{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T10:08:29Z","timestamp":1791108509650,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":2472,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1998,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We report the neuropathological features in 6 members of a Volga German family with autosomal dominant Alzheimer's disease linked to chromosome 1 who had a presenilin\u20102 mutation (N<jats:sup>141<\/jats:sup>I). The most significant feature in this family was the presence of severe or moderately severe amyloid angiopathy in five family members with clinical dementia. The index case with the presenilin\u20102 mutation had late\u2010onset dementia at age 73 years, died of an acute intracerebral hemorrhage, and pathologically showed severe amyloid angiopathy but only rare neuritic senile plaques and neurofibrillary tangles. That she was apolipoprotein E \u03f52\/3 heterozygous suggests that the \u03f52 allele may have exerted a selective protective effect resulting in late onset relatively mild Alzheimer's disease despite severe amyloid angiopathy. This family emphasizes the need for more investigation into the role of presenilin mutation in amyloid deposition, especially in the cerebral vasculature, and the role of these changes in clinical dementia.<\/jats:p>","DOI":"10.1002\/ana.410430124","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T15:10:19Z","timestamp":1104592219000},"page":"131-135","source":"Crossref","is-referenced-by-count":63,"title":["Amyloid angiopathy in a volga german family with Alzheimer's disease and a presenilin\u20102 mutation (N<sup>141<\/sup>I)"],"prefix":"10.1002","volume":"43","author":[{"given":"David","family":"Nochlin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas D.","family":"Bird","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen J.","family":"Nemens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melvyn J.","family":"Ball","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Mark","family":"Sumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410250104"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.7638622"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.4.479"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/375754a0"},{"key":"e_1_2_1_6_2","first-page":"1839","article-title":"Antibodies to presenilin proteins detect neurofibrillary tangles in Alzheimer's disease","volume":"149","author":"Murphy GM","year":"1997","journal-title":"Am J Pathol"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)92379-9"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410410110"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(85)90965-1"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00308809"},{"key":"e_1_2_1_11_2","first-page":"212","article-title":"Comparison of the severity of neuropathologic changes in familial and sporadic Alzheimer's disease","volume":"7","author":"Nochlin D","year":"1993","journal-title":"Alzheimer Dis Assoc Disord"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90317-R"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/95.2.387"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.58.6.699"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.14.6.915"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1996.00550010053016"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(96)00094-9"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.8346443"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390110"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.27.8.1333"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0694-180"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410370510"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.1994.1019"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410430124","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410430124","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,28]],"date-time":"2023-10-28T19:23:27Z","timestamp":1698521007000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410430124"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,1]]},"references-count":22,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1998,1]]}},"alternative-id":["10.1002\/ana.410430124"],"URL":"https:\/\/doi.org\/10.1002\/ana.410430124","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,1]]}}}