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Bueler","year":"1992","journal-title":"Nature"},{"issue":"6","key":"ref78","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1016\/j.bbi.2008.02.001","article-title":"Sleep-wake behavior and responses to sleep deprivation of mice lacking both interleukin-1 beta receptor 1 and tumor necrosis factor-alpha receptor 1","volume":"22","author":"F Baracchi","year":"2008","journal-title":"Brain Behav Immun"},{"issue":"7","key":"ref79","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nmeth.2019","article-title":"Fiji: an open-source platform for biological-image analysis","volume":"9","author":"J Schindelin","year":"2012","journal-title":"Nat Methods"},{"issue":"1","key":"ref80","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1111\/j.1750-3639.2000.tb00240.x","article-title":"Creutzfeldt-Jakob disease: Carnoy&apos;s fixative improves the immunohistochemistry of the proteinase K-resistant prion protein","volume":"10","author":"G 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