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Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P30]

ABSTRACT: Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P30]

PROVIDER: PRJNA529021 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR8785210_1.fastq.gz	Fastqsanger.gz
	SRR8785210_2.fastq.gz	Fastqsanger.gz
	SRR8785211.fastq.gz	Fastqsanger.gz
	SRR8785212.fastq.gz	Fastqsanger.gz
	SRR8785213.fastq.gz	Fastqsanger.gz

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Similar Datasets

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P30]

Project description:Williams syndrome (WS), caused by a heterozygous microdeletion in 7q11.23, is a neurodevelopmental disorder characterized by hypersociability and neurocognitive abnormalities. Of the deleted genes, general transcription factor II-i (Gtf2i) has been linked to hypersociability in WS, though the underlying mechanisms are poorly understood. We show that selective deletion of Gtf2i in forebrain excitatory neurons caused neuroanatomical defects, fine motor deficits, increased sociability and anxiety. Unexpectedly, 70% of the genes with significantly decreased mRNA levels in the mutant mouse cortex are involved in myelination, and mutant mice had reduced mature oligodendrocyte cell numbers, reduced myelin thickness and impaired axonal conductivity. Restoring myelination properties with clemastine or increasing axonal conductivity rescued the behavioural deficits. Frontal cortex from WS patients similarly showed reduced myelin thickness, mature oligodendrocyte cell numbers and mRNA levels of myelination-related genes. Our study provides molecular and cellular evidence for myelination deficits in WS linked to neuronal deletion of Gtf2i.

2019-03-26 | GSE128839 | GEO

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug

Project description:Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug

| PRJNA529019 | ENA

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [human]

Project description:Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [human]

| PRJNA529023 | ENA

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P1]

Project description:Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P1]

| PRJNA529020 | ENA

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [human]

2019-03-26 | GSE128840 | GEO

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug [mouse P1]

2019-03-26 | GSE128838 | GEO

Neuronal deletion of Gtf2i, associated with Williams syndrome, causes behavioural and myelin alterations rescuable by a remyelinating drug

Project description:This SuperSeries is composed of the SubSeries listed below.

2019-03-26 | GSE128841 | GEO

P30.Dataset

Project description:P30.Dataset

| PRJNA929186 | ENA

Murine Adult Cortex: Gtf2i (+/Δex2 ) VS WT (+/+)

Project description:Transcriptional profiling of mouse cortex tissue comparing control animals with Gtf2i-mutated mouse (Gtf2i+/Δex2 ). Goal was to determine the specific deregulated genes in the cortex of mutated animals.

2014-10-20 | GSE41251 | GEO

Expression data from GTF2i mutated ES cells

Project description:Data present the expression analysis of different mouse ES cell line with altered expression of GTF2I. We used microarrays to detail the global programme of gene expression underlying altered expression of GTF2I and identified distinct classes of deregulated genes

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