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Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

ABSTRACT: Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

PROVIDER: PRJNA911239 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR22684624.fastq.gz	Fastqsanger.gz
	SRR22684625.fastq.gz	Fastqsanger.gz
	SRR22684626.fastq.gz	Fastqsanger.gz
	SRR22684627.fastq.gz	Fastqsanger.gz
	SRR22684628.fastq.gz	Fastqsanger.gz

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Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

Project description:Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

| PRJNA913013 | ENA

Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

Project description:Heterotopic ossification (HO) is a non-physiological process of bone formation in which progenitor cells in soft tissues differentiate into chondrogenic cells. In the case of fibrodysplasia ossificans progressiva (FOP), a rare genetic disease characterized by progressive and systemic HO, the Activin A/mutated-ACVR1/mTORC1 cascade induces HO in progenitors, one of which is the fibro/adipogenic progenitors (FAPs) in muscle tissues. The relevant biological processes aberrantly regulated by activated mTORC1 remain unclear, however. RNA sequencing analyses revealed the enrichment of genes involved in oxidative phosphorylation (OXPHOS) during Activin A-induced chondrogenesis of mesenchymal stem cells derived from induced pluripotent stem cells (iPSCs) of FOP patients. Functional analyses showed a metabolic transition from glycolysis to OXPHOS during chondrogenesis, in conjunction with an upregulation of mitochondrial biogenesis. mTORC1 inhibition by rapamycin suppressed OXPHOS, and IACS-010759, an inhibitor of OXPHOS, inhibited cartilage matrix formation in vitro, indicating that OXPHOS is principally involved in mTORC1-induced chondrogenesis. Furthermore, IACS-010759 inhibited the muscle injury-induced enrichment of FAP genes and HO in transgenic mice carrying the mutated human ACVR1. These data indicated that OXPHOS is a critical downstream mediator of mTORC1 signaling in chondrogenesis and therefore is a potential FOP therapeutic target.

2024-02-16 | GSE221128 | GEO

Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva

2024-02-16 | GSE220725 | GEO

Homo sapiens

Project description:Immunological Imbalance in Fibrodysplasia Ossificans Progressiva Revealed by PBMC Transcriptome Analysis

| PRJNA312581 | ENA

Concurrent progress of reprogramming and gene-correction to overcome limitation of gene therapy of ALK2 mutant iPSCs

Project description:To ameliorate the cumbersome processes of reprogramming and subsequent gene-editing in vulnerable iPSCs, we have developed a greatly simplified one-step procedure, simultaneously introducing reprogramming and gene-editing components into human fibroblast cells. This not only serves to save time, labor, and costs, but opens up a new arena of research that is beyond the current application of gene-editing methodologies due to restrictive reprogramming concerns, inhibitory pluripotency maintenance requirements, and vulnerability of single-cell dissociated iPSCs. We generated iPSCs (mALK2-iPSCs) derived from Fibrodysplasia ossificans progressiva (FOP)-fibroblast cells carrying the ACVR1 p.R206H mutation and gene-corrected ALK2-iPSCs (cALK2-iPSCs). To identify gene-correction effects on global gene expression, gene expression profiling was measured in cALK2-iPSCs and mALK2-iPSCs, calibrated to WT-iPSCs with duplication.

2015-05-12 | E-GEOD-68766 | biostudies-arrayexpress

Oxidative phosphorylation promotes primary melanoma invasion

Project description:Oxidative phosphorylation promotes primary melanoma invasion

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Oxidative phosphorylation selectively orchestrates tissue macrophage homeostasis

Project description:Oxidative phosphorylation selectively orchestrates tissue macrophage homeostasis

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Project description:Gut microbiome ablation reduces heterotopic ossification in a mouse model of ACVR1R206H-driven fibrodysplasia ossificans progressiva

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Oxidative phosphorylation is a pivotal therapeutic target of fibrodysplasia ossificans progressiva.

Project description: Not available

| S-EPMC10875110 | biostudies-literature

Palovarotene action against heterotopic ossification includes the inhibition of local participating activin-A expressing cell populations

Project description:Heterotopic ossification (HO) consists of extraskeletal bone formation. One form of HO is acquired and instigated by traumas or surgery, and another form is genetic and characterizes Fibrodysplasia Ossificans Progressiva (FOP). We and others showed that activin A promotes both acquired and genetic HO and found that the retinoid agonist Palovarotene inhibits both HO forms in mice. We asked whether Palovarotene's action against HO may include an interference with endogenous activin A expression and/or function. Using a mouse model of acquired HO, we found that activin A and its encoding RNA (Inhba) were prominent in chondrogenic cells within developing HO masses in untreated mice. Single cell RNAseq (scRNAseq) assays verified that Inhba expression characterized chondroprogenitors and chondrocytes in untreated HO, in addition to its expected expression in inflammatory cells and macrophages. Palovarotene administration caused a sharp inhibition of both HO and amounts of activin A and Inhba transcripts. Bioinformatic analyses of scRNAseq datasets indicated that the drug had reduced interactions and crosstalk amongst cell populations. Our data reveal that Palovarotene markedly reduces the number of local Inhba-expressing HO-forming populations.

2023-07-01 | GSE233843 | GEO

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