Nephropathic cystinosis is a rare, autosomal recessive lysosomal storage disorder caused by mutations in the CTNS gene that codes for a cystine transporter in the lysosomal membrane. Affected patients store 50-100 times the normal amounts of cystine in their cells, and suffer renal tubular and glomerular disease, growth retardation, photophobia, and other systemic complications, including a myo...

Abstract Cysteamine functionalised reduced graphene oxide (rGO) was grafted to polypropylene-graft-maleic anhydride (PP-g-MA) and subsequently melt blended with PP. The covalent bridging of rGO PP-g-MA via the cysteamine molecule co-crystallization are routes promoting interfacial interactions between PP matrix. A rheological percolation threshold achieved for a nanofiller loading 3 wt% 5 wt%, ...

Subcutaneous administration of mepirizole (60 and 200 mg/kg) and cysteamine (100 and 300 mg/kg) to fasted rats consistently induced localized villous damage to the proximal duodenum after 6 to 8 hr. The severity of the damage in animals treated with the low doses remained unchanged at 12 hr. With the high doses, however, well-defined deep ulcers were evident by that time, the incidence being hi...

*疋 田光史,堀 川正克,森 俊雄:金 沢大学薬学部放射薬品化学教室,金 沢市宝町13-1〒920 The radioprotective action and cytotoxicity of various sulfhydryl compounds ; 2-mercapto propionylglycine (MPG), 2-mercaptopropionylglycine-amide (MPG-amide), 2-mercaptopropionyl phenylalanine (MPPA), 2-mercaptopropionylphenylglycine (MPPG), 3-mercaptopropionylglycine (3-MPG), AET, cysteine and cysteamine were compared in irradiated and unirradi...

OBJECTIVE To analyze the fertility status in adult, male cystinosis patients treated with cysteamine. Cystinosis is an autosomal recessive disease leading to intralysosomal cystine accumulation. Worldwide, a few female cystinosis patients have given birth. However, no male cystinosis patients are known to have induced pregnancy. Adequate cysteamine treatment might improve male fertility. PATI...

The in vitro developmental competence of equine oocytes is still low in comparison with other domestic mammals. A major factor affecting the viability of cells during in vitro culture is the increased oxidative stress. Oxidative modifications could be responsible for oocyte incompetence for in vitro maturation (IVM). Cysteamine, a glutathione (GSH) synthesis enhancer, has been shown to increase...

INTRODUCTION Cystinosis is a rare, metabolic, autosomal recessive, genetic lysosomal storage disorder characterized by an accumulation of cystine in various organs and tissues. Cysteamine bitartrate (CB) is a cystine-depleting aminothiol agent approved in the United States and Europe in immediate-release and delayed-release (DR) formulations for the treatment of nephropathic cystinosis in child...

Hyperpigmentation disorders are characterized by an overproduction of melanin. Treatments for pigmentary either disrupt the biosynthesis melanin or cytotoxic to melanocyte. Cysteamine a well-studied depigmenting agent inhibits synthesis at several steps in melanogenesis pathway. It is now through development novel complex combining stabilized cysteamine (st. Cys) with isobionic-amide, forming n...

2-Mercaptoethylamine (cysteamine) is an aminothiol compound used as a drug for the treatment of cystinosis, an autosomal recessive lysosomal storage disorder. Because of cysteamine's important role in clinical settings, its analysis by sensitive techniques has become pivotal. Unfortunately, the available methods are either complex or labor intensive. Therefore, we have developed a new rapid, se...

Selective neurotoxins have been of value in providing a means for specifically interfering with the actions of endogenous neurotransmitter candidates. Others have shown cysteamine (CSH) to deplete the gastrointestinal tract and hypothalamus of rats of immunoreactive somatostatin, suggesting a toxic action of that compound directed against somatostatin-containing cells. The present study further...