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Bulk RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

ABSTRACT: Bulk RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

PROVIDER: PRJNA838953 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR19241828.fastq.gz	Fastqsanger.gz
	SRR19241829.fastq.gz	Fastqsanger.gz
	SRR19241830.fastq.gz	Fastqsanger.gz
	SRR19241831.fastq.gz	Fastqsanger.gz
	SRR19241832.fastq.gz	Fastqsanger.gz

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Bulk RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

Project description:We sought to investigate changes in gene expression levels including changes in pri-microRNAs in FUS H517Q motor neurons Motor neurons were differentiated from patient-derived FUS H517Q and healthy iPSCs, and harvested at day 30. We performed gene expression profiling analysis using data obtained from bulk RNA-seq.

2022-05-20 | GSE203168 | GEO

Small RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

Project description:We sought to investigate changes in expression levels of small RNAs including microRNAs in FUS H517Q and healthy motor neurons Motor neurons were differentiated from patient-derived FUS H517Q and healthy iPSCs, and harvested at day 30. We performed gene expression profiling analysis using data obtained from bulk small RNA-seq.

2022-05-20 | GSE203170 | GEO

FUS H517Q and healthy iPSC-derived motor neurons

Project description:FUS H517Q and healthy iPSC-derived motor neurons

| PRJNA838945 | ENA

HTA2.0 (human transcriptome array) analysis of control iPSC-derived motor neurons (MN), FUS-H517D-hetero-iPSC-MN, and FUS-H517D-homo-iPSC-MNs

Project description:To assess RNA regulation in the MN possessing mutated FUS-H517D gene. Fused in sarcoma/translated in liposarcoma (FUS) is a causative gene of familial amyotrophic lateral sclerosis (fALS). Mutated FUS causes accumulation of DNA damage stress and stress granule (SG) formation, etc., thereby motor neuron (MN) death. However, key molecular etiology of mutated FUS-dependent fALS (fALS-FUS) remains unclear. Here, Bayesian gene regulatory networks (GRN) calculated by Super-Computer with transcriptome data sets of induced pluripotent stem cell (iPSC)-derived MNs possessing mutated FUSH517D (FUSH517D MNs) and FUSWT identified TIMELESS, PRKDC and miR-125b-5p as "hub genes" which influence fALS-FUS GRNs. miR-125b-5p expression up-regulated in FUSH517D MNs, showed opposite correlations against FUS and TIMELESS mRNA levels as well as reported targets of miR-125b-5p. In addition, ectopic introduction of miR-125b-5p could suppress mRNA expression levels of FUS and TIMELESS in the cells. Furthermore, we found TIMELESS and PRKDC among key players of DNA damage stress response (DDR) were down-regulated in FUSH517D MNs and cellular model analysis validated DDR under impaired DNA-PK activity promoted cytosolic FUS mis-localization to SGs. Our GRNs based on iPSC models would reflect fALS-FUS molecular etiology.

2021-04-19 | GSE118336 | GEO

Small RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

Project description:Small RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

| PRJNA838952 | ENA

Identifying miR-139 targets in motor neurons

Project description:We sought to investigate changes in expression levels of RNAs in response to miR-139 overexpression in human iPSC-derived motor neurons Motor neurons were differentiated from FUS H517Q iPSCs and transduced with AAVs expressing miR-139 or controls hairpins. RNA was harvested at day 34. We performed gene expression profiling analysis using data obtained from bulk RNA-seq.

2022-05-20 | GSE203172 | GEO

HTA2.0 (human transcriptome array) analysis of control iPSC-derived motor neurons (MN), FUS-H517D-hetero-iPSC-MN, and FUS-H517D-homo-iPSC-MNs

Project description:HTA2.0 (human transcriptome array) analysis of control iPSC-derived motor neurons (MN), FUS-H517D-hetero-iPSC-MN, and FUS-H517D-homo-iPSC-MNs

| PRJNA485257 | ENA

Expression profiles of primary motor neurons after knocking down Fus

Project description:To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary motor neurons by using exon-sensitive microarray technology. To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary glial cells by using exon-sensitive microarray technology. To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary cerebellar neurons by using exon-sensitive microarray technology.

2012-11-21 | GSE42421 | GEO

Changes in FUS interactome in motor neurons upon ALS-related mutation

Project description:FUS Immunoprecipitation in human induced pluripotent stem cells and human motor neurons

2024-03-04 | PXD047271 | Pride

Activation of polo-like kinase 1 expression in spinal motor neurons as vulnerability factor of spinal motor neurons in FUS- and other fALS

Project description:FUS ALS seems to preferentially affect sMNs, and cognitive dysfunction in FUS ALS is rare. Considering this, we wanted to analyze if cortical neurons behave differently than spinal motor neurons in response to FUS mutations. For this, we used cortical neurons derived from isogenic human induced pluripotent stem cells (hiPSCs) in which either WT or NLS mutant FUS P525L was tagged with eGFP using CRISPR/Cas9 and systematically compared them to sMNs of the identical iPSCs. Phenotypically, mutant FUS cortical neurons showed less impairment of FUS recruitment to DNA damage sites compared to mutant sMNs and less signs of DNA damage, which were similarly found in post mortem tissue. To advance our understanding of finding different molecular mechanisms and pathways related to FUS mutations in ALS disease, we have performed RNA sequencing of FUS ALS cortical and spinal motor neurons and our results revealed basic differences in their transcriptomes. Alternative splicing events in spinal motor neurons were different from cortical neurons also pointing towards DNA damage in FUS ALS.

2024-08-27 | GSE272827 | GEO

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