Cystinosis is a severe inherited metabolic storage disease caused by the lysosomal accumulation of cystine. Lifelong therapy with drug cysteamine bitartrate necessary. Cysteamine cleaves intralysosomal cystine, and thereafter, it can exit from organelle. The need for frequent dosing every 6 h high prevalence gastrointestinal side effects lead to poor adherence. purpose our study was improve tre...

OBJECTIVE Cystinosis causes renal and other organ failure. Regular 6-hourly cysteamine bitartrate (Cystagon; Mylan, Morgantown, West Virginia) reduces intracellular cystine and the rate of organ deterioration. A formulation of cysteamine requiring less frequent dosing may improve compliance and possibly patient outcome. METHODS Enteric-release cysteamine was prepared. For a period of 1 month,...

Cysteamine (beta-mercaptoethylamine, or MEA) is a thiol-reducing agent and has anti-HIV activity. Because of these properties, cysteamine was evaluated as a vaginal contraceptive and tested for its effects on sperm function and on other sexually transmitted microbes. Cysteamine was contraceptive in the rabbit. Conception was inhibited completely when sperm were pretreated with 500 microg/ml cys...

background: the aim of this study was to assess effects of cysteamine as an anti-oxidant on rate of in vitro maturation of oocyte and determination of its effects on spindle size and shape. methods: pre-mature mice were primed with pregnant mare stimulating gonadotrophin (pmsg) and germinal vesicle (gv) stage oocytes were obtained 48 h after. the oocytes were cultured in tissue culture medium (...

the manipulation of redox potential in secretory pathway by thiol reducing agents can be a strategy to improve the production levels of disulfide-bonded proteins including recombinant antibodies. here we have studied the influence of cysteamine on viability and the production level of igg4 in sp2.0 cells. for this purpose, the recombinant sp2.0 cells producing an anti cd33 igg4, were subjected ...

Abstract Background This study was established to assess cysteamine’s cytotoxic effect alone and in combination with various intracanal medications on fibroblast cells, because the biocompatibility of medication is considered one main factors that affect selection specific for usage near vital periodontal tissues. Methods All tested were prepared a solution form. Cysteamine preparation at 200 m...

Background: Effect of different doses of cysteamine on rate of in vitro maturation (IVM), in vitro fertilization (IVF) and glutathione (GSH) level was studied. Metaphase II (MII) spindle area was analyzed for quantification of shape and size of oocytes. Methods: Female mice were primed with 5 IU of pregnant mare’s stimulating gonadotrophin. Germinal vesicle (GV) oocytes were retrieved 48 hrs la...

Background: The aim of this study was to assess effects of cysteamine as an anti-oxidant on rate of in vitro maturation of oocyte and determination of its effects on spindle size and shape. Methods: Pre-mature mice were primed with pregnant mare stimulating gonadotrophin (PMSG) and germinal vesicle (GV) stage oocytes were obtained 48 h after. The oocytes were cultured in tissue culture medium (...

Cysteamine is approved for the treatment of cystinosis and is being evaluated for Huntington's disease and non-alcoholic fatty liver disease. Little is known about the bioavailability and biodistribution of the drug. The aim was to determine plasma, cerebrospinal fluid (CSF), and tissue (liver, kidney, muscle) cysteamine levels following intraduodenal delivery of the drug in rats pretreated and...

Therapy to slow the relentless expansion of interstitial extracellular matrix that leads to renal functional decline in patients with CKD is currently lacking. Because chronic kidney injury increases tissue oxidative stress, we evaluated the antifibrotic efficacy of cysteamine bitartrate, an antioxidant therapy for patients with nephropathic cystinosis, in a mouse model of unilateral ureteral o...