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Nephropathic cystinosis is a severe monogenic kidney disorder caused by mutations in CTNS, encoding the lysosomal transporter cystinosin, resulting in lysosomal cystine accumulation. The sole treatment, cysteamine, slows down the disease progression, but does not correct the established renal proxim...
2021-06-24 | MTBLS2538 | MetaboLights
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...
ORGANISM(S): Burkholderia cenocepacia 
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 cult...
ORGANISM(S): Pseudomonas aeruginosa UCBPP-PA14 
Cigarette smoking is the major cause of chronic inflammatory diseases such as Chronic Obstructive Pulmonary Disease (COPD). It is paramount to develop pharmacological interventions and delivery strategies against the cigarette smoke (CS) associated oxidative stress in COPD. This study in Wistar rats...
ORGANISM(S): Rattus Norvegicus 
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 pers...
ORGANISM(S): Pseudomonas aeruginosa PAO1 
2021-08-19 | PXD027156 | Pride
Fibroblast cell lines form cystinotic and non-cystinotic fibroblast were incubated for 48h with increasing amounts of cysteamine, the only available treatment for cystinosis, an incurable metabolic rare disease that is characterized by accumulation of cystine crystals in the cells and accompanied by...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2024-06-13 | MSV000095020 | MassIVE
Cigarette smoking is the major cause of chronic inflammatory diseases such as Chronic Obstructive Pulmonary Disease (COPD). It is paramount to develop pharmacological interventions and delivery strategies against the cigarette smoke (CS) associated oxidative stress in COPD. This study in Wistar rats...
ORGANISM(S): Rattus norvegicus (Rat) 
2023-08-21 | PXD033529 | Pride
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 ...
ORGANISM(S): Homo sapiens 
2019-03-01 | GSE119699 | GEO
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 ...
ORGANISM(S): Caenorhabditis elegans 
2019-03-01 | GSE119696 | GEO
Burkholderia cenocepacia J2315 exposed to the aminothiol cysteamine during growth in synthetic cystic fibrosis sputum media (SCFM2)
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