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@ARTICLE{Liu:279512,
author = {Liu, Haiyan and Marsh, Thomas W and Shi, Xinyu and Renton,
Alan E and Bowling, Kevin M and Ziegemeier, Ellen and Wang,
Guoqiao and Cao, Yuchen and Aristel, Alisha and Li, Jessie
and Dickson, Alexa and Perrin, Richard J and Goate, Alison M
and Fernández, Victoria and Day, Gregory S and Doering,
Michelle and Daniels, Alisha and Gordon, Brian A and
Benzinger, Tammie L S and Hassenstab, Jason and Ibanez,
Laura and Supnet-Bell, Charlene and Xiong, Chengjie and
Allegri, Ricardo and Berman, Sarah B and Fox, Nick C and
Ryan, Natalie S and Huey, Edward D and Vöglein, Jonathan
and Noble, James M and Roh, Jee Hoon and Jucker, Mathias and
Laske, Christoph and Ikeuchi, Takeshi and Sanchez-Valle,
Raquel and Schofield, Peter R and Mendez, Patricio Chrem and
Chhatwal, Jasmeer P and Farlow, Martin and Lee, Jae-Hong and
Levey, Allan I and Levin, Johannes and Lopera, Francisco and
Martins, Ralph and Niimi, Yoshiki and Rosa-Neto, Pedro and
Morris, John C and Bateman, Randall J and Karch, Celeste M
and Cruchaga, Carlos and McDade, Eric and Llibre-Guerra,
Jorge J},
title = {{T}he landscape of autosomal-dominant {A}lzheimer's
disease: global distribution and age of onset.},
journal = {Brain},
volume = {148},
number = {7},
issn = {0006-8950},
address = {Oxford},
publisher = {Oxford Univ. Press},
reportid = {DZNE-2025-00839},
pages = {2429 - 2440},
year = {2025},
abstract = {We present a comprehensive global analysis of genetic
variants associated with autosomal-dominant Alzheimer's
disease (ADAD). A total of 550 variants in the APP, PSEN1
and PSEN2 genes were identified, of which 279 were
classified as pathogenic or likely pathogenic based on
American College of Medical Genetics and Genomics and the
Association for Molecular Pathology criteria, utilizing data
from the Dominantly Inherited Alzheimer Network (DIAN),
literature and public databases. Symptomatic age at onset
(AAO) data were estimated for 227 of these variants,
allowing detailed characterization of their frequency,
pathogenicity and AAO. Importantly, 226 variants met
eligibility criteria for inclusion in disease-modifying
clinical trials. Furthermore, we demonstrated the predictive
value of mean variant AAO and parental AAO in predicting
symptomatic AAO, validated against converters who became
symptomatic during follow-up in the DIAN Observational
Study. This dataset provides critical insights into the
global landscape of ADAD and reveals the genetic and AAO
heterogeneity of ADAD variants while refining variant trial
eligibility criteria.},
keywords = {Humans / Alzheimer Disease: genetics / Alzheimer Disease:
epidemiology / Age of Onset / Presenilin-1: genetics /
Presenilin-2: genetics / Male / Female / Amyloid
beta-Protein Precursor: genetics / Middle Aged / Aged /
Mutation / Genetic Variation / amyloid precursor protein
(Other) / pathogenicity (Other) / presenilin-1 (Other) /
presenilin-2 (Other) / prevalence (Other) / Presenilin-1
(NLM Chemicals) / Presenilin-2 (NLM Chemicals) / PSEN1
protein, human (NLM Chemicals) / PSEN2 protein, human (NLM
Chemicals) / Amyloid beta-Protein Precursor (NLM Chemicals)
/ APP protein, human (NLM Chemicals)},
cin = {Clinical Research (Munich) / AG Jucker / AG Gasser / AG
Levin},
ddc = {610},
cid = {I:(DE-2719)1111015 / I:(DE-2719)1210001 /
I:(DE-2719)1210000 / I:(DE-2719)1111016},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 352 -
Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39903689},
pmc = {pmc:PMC12233549},
doi = {10.1093/brain/awaf038},
url = {https://pub.dzne.de/record/279512},
}