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@ARTICLE{Fodder:258679,
author = {Fodder, Katherine and Murthy, Megha and Rizzu, Patrizia and
Toomey, Christina E and Hasan, Rahat and Humphrey, Jack and
Raj, Towfique and Lunnon, Katie and Mill, Jonathan and
Heutink, Peter and Lashley, Tammaryn and Bettencourt,
Conceição},
title = {{B}rain {DNA} methylomic analysis of frontotemporal lobar
degeneration reveals {OTUD}4 in shared dysregulated
signatures across pathological subtypes.},
journal = {Acta neuropathologica},
volume = {146},
number = {1},
issn = {0001-6322},
address = {Heidelberg},
publisher = {Springer},
reportid = {DZNE-2023-00652},
pages = {77 - 95},
year = {2023},
abstract = {Frontotemporal lobar degeneration (FTLD) is an umbrella
term describing the neuropathology of a clinically,
genetically and pathologically heterogeneous group of
diseases, including frontotemporal dementia (FTD) and
progressive supranuclear palsy (PSP). Among the major FTLD
pathological subgroups, FTLD with TDP-43 positive inclusions
(FTLD-TDP) and FTLD with tau-positive inclusions (FTLD-tau)
are the most common, representing about $90\%$ of the cases.
Although alterations in DNA methylation have been
consistently associated with neurodegenerative diseases,
including Alzheimer's disease and Parkinson's disease,
little is known for FTLD and its heterogeneous subgroups and
subtypes. The main goal of this study was to investigate DNA
methylation variation in FTLD-TDP and FTLD-tau. We used
frontal cortex genome-wide DNA methylation profiles from
three FTLD cohorts (142 FTLD cases and 92 controls),
generated using the Illumina 450K or EPIC microarrays. We
performed epigenome-wide association studies (EWAS) for each
cohort followed by meta-analysis to identify shared
differentially methylated loci across FTLD
subgroups/subtypes. In addition, we used weighted gene
correlation network analysis to identify co-methylation
signatures associated with FTLD and other disease-related
traits. Wherever possible, we also incorporated relevant
gene/protein expression data. After accounting for a
conservative Bonferroni multiple testing correction, the
EWAS meta-analysis revealed two differentially methylated
loci in FTLD, one annotated to OTUD4 (5'UTR-shore) and the
other to NFATC1 (gene body-island). Of these loci, OTUD4
showed consistent upregulation of mRNA and protein
expression in FTLD. In addition, in the three independent
co-methylation networks, OTUD4-containing modules were
enriched for EWAS meta-analysis top loci and were strongly
associated with the FTLD status. These co-methylation
modules were enriched for genes implicated in the ubiquitin
system, RNA/stress granule formation and glutamatergic
synaptic signalling. Altogether, our findings identified
novel FTLD-associated loci, and support a role for DNA
methylation as a mechanism involved in the dysregulation of
biological processes relevant to FTLD, highlighting novel
potential avenues for therapeutic development.},
keywords = {Humans / Frontotemporal Dementia: pathology /
Frontotemporal Lobar Degeneration: pathology / Brain:
pathology / Pick Disease of the Brain: pathology / DNA / tau
Proteins: metabolism / Ubiquitin-Specific Proteases:
metabolism / Co-methylation (Other) / DNA methylation
(Other) / EWAS (Other) / Frontotemporal dementia (Other) /
Human brain tissue (Other) / Progressive supranuclear palsy
(Other) / DNA (NLM Chemicals) / tau Proteins (NLM Chemicals)
/ OTUD4 protein, human (NLM Chemicals) / Ubiquitin-Specific
Proteases (NLM Chemicals)},
cin = {AG Rizzu / AG Heutink 1},
ddc = {610},
cid = {I:(DE-2719)1210009 / I:(DE-2719)1210002},
pnm = {354 - Disease Prevention and Healthy Aging (POF4-354)},
pid = {G:(DE-HGF)POF4-354},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37149835},
pmc = {pmc:PMC10261190},
doi = {10.1007/s00401-023-02583-z},
url = {https://pub.dzne.de/record/258679},
}