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Links from GEO DataSets

Items: 20

1.

Comparison of translational profiles in Motor Neurons (CHAT), to all neurons (Snap25) in the spinal cord.

(Submitter supplied) Translating ribosome affinity purification (TRAP) was performed on spinal cord dissections pooled from 3-4 mice 21 days post birth that were positive for the eGFP-L10A fusion ribosomal marker protein under the expression of either the Chat promoter (Tg(Chat-EGFP/Rpl10a)DW167Htz) or the Snap25 promoter (Tg(Snap25-EGFP/Rpl10a)JD362Jdd). RNA-sequencing was performed on both TRAP and pre-immunoprecipitation (PreIP) control RNA samples.
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL17021
12 Samples
Download data: CSV
Series
Accession:
GSE93412
ID:
200093412
2.

Micro-RNAs secreted through astrocyte-derived extracellular vesicles cause neuronal network degeneration in C9orf72 ALS

(Submitter supplied) Background: Astrocytes regulate neuronal function, synaptic formation and maintenance partly through secreted extracellular vesicles (EVs). In amyotrophic lateral sclerosis (ALS) astrocytes display a toxic phenotype that contributes to motor neuron (MN) degeneration. Methods: We used human induced astrocytes (iAstrocytes) from 3 ALS patients carrying C9orf72 mutations and 3 non-affected donors to investigate the role of astrocyte-derived EVs (ADEVs) in ALS astrocyte toxicity. more...
Organism:
synthetic construct; Homo sapiens
Type:
Non-coding RNA profiling by array
Platform:
GPL21572
6 Samples
Download data: CEL
Series
Accession:
GSE122640
ID:
200122640
3.

Downstream Effects of Mutations in SOD1 and TARDBP Converge on Gene Expression Impairment in Patient-Derived Motor Neurons

(Submitter supplied) We established iPSCs from healthy donors, SOD1-ALS and TDP43-ALS patients. Using our differentiation protocol originally developed by Reinhardt et al.,2013, we diferentiated these iPSCs toward spinal motor neurons (MNs) and reproduce ALS pathology in a dish. To extend our understanding of finding different molecular mechanisms and pathways related to SOD1- and TDP43 mutations in ALS disease, we have performed a comprehensive gene expression profiling study using RNA-Seq of the iPSC-derived MN models from control individuals and carefully compared with those from SOD1-ALS and TDP43-ALS patients. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing; Non-coding RNA profiling by high throughput sequencing
Platform:
GPL16791
16 Samples
Download data: XLSX
Series
Accession:
GSE210969
ID:
200210969
4.

A regulatory circuitry between the Gria2 mRNA and miR-409/miR-495 is altered in mESC-derived motor neurons carrying an ALS-associated FUS mutation

(Submitter supplied) Mutations in FUS/TLS have been genetically associated to Amyotrophic Lateral Sclerosis (ALS). Since FUS is a multifunctional protein involved in the biogenesis and activity of several types of RNAs, the understanding of the molecular basis of ALS pathogenesis should take into account both direct effects of FUS mutation through gain- and loss-of function mechanisms as well as indirect effects due to the crosstalk between different classes of RNAs. more...
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing; Non-coding RNA profiling by high throughput sequencing
Platform:
GPL17021
15 Samples
Download data: TSV
Series
Accession:
GSE101097
ID:
200101097
5.

Whole genome transcriptome analysis identifies indices of fast and slow disease progression in two ALS mouse models

(Submitter supplied) Microarray analysis has been applied to the study of ALS in order to investigate gene expression in whole spinal cord homogenates of SOD1 G93A mice and human ALS cases, although the massive presence of glial cells and inflammatory factors has made it difficult to define which gene expression changes were motor neuron specific. Recently, laser capture microdissection (LCM), combined with microarray analysis, has allowed the identification of motor neuron specific changes in gene expression in mouse and human ALS cases. more...
Organism:
Mus musculus
Type:
Expression profiling by array
Platform:
GPL1261
64 Samples
Download data: CEL
Series
Accession:
GSE46298
ID:
200046298
6.

TRAP-seq of spinal cord motor neurons from ALS diseased mice versus healthy mice

(Submitter supplied) Amyotrophic lateral sclerosis (ALS) is an incurable neurological disease featuring progressive loss of motor neuron (MN) function in the brain and spinal cord. Mutations in TARDBP, encoding the RNA-binding protein TDP-43, are one cause of ALS and TDP-43 mislocalization in MNs is a key pathological feature of >95% of ALS cases. While numerous studies support altered RNA regulation by TDP-43 as a major cause of disease, specific changes within MNs that trigger disease onset remain unclear. more...
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL21103
56 Samples
Download data: TXT
Series
Accession:
GSE144640
ID:
200144640
7.

FUS H517Q and healthy iPSC-derived motor neurons

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing; Non-coding RNA profiling by high throughput sequencing
Platforms:
GPL20301 GPL15520
18 Samples
Download data: TXT
Series
Accession:
GSE203173
ID:
200203173
8.

Identifying miR-139 targets in motor neurons

(Submitter supplied) 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.
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL15520
6 Samples
Download data: TXT
Series
Accession:
GSE203172
ID:
200203172
9.

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

(Submitter supplied) 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.
Organism:
Homo sapiens
Type:
Non-coding RNA profiling by high throughput sequencing
Platform:
GPL15520
8 Samples
Download data: TXT
Series
Accession:
GSE203170
ID:
200203170
10.

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

(Submitter supplied) 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.
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL20301
4 Samples
Download data: TXT
Series
Accession:
GSE203168
ID:
200203168
11.

Progressive motor neuron pathology and the role of astrocytes in a human stem cell model of VCP-related ALS [terminally differentiated iPSC-derived astrocytes]

(Submitter supplied) Motor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interaction and the sequence of molecular events leading to MN death remain unresolved. Herewe optimized directed differentiation of induced pluripotent stem cells (iPSCs) into highly enriched (>85%) functional populations of spinal cord MNs and ACs. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL16791
4 Samples
Download data: TXT
12.

Post-transcriptional remodelling is temporally deregulated during motor neurogenesis in human ALS models

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL16791
35 Samples
Download data: TXT
Series
Accession:
GSE98290
ID:
200098290
13.

Post-transcriptional remodelling is temporally deregulated during motor neurogenesis in human ALS models

(Submitter supplied) Mutations causing amyotrophic lateral sclerosis (ALS) strongly implicate regulators of RNA-processing that are ubiquitously expressed throughout development. To understand the molecular impact of ALS-causing mutations on early neuronal development and disease, we performed transcriptomic analysis of differentiated human control and VCP-mutant induced pluripotent stem cells (iPSCs) during motor neurogenesis. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL16791
11 Samples
Download data: TXT
14.

Progressive motor neuron pathology and the role of astrocytes in a human stem cell model of VCP-related ALS

(Submitter supplied) Motor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interaction and the sequence of molecular events leading to MN death remain unresolved. Herewe optimized directed differentiation of induced pluripotent stem cells (iPSCs) into highly enriched (>85%) functional populations of spinal cord MNs and ACs. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL16791
20 Samples
Download data: TXT
15.

Human iPSC-derived astrocytes from ALS patients with mutated C9ORF72 show increased oxidative stress and neurotoxicity

(Submitter supplied) Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons (MNs). It was shown that human astrocytes with mutations in genes associated with ALS, like C9orf72 (C9) or SOD1, reduce survival of MNs. Astrocyte toxicity may be related to their dysfunction or the release of neurotoxic factors. We used human induced pluripotent stem cell-derived astrocytes from ALS patients carrying C9orf72 mutations and non-affected donors. more...
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL11154
4 Samples
Download data: XLSX
Series
Accession:
GSE142730
ID:
200142730
16.

The involvement of motor neuron specific miR-218 in human ALS

(Submitter supplied) Motor-neuron specific microRNA-218 (miR-218) was recently put in the spotlight because of its striking roles in mouse development. However, miR-218 relevance to human motor neuron disease was not yet explored. Here, we demonstrate by neuropathology that miR-218 is abundant in healthy human motor neurons. However, in amyotrophic lateral sclerosis (ALS) motor neurons miR-218 is downregulated and its mRNA targets are reciprocally upregulated (de-repressed). more...
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL17021
7 Samples
Download data: XLSX
Series
Accession:
GSE136409
ID:
200136409
17.

Expression data in muscle cells (myotubes) of motor neuron disorders (ALS, SBMA, SMA-IV) and healthy controls

(Submitter supplied) Despite the discovery of many genetic risk factors, the cause of the motor neuron death that drives terminal pathology in Amyotrophic Lateral Sclerosis (ALS) remains unknown. We report that the skeletal muscle of ALS patients secretes exosomal vesicles that are specifically toxic to motor neurons. This could not be attributed to a trivial down-stream consequence of muscle denervation. In a study of muscle biopsies and biopsy-derived denervation-naïve differentiated muscle stem cells (myotubes) from 67 human subjects, including healthy and disease controls, ALS myotubes had a consistent signature of disrupted exosome biogenesis and RNA-processing, and their exosomes induced shortened, less branched, neurites, greater death, and disrupted localization of RNA and RNA-processing proteins in motor neurons. more...
Organism:
Homo sapiens
Type:
Expression profiling by array
Platform:
GPL5175
12 Samples
Download data: CEL
Series
Accession:
GSE122261
ID:
200122261
18.

scRNAseq of human iPSC-derived motor neurons from control and ALS patients.

(Submitter supplied) We compare single cell transcriptomic profiles of motor neurons differentiated in vitro from human induced pluripotent stem cells derived from control and ALS patients.
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL18573
25 Samples
Download data: CSV
Series
Accession:
GSE138121
ID:
200138121
19.

Gene expression changes in cervical motor neuron transcriptomes after loss of Hox5 transcription factors

(Submitter supplied) The goal of this study was to identify potential Hox5 target genes in cervical motor neurons (C3-C6) using unbiased genomic screens (RNA-seq). We examined expression changes independently of motor neuron cell death by deleting the pro-apoptotic gene Bax in both control and experimental mice.
Organism:
Mus musculus
Type:
Expression profiling by high throughput sequencing
Platform:
GPL17021
6 Samples
Download data: XLSX
Series
Accession:
GSE138085
ID:
200138085
20.

Differential expression of small RNAs in muscle tissue of patients with Amyotrophic lateral sclerosis and healthy controls

(Submitter supplied) We report the differential expression of small RNAs in muscle tissue of patients with Amyotrophic lateral sclerosis and healthy controls
Organism:
Homo sapiens
Type:
Expression profiling by high throughput sequencing
Platform:
GPL18573
22 Samples
Download data: XLSX
Series
Accession:
GSE100188
ID:
200100188
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