Unknown,Transcriptomics,Genomics,Proteomics

Dataset Information

RBFOX3/NeuN is required for hippocampal circuit balance and function

ABSTRACT: RBFOX3 mutations are linked to epilepsy and cognitive impairments, but the underlying pathophysiology of these disorders is poorly understood. Here we report replication of human symptoms in a mouse model with disrupted Rbfox3. Rbfox3 knockout mice displayed increased seizure susceptibility and decreased anxiety-related behaviors. Focusing on hippocampal phenotypes, we found Rbfox3 knockout mice showed increased expression of plasticity genes Egr4 and Arc, and the synaptic transmission and plasticity were defective in the mutant perforant pathway. The mutant dentate granules cells exhibited an increased frequency, but normal amplitude, of excitatory synaptic events, and this change was associated with an increase in the neurotransmitter release probability and dendritic spine density. Together, our results demonstrate anatomical and functional abnormality in Rbfox3 knockout mice, and may provide mechanistic insights for RBFOX3-related human brain disorders. Two samples (one is from wild-type and the other is from Rbfox3 homozygous knockout mice)

ORGANISM(S): Mus musculus

SUBMITTER: Hsien-Sung Huang

PROVIDER: E-GEOD-84786 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

RBFOX3/NeuN is Required for Hippocampal Circuit Balance and Function.

Wang Han-Ying HY Hsieh Pei-Fen PF Huang De-Fong DF Chin Pey-Shyuan PS Chou Chih-Hsuan CH Tung Chun-Che CC Chen Shin-Yuan SY Lee Li-Jen LJ Gau Susan Shur-Fen SS Huang Hsien-Sung HS

Scientific reports 20151201

RBFOX3 mutations are linked to epilepsy and cognitive impairments, but the underlying pathophysiology of these disorders is poorly understood. Here we report replication of human symptoms in a mouse model with disrupted Rbfox3. Rbfox3 knockout mice displayed increased seizure susceptibility and decreased anxiety-related behaviors. Focusing on hippocampal phenotypes, we found Rbfox3 knockout mice showed increased expression of plasticity genes Egr4 and Arc, and the synaptic transmission and plast ...[more]

PMID: 26619789

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Dataset Information

RBFOX3/NeuN is required for hippocampal circuit balance and function

Publications

RBFOX3/NeuN is Required for Hippocampal Circuit Balance and Function.

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