Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Gene expression data from iPSC-derived astrocytes, comparison

ABSTRACT: Alexander disease (AxD) is a fatal neurological illness characterized by white-matter degeneration and formation of Rosenthal fibers, which contain glial fibrillary acidic protein (GFAP) as astrocytic inclusion. AxD is mainly caused by a gene mutation encoding GFAP, although the underlying pathomechanism remains unclear.

ORGANISM(S): Homo sapiens

PROVIDER: GSE83374 | GEO | 2016/09/30

SECONDARY ACCESSION(S): PRJNA325729

REPOSITORIES: GEO

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
		Other

Items per page:

1 - 1 of 1

Similar Datasets

Hippocampal gene expression in the GFAP-R237H rat model of Alexander disease at early and late stages of disease

Project description:Alexander disease is a rare neurodegenerative disorder caused by mutations in the gene for glial fibrillary acidic protein (GFAP), the major intermediate filament of astrocytes in the central nervous system. GFAP mutation causes a toxic gain-of-function and protein aggregation, ultimately leading to gliosis, astrocyte dysfunction, and neurodegeneration. To better understand the disease process, a rat model has been generated to mimic the common R239H mutation observed in the human disease (R237H in the rat). This study focuses on hippocampus, a brain region with a heavy burden of pathology, to determine the impact of GFAP mutation at presymptomatic (3 weeks of age) and severe stages ( 8 weeks of age) of disease. Transcription profiling shows progressive neuroinflammation and neurodegeneration.

2025-04-02 | GSE278645 | GEO

GFAP transgenic mice expression analysis

Project description:Olfactory bulb transcript levels for GFAP transgenic mice compared to wildtype at 3wks and 4mos Keywords = GFAP Keywords = Rosenthal fibers Keywords = Alexander disease Keywords: other

2005-07-20 | GSE977 | GEO

Characterization of A Novel Mengingoencephalomyelitis Associated with Vimentin IgG Autoantibodies

Project description:Autoantibodies are crucial for diagnosing central nervous system (CNS) autoimmune disorders. In autoimmune astrocytopathy, autoantibodies targeting astrocytes, such as those against glial fibrillary acidic protein (GFAP) or aquaporin protein 4 (AQP4), serve as indispensable diagnostic markers. Nevertheless, the diagnostic process remains challenging for patients who exhibit astrocytic reactivity on immunological tests but lack detectable specific autoantibodies

2025-01-22 | PXD056570 | Pride

Aberrant neurodevelopment in human iPS cell-derived models of Alexander disease

Project description:Alexander disease (AxD) is a rare, severe neurodegenerative disorder caused by mutations in the glial fibrillary acidic protein (GFAP). While the exact disease mechanism remains unknown, existing studies suggest that the mutant GFAP influences many cellular processes, including cytoskeleton stability, mechanosensing, cell energetics, and proteasome function. While most studies have primarily focused on GFAP-expressing astrocytes, this protein is also expressed by radial glia and neural progenitor cells, prompting questions about the impact of GFAP mutations on central nervous system (CNS) development. In this study, we present an intriguing observation of an impaired differentiation of astrocytes and neurons in co-cultures of astrocytes and neurons, as well as in brain organoids, both generated from patient-derived induced pluripotent stem (iPS) cells with a GFAP (R239C) mutation. Leveraging single-cell RNA sequencing (scRNA-seq), we identified distinct cell populations and transcriptomic changes between the GFAP mutant cells and an isogenic corrected control. These findings are supported with immunocytochemistry and proteomics. In co-cultures, the AxD mutation resulted in an increased abundance of immature cells, while in organoids, we observed an altered cell differentiation and reduced abundance of astrocytes. Additionally, gene expression analysis associated the AxD mutation with increased stress susceptibility, cytoskeletal abnormalities, and altered extracellular matrix and cell-cell communication patterns. Overall, our results suggest the possibility of a faulty differentiation in human iPS cell-derived models of AxD, opening new avenues for AxD research.

2024-09-09 | PXD048606 | Pride

gfap

Project description:Danio rerio gfap, glial fibrillary acidic protein [Source:NCBI gene;Acc:30646], is expressed in 1 baseline experiment(s);

| ENSDARG00000025301 | ExpressionAtlas

Gfap

Project description:Rattus norvegicus Gfap, glial fibrillary acidic protein [Source:RGD Symbol;Acc:2679], is expressed in 4 baseline experiment(s);

| ENSRNOG00000002919 | ExpressionAtlas

GFAP

Project description:Monodelphis domestica GFAP, glial fibrillary acidic protein [Source:NCBI gene;Acc:100020340], is expressed in 1 baseline experiment(s);

| ENSMODG00000011301 | ExpressionAtlas

Gfap

Project description:Rattus norvegicus Gfap, glial fibrillary acidic protein [Source:RGD Symbol;Acc:2679], is differentially expressed in 28 experiment(s);

| ENSRNOG00000002919 | ExpressionAtlas

Identification of genes associated with the astrocyte-specific gene Gfap during astrocyte differentiation

Project description:Chromosomes and genes are non-randomly arranged within the mammalian cell nucleus. Clustering of genes is of great significance in transcriptional regulation. However, the relevance of gene clustering in their expression during differentiation of neural precursor cells (NPCs) into astrocytes remains unclear. We performed a genome-wide enhanced circular chromosomal conformation capture (e4C) to screen genes associated with an astrocyte-specific gene, glial fibrillary acidic protein (Gfap), during astrocyte differentiation. We identified 13 genes that were specifically associated with Gfap and expressed in NPC-derived astrocytes. These results provide evidence for functional significance of gene clustering in transcriptional regulation during NPCs differentiation. comparison of NPCs vs LIF+ vs LIF- cells.

2015-02-20 | E-GEOD-66097 | biostudies-arrayexpress

GFAP

Project description:Sus scrofa GFAP, glial fibrillary acidic protein [Source:VGNC Symbol;Acc:VGNC:88419], is differentially expressed in 1 experiment(s);

| ENSSSCG00000017343 | ExpressionAtlas

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data