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E-GEOD-52202

ABSTRACT: Transcription profiling by high throughput sequencing of iPSC derived motor neuron cultures from C9ORF72 carriers

OTHER RELATED OMICS DATASETS IN: PRJNA227155

PROVIDER: E-GEOD-52202 | ExpressionAtlas | 2025-01-24

REPOSITORIES: ExpressionAtlas

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			Action	DRS
	E-GEOD-52202-analysis-methods.tsv	Tabular
	E-GEOD-52202-analytics.tsv	Tabular
	E-GEOD-52202-analytics.tsv.undecorated	Tabular
	E-GEOD-52202-analytics.tsv.undecorated.unrounded	Tabular
	E-GEOD-52202-analytics.tsv.unrounded	Tabular

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Project description:iPSC derived motor neuron cultures from C9ORF72 carriers

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PIKFYVE Inhibition Mitigates Disease in Models of Diverse Forms of ALS [1]

Project description:RNA sequencing analysis of human iPSC-derived motor neurons generated from one C9ORF72 ALS/FTD patient line, treated with DMSO or Apilimod. The goal of this study is to evaluate the effect of PIKFYVE inhibitor in rescuing ALS motor neuron degeneration.

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PIKFYVE Inhibition Mitigates Disease in Models of Diverse Forms of ALS [2]

Project description:RNA sequencing analysis of human iPSC-derived motor neurons generated from two C9ORF72 ALS/FTD patient lines, treated with negative control ASO (NC ASO) or PIKFYVE ASO. The goal of this study is to evaluate the effect of PIKFYVE suppression in rescuing ALS motor neuron degeneration.

2022-12-18 | GSE221016 | GEO

E-GEOD-55282

Project description:Transcription profiling by high throughput sequencing in osteoblast cultures from non-syndromic craniosynostosis bone

2025-01-24 | E-GEOD-55282 | ExpressionAtlas

Gene expression data from human fibroblasts and induced Pluripotent Stem cells from amyotrophic lateral sclerosis patients

Project description:We have generated human induced Pluripotent Stem cells (hiPSc) from amyotrophic lateral sclerosis (ALS, motor neuron disease) patients, using Sendai virus-mediated delivery of reprogramming factors. hiPSc lines have been screened using SNP array to assess chromosomal stability (alongside the fibroblast lines from which they derived), and validation of the pluripotency of the hiPSc lines is provided by Pluritest assessment of transcriptome datasets, prior to differentiation to motor neuron cultures and downstream functional assays. Mutihac R., Scaber J., Lalic T., Ababneh N., Vowles, J., Fletcher-Jones A., Douglas A.G.L., Browne C., Nakanishi M., Turner M., Wade-Martins R., Cowley S.A. and Talbot K. Altered ER calcium homeostasis and stress granule formation in iPSC-derived motor neurons from ALS/FTD patients with C9orf72 expansions. Submitted

2014-12-31 | GSE64583 | GEO

SNP data from human fibroblasts and induced Pluripotent Stem cells from amyotrophic lateral sclerosis patients

2014-12-31 | GSE64582 | GEO

E-GEOD-77720

Project description:Transcription profiling by high throughput sequencing in brain and spinal cord of C9orf72 BAC transgenic mice

2019-05-14 | E-GEOD-77720 | ExpressionAtlas

E-MTAB-8802

Project description:RNA sequencing of human iPSC-derived brain microvascular endothelial cells.

2025-01-16 | E-MTAB-8802 | ExpressionAtlas

C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9

Project description:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron loss, with additional pathophysiological involvement of non-neuronal cells such as microglia. The commonest ALS-associated genetic variant is a hexanucleotide repeat expansion (HRE) mutation in C9orf72. Here, we study its consequences for microglial function using human iPSC-derived microglia. By RNA-sequencing, we identify enrichment of pathways associated with immune cell activation and cyto-/chemokines in C9orf72 HRE mutant microglia versus healthy controls, most prominently after LPS priming. Specifically, LPS-primed C9orf72 HRE mutant microglia show consistently increased expression and release of matrix metalloproteinase-9 (MMP9). LPS-primed C9orf72 HRE mutant microglia are toxic to co-cultured healthy motor neurons, which is ameliorated by concomitant application of an MMP9 inhibitor. Finally, we identify release of dipeptidyl peptidase-4 (DPP4) as a marker for MMP9-dependent microglial dysregulation in co-culture. These results demonstrate cellular dysfunction of C9orf72 HRE mutant microglia, and a non-cell-autonomous role in driving C9orf72-ALS pathophysiology in motor neurons through MMP9 signaling.

2023-08-20 | GSE217625 | GEO

iPSC derived motor neuron cultures from C9ORF72 carriers

Project description:Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition characterized by loss of motor neurons in the brain and spinal cord. Expansions of a hexanucleotide repeat (GGGGCC) in the noncoding region of the C9ORF72 gene are the most common cause of the familial form of ALS (C9-ALS), as well as frontotemporal lobar degeneration and other neurological diseases. How the repeat expansion causes disease remains unclear, with both loss of function (haploinsufficiency) and gain of function (either toxic RNA or protein products) proposed. We report a cellular model of C9-ALS with motor neurons differentiated from induced pluripotent stem cells (iPSCs) derived from ALS patients carrying the C9ORF72 repeat expansion. No significant loss of C9ORF72 expression was observed, and knockdown of the transcript was not toxic to cultured human motor neurons. Transcription of the repeat was increased, leading to accumulation of GGGGCC repeat–containing RNA foci selectively in C9-ALS iPSC-derived motor neurons. Repeat-containing RNA foci colocalized with hnRNPA1 and Pur-?, suggesting that they may be able to alter RNA metabolism. C9-ALS motor neurons showed altered expression of genes involved in membrane excitability including DPP6, and demonstrated a diminished capacity to fire continuous spikes upon depolarization compared to control motor neurons. Antisense oligonucleotides targeting the C9ORF72 transcript suppressed RNA foci formation and reversed gene expression alterations in C9-ALS motor neurons. These data show that patient-derived motor neurons can be used to delineate pathogenic events in ALS. Transcriptome profiling from iPSC derived motor neurons compared to controls

2013-11-07 | E-GEOD-52202 | biostudies-arrayexpress

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