Transcriptome analysis of hIPSCs differentiation towards the skeletal muscle and motor neurons lineages
Ontology highlight
ABSTRACT:
ORGANISM(S): Homo sapiens
PROVIDER: GSE142042 | GEO | 2020/12/20
REPOSITORIES: GEO
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Project description:Transcriptome analysis of hIPSCs differentiation towards the skeletal muscle and motor neurons lineages
| PRJNA595642 | ENA
Project description:Gene expression changes in spinal motor neurons of the SOD1G93A-transgenic model for ALS after treatment with G-CSF. To gain insight into the mode of action of G-CSF, we performed gene expression profiling on isolated lumbar motor neurons from SOD1G93A mice, the most frequently studied animal model for ALS, with and without G-CSF treatment. A first group of SOD1G93A and WT mice was included in the study at week 11 of age when SOD1G93A mice present no signs of motor dysfunction but subtle signs of denervation detectable by electromyography. The second cohort of mice was treated with G-CSF or vehicle from week 11 to week 15. At the time of study completion, SOD1G93A mice presented clear motor impairment and motor neuron degeneration is documented. This design should provide information on genes altered in motor neurons of SOD1G93A mice from the clinically non-symptomatic to an early symptomatic stage, and give insight into genes influenced by G-CSF treatment. We sampled 300 motoneurons per mouse spinal cord by laser microdissection.
2014-09-15 | E-GEOD-60856 | biostudies-arrayexpress
Project description:Spinal muscular atrophy (SMA) is a neuromuscular disease caused by mutations in the survival of motor neuron (SMN) gene. Although the primary manifestation of SMA is degeneration of motor neurons in a dying-back manner, deficient SMN intrinsic to motor neurons does not cause severe motor neuron loss, as is the case in SMA mouse models. Thus, the involvement of non-neuronal cells in the pathogenesis of SMA has been suggested. Here, we report that a novel subset of fibro-adipogenic progenitors expressing Dpp4 (Dpp4+ FAPs) is required for the survival of motor neurons, in which BRCA1-associating protein 1 (Bap1) is crucial for the stabilization of SMN by preventing its ubiquitination-dependent degradation. Inactivation of Bap1 in FAPs decreased SMN levels and accompanied degeneration of the neuromuscular junction, leading to dying-back loss of motor neurons and muscle atrophy, reminiscent of SMA pathogenesis. Overexpression of ubiquitination-resistant SMN variant, SMNK186R, in Bap1-null FAPs completely prevented neuromuscular degenerations. In addition, transplantation of Dpp4+ FAPs, but not Dpp4- FAPs, completely rescued neuromuscular defects in the mutant mice. Our data revealed that Bap1-mediated stabilization of SMN in Dpp4+ FAPs is crucial for the survival of motor neurons and provided a new therapeutic approach to treat SMA.
2022-06-30 | GSE190426 | GEO
Project description:Gene expression from human iPSC derived motor neurons.
| EGAS00001003785 | EGA
Project description:The expression of v5-tagged Hoxc9 is induced and ChIP-seq is used to profile genome-wide occupancy in differentiating motor neurons The differentiation of ventral motor neurons is induced by treating embryonic stem cell cultures with retinoic acid and hedgehog signaling. Here, ChIP-seq is used to profile the genome-wide occupancy of Hoxc9 after five days of differentiation.
2010-09-08 | E-GEOD-21812 | biostudies-arrayexpress
Project description:Progenitor motor neurons can be generated with high-efficiency by differentiating ES cells in vitro in the presence of retinoic acid and hedgehog signalling. Here, we characterize the chromatin landscape associated with progenitor motor neurons (pMNs) in order to assess how histone modification domains shift during the differentiation process. In this study, we characterize the genomic occupancy of H3K27me3, H3K4me3, H3K79me2 and Pol2 using ChIP-seq in progenitor motor neurons that have been differentiated in vitro from ES cells. An appropriate whole-cell extract control experiment for these ChIP-seq experiments is also included.
2013-07-04 | E-GEOD-39434 | biostudies-arrayexpress
Project description:Generic spinal motor neuron identity is specified by cooperative binding of selector transcription factors to motor neuron specific enhancers. Whether these enhancers remain active to maintain the motor neuron expression program following downregulation of selector factors in maturing motor neurons remains unknown. We demonstrate that enhancers established by selector genes are highly transient during motor neuron differentiation. The chromatin immunoprecipitation-exonuclease (ChIP-exo) assay reveals that Isl1 is anchored to nascent motor neuron enhancers through protein-protein interaction. Stage-specific recruitment of transcription factors correlates with active enhancer marks. The majority of genes contain distinct early and late enhancers suggesting that the motor neuron expression program is maintained by a dynamic regulatory landscape.
2016-12-05 | GSE79561 | GEO
Project description:Gene expression profiling has been performed previously on motor cortex and spinal cord homogenates and of sporadic ALS cases and controls, to identify genes and pathways differentially expressed in ALS. More recent studies have combined the use of laser capture microdissection (LCM) with gene expression profiling to isolate the motor neurons from the surrounding cells, such as microglia and astrocytes, in order to determine those genes differentially expressed in the vulnerable cell population – i.e. motor neuron. The aim of the present study is to combine LCM and microarray analysis to determine those genes and pathways differentially expressed in MNs from human SOD1-related MND and to establish potential pathways for therapeutic intervention. Keywords: Human motor neurons The aim of this study was to determine the gene expression profiles from a small subset of cases which all carry mutations in the SOD1 gene. Expression profiles from isolated motor neurons in SOD1-related ALS cases were compared to those from control motor neurons, in order to establish the pathways implicated in SOD1-related motor neuronal cell death. The 'control' samples were originally submitted to GEO as GSE19332.
2011-01-01 | E-GEOD-20589 | biostudies-arrayexpress
Project description:Differentiated motor neurons from hiPSC derived from peripheral nerve fibroblasts of sporadic ALS patients and evaluated the gene expression profile by means microarray-linked to specific analysis tools. Two-condition experiment, ALS patients motor neurons vs. controls. Biological replicates: 3 ALS replicates, 3 control replicates.
2015-04-25 | E-GEOD-68240 | biostudies-arrayexpress
Project description:Recently, three-dimensional small intestinal organoids (enteroids) have been developed from cultures of intestinal stem cells which differentiate in vitro to generate all the differentiated epithelial cell types associated with the intestine and mimic the structural properties of the intestine observed in vivo. Small-molecule drug treatment can skew organoid epithelial cell differentiation towards particular lineages, and these skewed enteroids may provide useful tools to study specific epithelial cell populations, such as goblet and Paneth cells. However, the use and characterisation of enteroid models has not yet been fully explored, such that the extent to which differentiated epithelial cell populations in these skewed enteroids represent their in vivo counterparts is not fully understood. In this study, we have performed label-free quantitative proteomics to determine whether skewing murine enteroid cultures towards the goblet or Paneth cell lineages results in changes in abundance of proteins associated with these cell lineages in vivo. Our data confirm that skewed enteroids recapitulate important features of the in vivo gut environment, confirming that they can serve as useful models for the investigation of normal and disease processes in the intestine. Furthermore, by comparison of our mass spectrometry data with histology data contained within the Human Protein Atlas, we identify putative novel markers for goblet and Paneth cells.
2019-01-11 | PXD005006 | Pride