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A\u03b2 can also deposit in blood vessel walls leading to cerebral amyloid angiopathy (CAA). While amyloid plaques in AD brains are constant, CAA varies among cases. The study focuses on differences observed between rare and poorly studied patient groups with <jats:italic>APP<\/jats:italic> duplications (<jats:italic>APP<\/jats:italic>dup) and Down syndrome (DS) reported to have higher frequencies of elevated CAA levels in comparison to sporadic AD (sAD), most of <jats:italic>APP<\/jats:italic> mutations, and controls. We compared A\u03b2 and tau pathologies in <jats:italic>postmortem<\/jats:italic> brain tissues across cases and A\u03b2 peptides using mass spectrometry (MS). We further characterized the spatial distribution of A\u03b2 peptides with MS-brain imaging. While intraparenchymal A\u03b2 deposits were numerous in sAD, DS with AD (DS-AD) and AD with <jats:italic>APP<\/jats:italic> mutations, these were less abundant in <jats:italic>APP<\/jats:italic>dup. On the contrary, A\u03b2 deposits in the blood vessels were abundant in <jats:italic>APP<\/jats:italic>dup and DS-AD while only <jats:italic>APP<\/jats:italic>dup cases displayed high A\u03b2 deposits in capillaries. Investigation of A\u03b2 peptide profiles showed a specific increase in A\u03b2x-37, A\u03b2x-38 and A\u03b2x-40 but not A\u03b2x-42 in <jats:italic>APP<\/jats:italic>dup cases and to a lower extent in DS-AD cases. Interestingly, N-truncated A\u03b22-x peptides were particularly increased in <jats:italic>APP<\/jats:italic>dup compared to all other groups. This result was confirmed by MS-imaging of leptomeningeal and parenchymal vessels from an <jats:italic>APP<\/jats:italic>dup case, suggesting that CAA is associated with accumulation of shorter A\u03b2 peptides truncated both at N- and C-termini in blood vessels. Altogether, this study identified striking differences in the localization and composition of A\u03b2 deposits between AD cases, particularly <jats:italic>APP<\/jats:italic>dup and DS-AD, both carrying three genomic copies of the <jats:italic>APP<\/jats:italic> gene. Detection of specific A\u03b2 peptides in CSF or plasma of these patients could improve the diagnosis of CAA and their inclusion in anti-amyloid immunotherapy treatments.<\/jats:p>","DOI":"10.1007\/s00401-024-02756-4","type":"journal-article","created":{"date-parts":[[2024,7,18]],"date-time":"2024-07-18T22:01:30Z","timestamp":1721340090000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Amyloid-\u03b2 peptide signature associated with cerebral amyloid angiopathy in familial Alzheimer\u2019s disease with APPdup and Down syndrome"],"prefix":"10.1007","volume":"148","author":[{"given":"Amal","family":"Kasri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Camporesi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleni","family":"Gkanatsiou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Boluda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunnar","family":"Brinkmalm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lev","family":"Stimmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junyue","family":"Ge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"Hanrieder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Villain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Duyckaerts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yannick","family":"Vermeiren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah E.","family":"Pape","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ga\u00ebl","family":"Nicolas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annie","family":"Laquerri\u00e8re","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter Paul","family":"De Deyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Wallon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaj","family":"Blennow","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andre","family":"Strydom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Zetterberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2462-7150","authenticated-orcid":false,"given":"Marie-Claude","family":"Potier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,18]]},"reference":[{"key":"2756_CR1","doi-asserted-by":"publisher","first-page":"303","DOI":"10.3233\/JAD-2011-110911","volume":"28","author":"A Antonell","year":"2012","unstructured":"Antonell A, Gelpi E, Sanchez-Valle R, Martinez R, Molinuevo JL, Llado A (2012) Breakpoint sequence analysis of an AbetaPP locus duplication associated with autosomal dominant Alzheimer\u2019s disease and severe cerebral amyloid angiopathy. 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Ltd, Novartis, Ono Pharma, Prothena, Roche Diagnostics, and Siemens Healthineers; has served at data monitoring committees for Julius Clinical and Novartis; has given lectures, produced educational materials and participated in educational programs for AC Immune, Biogen, Celdara Medical, Eisai and Roche Diagnostics; and is a co-founder of Brain Biomarker Solutions in Gothenburg AB (BBS), which is a part of the GU Ventures Incubator Program, outside the work presented in this paper. HZ has served at scientific advisory boards and\/or as a consultant for Abbvie, Acumen, Alector, Alzinova, ALZPath, Amylyx, Annexon, Apellis, Artery Therapeutics, AZTherapies, Cognito Therapeutics, CogRx, Denali, Eisai, Merry Life, Nervgen, Novo Nordisk, Optoceutics, Passage Bio, Pinteon Therapeutics, Prothena, Red Abbey Labs, reMYND, Roche, Samumed, Siemens Healthineers, Triplet Therapeutics, and Wave, has given lectures in symposia sponsored by Alzecure, Biogen, Cellectricon, Fujirebio, Lilly, and Roche, and is a co-founder of Brain Biomarker Solutions in Gothenburg AB (BBS), which is a part of the GU Ventures Incubator Program (outside submitted work). AS has served on scientific advisory boards for AC-Immune, and ProMIS Neurosciences.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}}],"article-number":"8"}}