{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T10:52:04Z","timestamp":1791111124170,"version":"4.1.0"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,9,27]],"date-time":"2006-09-27T00:00:00Z","timestamp":1159315200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2006,10]]},"abstract":"<jats:p>Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP\u201017) is a hereditary progressive neurodegenerative disorder. FTDP\u201017 was originally defined in Ann Arbor, Michigan, in 1996. Since then, more than 100 families with FTDP\u201017 have been described throughout the world, including 18 families identified in Japan. Genetic studies have identified 40 different mutations in the microtubule\u2010associated protein tau (<jats:italic>MAPT<\/jats:italic>) gene. The clinical features of FTDP\u201017 are characterized by behavioral, cognitive and motor disturbances that may occur in various combinations and degrees. Neuropathologic examination shows that various degrees of atrophy may be present in the frontal and temporal lobes, basal ganglia, amygdala and hippocampus. All the brains from patients with FTDP\u201017 have also shown the presence of tau deposits in neurons and glial cells. Mutations in <jats:italic>MAPT<\/jats:italic> may result in the increased splicing of exon 10, leading to 4\u2010repeat tau depositions in both neurons and glial cells. <jats:italic>MAPT<\/jats:italic> mutations outside of exon 10 show 3\u2010 and 4\u2010repeat tau deposits, predominantly in neurons with less glial pathology. Neuronal pathology may resemble that of Alzheimer\u2019s disease or Pick\u2019s disease because of the presence of neurofibrillary tangles or Pick\u2010like bodies, whereas glial pathology may resemble that of progressive supranuclear palsy or corticobasal degeneration because of the presence of coiled bodies, tufted astrocytes or astrocytic plaques. Correlations between genetic mutations and the heterogeneity of clinical and neuropathologic features remain unclear.<\/jats:p>","DOI":"10.1111\/j.1440-1789.2006.00702.x","type":"journal-article","created":{"date-parts":[[2006,9,27]],"date-time":"2006-09-27T09:21:05Z","timestamp":1159348865000},"page":"471-474","source":"Crossref","is-referenced-by-count":15,"title":["Neuropathology of familial tauopathy"],"prefix":"10.1111","volume":"26","author":[{"given":"Yoshio","family":"Tsuboi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,9,27]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.13.7737"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410430617"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/31508"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410410606"},{"key":"e_1_2_4_6_2","first-page":"107","article-title":"Japanese contribution to the understanding of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP\u201017)","volume":"55","author":"Tsuboi Y","year":"2003","journal-title":"No To Shinkei"},{"key":"e_1_2_4_7_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.60.10.1454"},{"key":"e_1_2_4_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-4580(00)00202-5"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199806000-00006"},{"key":"e_1_2_4_10_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.24.1.1121"},{"key":"e_1_2_4_11_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.10.5598"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1440-1789.2006.00702.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1440-1789.2006.00702.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T03:43:28Z","timestamp":1696909408000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1440-1789.2006.00702.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,9,27]]},"references-count":10,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2006,10]]}},"alternative-id":["10.1111\/j.1440-1789.2006.00702.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1440-1789.2006.00702.x","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,9,27]]}}}