK Binder","year":"2001","unstructured":"Binder DK, Croll SD, Gall CM, Scharfman HE: BDNF and epilepsy: too much of a good thing?. Trends Neurosci. 2001, 19: 47-53.","journal-title":"Trends Neurosci"},{"key":"253_CR51","doi-asserted-by":"publisher","first-page":"318","DOI":"10.1016\/S0166-2236(00)01869-5","volume":"24","author":"S Reibel","year":"2001","unstructured":"Reibel S, Depaulis A, Larmet Y: BDNF and epilepsy\u2013the bad could turn out to be good. Trends Neurosci. 2001, 24: 318-319. 10.1016\/S0166-2236(00)01869-5.","journal-title":"Trends Neurosci"},{"key":"253_CR52","doi-asserted-by":"crossref","first-page":"6340","DOI":"10.1523\/JNEUROSCI.20-17-06340.2000","volume":"20","author":"WJ Friedman","year":"2000","unstructured":"Friedman WJ: Neurotrophins induce death of hippocampal neurons via the p75 receptor. J Neurosci. 2000, 20: 6340-6346.","journal-title":"J Neurosci"},{"key":"253_CR53","doi-asserted-by":"crossref","first-page":"2409","DOI":"10.1523\/JNEUROSCI.22-07-02409.2002","volume":"22","author":"T Naumann","year":"2002","unstructured":"Naumann T, Casademunt E, Hollerbach E, Hofmann J, Dechant G, Frotscher M, Barde YA: Complete deletion of the neurotrophin receptor p75NTR leads to long-lasting increases in the number of basal forebrain cholinergic neurons. J Neurosci. 2002, 22: 2409-2418.","journal-title":"J Neurosci"},{"key":"253_CR54","doi-asserted-by":"publisher","first-page":"20358","DOI":"10.1074\/jbc.M109.082834","volume":"285","author":"RS Kenchappa","year":"2010","unstructured":"Kenchappa RS, Tep C, Korade Z, Urra S, Bronfman FC, Yoon SO, Carter BD: p75 neurotrophin receptor-mediated apoptosis in sympathetic neurons involves a biphasic activation of JNK and up-regulation of tumor necrosis factor alpha converting enzyme\/ADAM17. J Biol Chem. 2010, 285: 20358-20368. 10.1074\/jbc.M109.082834.","journal-title":"J Biol Chem"},{"key":"253_CR55","doi-asserted-by":"publisher","first-page":"42161","DOI":"10.1074\/jbc.M306028200","volume":"278","author":"KM Jung","year":"2003","unstructured":"Jung KM, Tan S, Landman N, Petrova K, Murray S, Lewis R, Kim PK, Kim DS, Ryu SH, Chao MV, Kim TW: Regulated intramembrane proteolysis of the p75 neurotrophin receptor modulates its association with the TrkA receptor. J Biol Chem. 2003, 278: 42161-42169. 10.1074\/jbc.M306028200.","journal-title":"J Biol Chem"},{"key":"253_CR56","doi-asserted-by":"crossref","first-page":"5425","DOI":"10.1523\/JNEUROSCI.23-13-05425.2003","volume":"23","author":"KC Kanning","year":"2003","unstructured":"Kanning KC, Hudson M, Amieux PS, Wiley JC, Bothwell M, Schecterson LC: Proteolytic processing of the p75 neurotrophin receptor and two homologues generates C-terminal fragments with signaling capability. J Neurosci. 2003, 23: 5425-5436.","journal-title":"J Neurosci"},{"key":"253_CR57","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1016\/S0896-6273(02)00704-3","volume":"34","author":"JO Ebinu","year":"2002","unstructured":"Ebinu JO, Yankner BA: A RIP tide in neuronal signal transduction. Neuron. 2002, 34: 499-502. 10.1016\/S0896-6273(02)00704-3.","journal-title":"Neuron"},{"key":"253_CR58","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/S0165-0173(99)00003-X","volume":"29","author":"JB Cavanagh","year":"1999","unstructured":"Cavanagh JB: Corpora-amylacea and the family of polyglucosan diseases. Brain Res Brain Res Rev. 1999, 29: 265-295. 10.1016\/S0165-0173(99)00003-X.","journal-title":"Brain Res Brain Res Rev"},{"key":"253_CR59","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/j.mcn.2010.09.006","volume":"46","author":"AL Perdigoto","year":"2011","unstructured":"Perdigoto AL, Chaudhry N, Barnes GN, Filbin MT, Carter BD: A novel role for PTEN in the inhibition of neurite outgrowth by muelin-associated glycoprotein in cortical neurons. Mol Cell Neurosci. 2011, 46: 235-244. 10.1016\/j.mcn.2010.09.006.","journal-title":"Mol Cell Neurosci"},{"key":"253_CR60","doi-asserted-by":"publisher","first-page":"25372","DOI":"10.1074\/jbc.M110.148668","volume":"285","author":"AA DePaoli-Roach","year":"2010","unstructured":"DePaoli-Roach AA, Tagliabracci VS, Segvich DM, Meyer CM, Irimia JM, Roach PJ: Genetic depletion of the malin E3 ubiquitin ligase in mice leads to lafora bodies and the accumulation of insoluble laforin. J Biol Chem. 2010, 285: 25372-25381. 10.1074\/jbc.M110.148668.","journal-title":"J Biol Chem"},{"key":"253_CR61","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1212\/WNL.0b013e31825dcea6","volume":"79","author":"AV Delgado-Escueta","year":"2012","unstructured":"Delgado-Escueta AV: Lafora progressive myoclonus epilepsy: glycogen storage disease vs neurodegenerative disease. Neurology. 2012, 79: 21-22. 10.1212\/WNL.0b013e31825dcea6.","journal-title":"Neurology"},{"key":"253_CR62","doi-asserted-by":"publisher","first-page":"11960","DOI":"10.1073\/pnas.220413597","volume":"97","author":"X Fang","year":"2000","unstructured":"Fang X, Yu SX, Lu Y, Bast RC, Woodgett JR, Mills GB: Phosphorylation and inactivation of glycogen synthase kinase 3 by protein kinase A. Proc Natl Acad Sci USA. 2000, 97: 11960-11965. 10.1073\/pnas.220413597.","journal-title":"Proc Natl Acad Sci USA"},{"key":"253_CR63","doi-asserted-by":"publisher","first-page":"37526","DOI":"10.1074\/jbc.M502699200","volume":"280","author":"E Szatmari","year":"2005","unstructured":"Szatmari E, Habas A, Yang P, Zheng JJ, Hagg T, Hetman M: A positive feedback loop between glycogen synthase kinase 3beta and protein phosphatase 1 after stimulation of NR2B NMDA receptors in forebrain neurons. J Biol Chem. 2005, 280: 37526-37535. 10.1074\/jbc.M502699200.","journal-title":"J Biol Chem"},{"key":"253_CR64","first-page":"60","volume":"13","author":"HL Busard","year":"1994","unstructured":"Busard HL, Span JP, Renkawek K, Renier WO, Gabre\u00ebls FJ, Slooff JL, Hof MA V\u2019t: Polyglucosan bodies in brain tissue: a systematic study. Clin Neuropathol. 1994, 13: 60-63.","journal-title":"Clin Neuropathol"},{"key":"253_CR65","doi-asserted-by":"publisher","first-page":"628","DOI":"10.1016\/j.tibs.2009.08.002","volume":"34","author":"MS Gentry","year":"2009","unstructured":"Gentry MS, Dixon JE, Worby CA: Lafora disease: insights into neurodegeneration from plant metabolism. Trends Biochem Sci. 2009, 34: 628-639. 10.1016\/j.tibs.2009.08.002.","journal-title":"Trends Biochem Sci"},{"key":"253_CR66","doi-asserted-by":"publisher","first-page":"719","DOI":"10.1002\/emmm.201200241","volume":"4","author":"J Duran","year":"2012","unstructured":"Duran J, Tevy MF, Garcia-Rocha M, Calb\u00f3 J, Mil\u00e1n M, Guinovart JJ: Deleterious effects of neuronal accumulation of glycogen in flies and mice. EMBO Mol Med. 2012, 4: 719-729. 10.1002\/emmm.201200241.","journal-title":"EMBO Mol Med"},{"key":"253_CR67","doi-asserted-by":"publisher","first-page":"5608","DOI":"10.1016\/j.biomaterials.2010.03.042","volume":"31","author":"C Spuch","year":"2010","unstructured":"Spuch C, Antequera D, Portero A, Orive G, Hern\u00e1ndez RM, Molina JA, Bermejo-Pareja F, Pedraz JL, Carro E: The effect of encapsulated VEGF-secreting cells on brain amyloid load and behavioral impairment in a mouse model of Alzheimer\u2019s disease. Biomaterials. 2010, 31: 5608-5618. 10.1016\/j.biomaterials.2010.03.042.","journal-title":"Biomaterials"},{"key":"253_CR68","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1006\/dbio.1995.1019","volume":"167","author":"LJ Huber","year":"1995","unstructured":"Huber LJ, Chao MV: Mesenchymal and neuronal cell expression of the p75 neurotrophin receptor gene occur by different mechanisms. Dev Biol. 1995, 167: 227-238. 10.1006\/dbio.1995.1019.","journal-title":"Dev Biol"},{"key":"253_CR69","doi-asserted-by":"publisher","first-page":"1082","DOI":"10.1016\/j.neuroscience.2009.04.031","volume":"161","author":"ZC Baquet","year":"2009","unstructured":"Baquet ZC, Williams D, Brody J, Smeyne RJ: A comparison of model-based (2D) and design-based (3D) stereological methods for estimating cell number in the substantia nigra pars compacta (SNpc) of the C57BL\/6\u00a0J Mouse. Neuroscience. 2009, 161: 1082-1090. 10.1016\/j.neuroscience.2009.04.031.","journal-title":"Neuroscience"}],"container-title":["Molecular Brain"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/1756-6606-7-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,23]],"date-time":"2024-05-23T18:51:43Z","timestamp":1716490303000},"score":1,"resource":{"primary":{"URL":"https:\/\/molecularbrain.biomedcentral.com\/articles\/10.1186\/1756-6606-7-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1,28]]},"references-count":69,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014,12]]}},"alternative-id":["253"],"URL":"https:\/\/doi.org\/10.1186\/1756-6606-7-7","relation":{},"ISSN":["1756-6606"],"issn-type":[{"value":"1756-6606","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,1,28]]},"assertion":[{"value":"6 November 2013","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 January 2014","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 January 2014","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"7"}}