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@ARTICLE{Sen:274003,
author = {Sen, Paromita and Ortiz, Oskar and Brivio, Elena and
Menegaz, Danusa and Sotillos Elliott, Laura and Du, Ying and
Ries, Clemens and Chen, Alon and Wurst, Wolfgang and Lopez,
Juan Pablo and Eder, Matthias and Deussing, Jan M},
title = {{A} bipolar disorder-associated missense variant alters
adenylyl cyclase 2 activity and promotes mania-like
behavior.},
journal = {Molecular psychiatry},
volume = {30},
number = {1},
issn = {1359-4184},
address = {London},
publisher = {Macmillan},
reportid = {DZNE-2024-01431},
pages = {97 - 110},
year = {2025},
abstract = {The single nucleotide polymorphism rs13166360, causing a
substitution of valine (Val) 147 to leucine (Leu) in the
adenylyl cyclase 2 (ADCY2), has previously been associated
with bipolar disorder (BD). Here we show that the
disease-associated ADCY2 missense mutation diminishes the
enzyme´s capacity to generate the second messenger
3',5'-cylic adenosine monophosphate (cAMP) by altering its
subcellular localization. We established mice specifically
carrying the Val to Leu substitution using CRISPR/Cas9-based
gene editing. Mice homozygous for the Leu variant display
symptoms of a mania-like state accompanied by cognitive
impairments. Mutant animals show additional characteristic
signs of rodent mania models, i.e., they are hypersensitive
to amphetamine, the observed mania-like behaviors are
responsive to lithium treatment and the Val to Leu
substitution results in a shifted excitatory/inhibitory
synaptic balance towards more excitation. Exposure to
chronic social defeat stress switches homozygous Leu variant
carriers from a mania- to a depressive-like state, a
transition which is reminiscent of the alternations
characterizing the symptomatology in BD patients.
Single-cell RNA-seq (scRNA-seq) revealed widespread Adcy2
mRNA expression in numerous hippocampal cell types.
Differentially expressed genes particularly identified from
glutamatergic CA1 neurons point towards ADCY2
variant-dependent alterations in multiple biological
processes including cAMP-related signaling pathways. These
results validate ADCY2 as a BD risk gene, provide insights
into underlying disease mechanisms, and potentially open
novel avenues for therapeutic intervention strategies.},
keywords = {Bipolar Disorder: genetics / Bipolar Disorder: metabolism /
Adenylyl Cyclases: genetics / Adenylyl Cyclases: metabolism
/ Animals / Mice / Mutation, Missense: genetics / Male /
Humans / Mania: genetics / Polymorphism, Single Nucleotide:
genetics / Disease Models, Animal / Cyclic AMP: metabolism /
Hippocampus: metabolism / Mice, Inbred C57BL / Female /
Adenylyl Cyclases (NLM Chemicals) / adenylyl cyclase 2 (NLM
Chemicals) / Cyclic AMP (NLM Chemicals)},
cin = {AG Wurst},
ddc = {610},
cid = {I:(DE-2719)1140001},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC11649569},
pubmed = {pmid:39003412},
doi = {10.1038/s41380-024-02663-w},
url = {https://pub.dzne.de/record/274003},
}