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(2006). ALLEN BRAIN ATLAS. Available from: <ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/www.brain-map.org\/\" xlink:type=\"simple\">http:\/\/www.brain-map.org\/<\/ext-link>."},{"key":"ref30","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1007\/s00204-008-0360-4","article-title":"The use of Fluoro-Jade in primary neuronal cell cultures","volume":"83","author":"G Schmuck","year":"2009","journal-title":"Arch Toxicol"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/S1385-299X(98)00050-6","article-title":"Markers for neuronal degeneration in organotypic slice cultures","volume":"3","author":"J Noraberg","year":"1999","journal-title":"Brain Res Brain Res Protoc"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1111\/j.1530-0277.1993.tb00825.x","article-title":"Thiamin and thiamin phosphate ester deficiency assessed by high performance liquid chromatography in four clinical cases of Wernicke 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