BACKGROUND Nephropathic cystinosis is an autosomal recessive disorder resulting in an impaired transport of cystine trough the lysosomal membrane causing an accumulation of free cystine in lysosomes. The only specific treatment for nephropathic cystinosis is cysteamine bitartrate. This study was aimed to describe the relationship between cysteamine plasma concentrations and white blood cell cys...

Children suffering from cystinosis, a genetic disease characterized by high levels of lysosomal cystine, are currently being treated with cysteamine to lower the cystine levels in their cells. In fibroblasts from these patients, cysteamine and its disulfide, cystamine, are equally effective in lowering cystine levels. We recently reported that pantethine, a dietary precursor of coenzyme A, depl...

Alkaline phosphatases (EC 3.1.3.1) purified from human placenta, neutrophils, liver, kidney, and small intestinal mucosa showed a range of Kmvalues for p-nitrophenyl phosphate from 0.017 to 0.038 rriM. The Kmvalues for cysteamine S-phosphate were more variable and ranged from 0.09 to 1.00 HIM. The ratios of the rate of hydrolysis of p-nitrophenyl phosphate to the rate of hydrolysis of cysteamin...

introduction: in this study in vitro maturation, resumption of meiosis and development of immature mouse oocytes in the presence of cysteamine was evaluated. meterial and methods: immature oocytes recovered from nmri mouse strain (4-6 weeks) were categorized in three experimental and one control group. the control included 345 immature oocytes which were matured in mem medium contain 5% fcs. in...

Age related macular degeneration (AMD) is one of the leading causes of blindness. Genetics, environmental insult, and age-related factors all play a key role in altering proteostasis, the homeostatic process regulating protein synthesis, degradation and processing. These factors also play a role in the pathogenesis of AMD and it has been well established that cigarette smoking (CS) initiates AM...

Several hypotheses have been advanced in attempts to account for the ability of certain thiol (SH)or disulfide (SS)-containing compounds to reduce the biological lesions caused by ionizing radiation (1, 2). It is clear that, in pure aqueous solutions, radiochemical transformations are for the greater part mediated by products formed by the interaction of ionizing radiation with water molecules ...

Radioprotector/Cysteamine /Mitosis/HeLa Mitotic cells disappeared nearly completely within 60 min after the addition of 30 mM cysteamine in HeLa S-3 cell culture, while the control culture contained mitotic cells with the frequency of 3.4%. Inhibition of mitosis became gradually irreversible when the treatment was extended over 60 min, but cytolysis occurred much more slowly. Ethanolamine exhib...

Cystinosis as a clinical entity is a progressive dysfunction of multiple organs caused by the accumulation of cystine in the tissues, leading, for example, to end-stage renal failure, diabetes, hypothyroidism, myopathy, and central nervous system deterioration. Brodin-Sartorius and colleagues present a long-term study on the impact of cysteamine therapy on these complications. The data show tha...

rate of in vitro maturation of oocytes is one of the challenges of assisted reproductive techniques. in this study we investigated the effects of supplementation of cysteamine on the rate of in vitro maturation of oocytes in two different media. germinal vesicle oocytes were collected from mouse ovary and cultured in two media (tcm199 and meme) with 0, 50, 100, 200, 500 µm/ml cysteamine. number...

The project has progressed according to the original research plan with the most significant findings detailed in Aim 2 demonstrating the anti-fibrotic potential of cysteamine. Current efforts are focused on the investigating the effect of cysteamine on myofibroblasts, the primary cell producing extracellular matrix during kidney fibrosis. Furthermore, in our investigation of fibrogenic mechani...