Science Advances · 2024 · 12 citations · 33 references
Life SciencesGeneticsNeuroscienceEpigeneticsMolecular NeuroscienceSynaptic PlasticityEpigenetic Regulation In DevelopmentEpigenetic Regulation In NeuronsIntellectual Disability GeneticsMolecular NeurogeneticsSynaptic DysfunctionWnt SignalingCa3 FunctionEpigenetic MarksEpigenetic Regulation By Transcription FactorsHippocampal PlasticityHistone AcetyltransferasesHistone-lysine AcetylationSynaptic Plasticity EpigeneticsWnt Signaling In Neurons
Intellectual disability (ID) affects ~2% of the population and ID-associated genes are enriched for epigenetic factors, including those encoding the largest family of histone lysine acetyltransferases (KAT5-KAT8). Among them is KAT6A , whose mutations cause KAT6A syndrome, with ID as a common clinical feature. However, the underlying molecular mechanism remains unknown. Here, we find that KAT6A deficiency impairs synaptic structure and plasticity in hippocampal CA3, but not in CA1 region, resulting in memory deficits in mice. We further identify a CA3-enriched gene Rspo2 , encoding Wnt activator R-spondin 2, as a key transcriptional target of KAT6A. Deletion of Rspo2 in excitatory neurons impairs memory formation, and restoring RSPO2 expression in CA3 neurons rescues the deficits in Wnt signaling and learning-associated behaviors in Kat6a mutant mice. Collectively, our results demonstrate that KAT6A-RSPO2-Wnt signaling plays a critical role in regulating hippocampal CA3 synaptic plasticity and cognitive function, providing potential therapeutic targets for KAT6A syndrome and related neurodevelopmental diseases.
33
Requirement for Hippocampal CA3 NMDA Receptors in Associative Memory Recall
Kazu Nakazawa, Michael C. Quirk, Raymond A. Chitwood et al. · Science · 2002 · 1.1K citations
ZNRF3 promotes Wnt receptor turnover in an R-spondin-sensitive manner
Huai-Xiang Hao, Yang Xie, Yue Zhang et al. · Nature · 2012 · 976 citations
Recurrent R-spondin fusions in colon cancer
Somasekar Seshagiri, Eric Stawiski, Steffen Durinck et al. · Nature · 2012 · 975 citations · Full text
Genetic targeting of principal neurons in neocortex and hippocampus of NEX-Cre mice
Sandra Goebbels, Ingo Bormuth, Ulli Bode et al. · genesis · 2006 · 615 citations