Cystinosis is an autosomal recessive metabolic disease in which the amino acid cystine accumulates in lysosomes due to a defect in lysosomal cystine transport. Cystinosis in infancy is associated with poor growth, muscle wastage, and death at about age 10 due to kidney failure. Treatment with cysteamine and kidney transplantation enables cystinotic girls to reach reproductive age and to be heal...

Ghrelin, produced and secreted by the A-like cells of the stomach, stimulates growth hormone secretion, gastric motility, and food intake. Cysteamine inhibits the release of somatostatin and induces the formation of duodenal ulcers in rats. The present study was conducted to investigate the dynamics of ghrelin secretion in rats treated with cysteamine. Male Wistar rats (7 wk old) were administe...

The long-term objective of this grant is to identify the membrane transporters underlying the cystine-depleting effect of cysteamine as this would provide a rationale to improve its efficiency or discover more efficient, better tolerated drugs. According to the current model of cysteamine therapy, cystine depletion is mediated by an unknown cationic amino acid transporter, which exports a mixed...

Cysteamine has been reported to modulate energy homeostasis and exert significant growth-promoting effects in broiler chickens. However, little is known concerning its effects on egg production of hens and the growth rate of their offspring. In the present study, 67-wk-old broiler breeders were allotted at random to control and cysteamine-supplemented (400 mg/kg) groups for 8 wk. The hatchlings...

background: preparation of oocytes is one of the critical factors that determine the developmental competence of embryos produced by in vitro fertilization (ivf). objective: in this study, the effect of cysteamine, type of media and glutathione (gsh) level on blastocysts development after in vitro maturation of mouse oocytes were investigated. materials and methods: premature female mice were p...

Recent evidence implies that impaired metabolism of glutathione has a role in the pathogenesis of nephropathic cystinosis. This recessive inherited disorder is characterized by lysosomal cystine accumulation and results in renal Fanconi syndrome progressing to end stage renal disease in the majority of patients. The most common treatment involves intracellular cystine depletion by cysteamine, d...

The aims of the present study were to assess the effects of cysteamine as an anti-oxidant on the rate of in vitro maturation (IVM) of buffalo oocytes (experiment 1), and their viability and nuclear status following vitrification (experiment 2). Immature oocytes with compact cumulus cells obtained from the ovaries of slaughtered animals were harvested and then cultured in the maturation medium w...

Cystinosis is an autosomal recessive inherited lysosomal storage disease. It is characterized by generalized proximal tubular dysfunction known as renal Fanconi syndrome and causes end-stage renal disease by the age of about 10 years if left untreated. Extrarenal organs are also affected, including the thyroid gland, gonads, pancreas, liver, muscle, and brain. Treatment consists of administrati...

Although not initially recognized when there were few long-term survivors with cystinosis, the use of renal transplantation in the 1960s permitted observation of a number of additional clinical disabilities, as patients with nephropathic cystinosis survived past young childhood for the first time. Among these, serious distal myopathy has emerged as a significant problem of early adulthood in un...

AIMS Cysteamine is used to reduce tissue cystine content in patients suffering from nephropathic cystinosis. The objectives of the current study were to investigate pharmacokinetics and pharmacodynamics of cysteamine bitartrate in children and young adults with nephropathic cystinosis. METHODS Cysteamine bitartrate was administered to 11 cystinosis patients at their regular dose level in a si...