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Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

ABSTRACT: Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

PROVIDER: PRJNA490135 | ENA |

REPOSITORIES: ENA

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Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (human)

Project description:Pre-clinical studies of cysteamine bitartrate demonstrate its narrow therapeutic window, where toxicity at millimolar levels correlates with a marked induction of hydrogen peroxide production. At micromolar range concentrations, cysteamine bitartrate yielded a survival benefit in RC disease subject fibroblasts, and protection from brain death in RC complex IV deficient zebrafish. Mitochondrial oxidative stress and membrane potential were significantly improved by micromolar cysteamine bitartrate in RC complex I disease worms, translating to a modest improvement of animal development and fecundity but not lifespan. Overall, these data suggest that cysteamine bitartrate may hold therapeutic potential but requires careful consideration of safe and effective dose to further evaluate in clinical trials of human subjects with primary mitochondrial disease.

2019-03-01 | GSE119699 | GEO

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (worm)

2019-03-01 | GSE119696 | GEO

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (worm)

Project description:Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (worm)

| PRJNA490034 | ENA

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (human)

Project description:Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease (human)

| PRJNA490137 | ENA

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

Project description:This SuperSeries is composed of the SubSeries listed below.

2019-03-01 | GSE119723 | GEO

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease.

Project description: Not available

| S-EPMC6522065 | biostudies-literature

Burkholderia cenocepacia J2315 exposed to the aminothiol cysteamine during growth in synthetic cystic fibrosis sputum media (SCFM2)

Project description:Derived from coenzyme A degradation in mammalian cells, the aminothiol cysteamine may represent a promising new adjunct treatment for pulmonary exacerbations in cystic fibrosis (CF). This experiment aims to characterise the effect of sub-MIC cysteamine on Burkholderia cenocepacia J2315. Samples were cultured in SCFM2 (triplicate), and exposed to either cysteamine or a H2O control.

2021-11-01 | E-MTAB-10327 | biostudies-arrayexpress

Pseudomonas aeruginosa PA14 exposed to the aminothiol cysteamine during growth in synthetic cystic fibrosis sputum media (SCFM2)

Project description:Derived from coenzyme A degradation in mammalian cells, the aminothiol cysteamine may represent a promising new adjunct treatment for pulmonary exacerbations in cystic fibrosis (CF). This experiment aims to characterise the effect of sub-MIC cysteamine on P. aeruginosa strain PA14. Samples were cultured in SCFM2 (triplicate), and exposed to either cysteamine or a H2O control.

2021-11-01 | E-MTAB-10326 | biostudies-arrayexpress

Cysteamine inhibits glycine utilisation and disrupts virulence in Pseudomonas aeruginosa

Project description:Pseudomonas aeruginosa is a major opportunistic human pathogen which employs a myriad of virulence factors. In people with cystic fibrosis (CF) P. aeruginosa frequently colonises the lungs and becomes a chronic infection that evolves to become less virulent over time, but often adapts to favour persistence in the host with alginate-producing mucoid, slow-growing, and antibiotic resistant phenotypes emerging. Cysteamine is an endogenous aminothiol which has been shown to prevent biofilm formation, reduce phenazine production, and potentiate antibiotic activity against P. aeruginosa, and has been investigated in clinical trials as an adjunct therapy for pulmonary exacerbations of CF. Here we demonstrate (for the first time in a prokaryote) that cysteamine prevents glycine utilisation by P. aeruginosa in common with reported activity blocking the glycine cleavage system in human cells. Despite the inhibition of glycine metabolism, cysteamine inhibits hydrogen cyanide (HCN) production by P. aeruginosa, implicating interference in the regulation of virulence. Cysteamine impaired chemotaxis and reductions in pyocyanin and exopolysaccharide production in cysteamine-treated P. aeruginosa and reduced toxicity of secreted factors in a Galleria mellonella model. Thus, cysteamine has additional potent anti-virulence properties targeting P. aeruginosa, further supporting its therapeutic potential in CF and other infections.

2021-08-19 | PXD027156 | Pride

Pre-clinical evaluation of the hypomethylating agent decitabine for the treatment of T-cell acute lymphoblastic lymphoma

Project description:T-cell acute lymphoblastic lymphoma (T-LBL) is a rare and aggressive blood cancer, most often diagnosed in teenagers and young adults. Patients are usually treated with intensive chemotherapy which causes many side effects and relapse still occurs. Therefore, the search toward less toxic anti-leukemic therapies is ongoing. In line with this, we here focused on targeting a recently described perturbed DNA methylation profile in T-LBL. This aberrantly methylated DNA can serve as a new therapeutic target for DNA hypomethylating epidrugs like decitabine. We evaluated the anti-leukemic properties and downstream effects of decitabine using T-LBL patient derived xenograft (PDX) models. Interestingly, decitabine treatment resulted in prolonged leukemia-free survival in all T-LBL PDX models, which was mediated by i.a. downregulation of the oncogenic MYC pathway. However, some PDX models had more benefit of being treated with decitabine compared to others. Remarkably, differentially methylated CpG islands regions resulted in significantly more differentially expressed genes in open chromatin in the more sensitive group. This resulted in i.a. stronger downregulation of cell cycle genes and upregulation of immune response activating genes. Furthermore, we suggested a gene signature for high decitabine sensitivity. In conclusion, we delivered pre-clinical data of the potential of decitabine to act as a new therapeutic agent in T-LBL.

2023-03-02 | GSE218060 | GEO

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