Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulation of cholesterol and gangliosides, predominantly in endolysosomes, mitochondrial cholesterol accu...

Kombu RS, Zhang GF, Abbas R, Mieyal JJ, Anderson VE, Kelleher JK, Sanabria JR, Brunengraber H. Dynamics of glutathione and ophthalmate traced with H-enriched body water in rats and humans. Am J Physiol Endocrinol Metab 297: E260–E269, 2009. First published April 28, 2009; doi:10.1152/ajpendo.00080.2009.—We developed a LC-MS-MS assay of the H labeling of free glutathione (GSH) and bound glutathi...

conclusions se supplementation can increase plasma se concentration and rbc gsh-pxs activity in patients with different stages of ckd. background plasma selenium (se) concentration and glutathione peroxidase (gsh-pxs) enzyme activity of the patients with chronic kidney disease (ckd) are usually lower than healthy individuals; however, the effect of se supplementation on the gsh-pxs activity in ...

background: from the time immemorial man has been exposed to ionizing radiation from the environment in which he lives. radiation protection concepts and philosophy have been evolving over the past several decades. materials and methods: the radioprotective of effect of aloe vera leaf extract (1000 mg /kg b.wt. orally for 15 consecutive days) has been studied against 6 gy of gamma radiation in ...

Reduced glutathione (GSH) has been demonstrated in benign and malignant human breast lesions using a newly.developed histofluorescence technique. GSH was present in every lesion and in each case was localised to the epithelium. A semi-quantitative assessment revealed a moderate amount of GSH in normal epithelium and fibroadenoma and a high level in apocrine metaplasia, epitheliosis and intraduc...

Glutathione (GSH) is thought to play critical roles in oocyte function including spindle maintenance and provision of reducing power needed to initiate sperm chromatin decondensation. Previous observations that GSH concentrations are higher in mature than immature oocytes and decline after fertilization, suggest that GSH synthesis may be associated with cell cycle events. To explore this possib...

The present study examined the impact of the supplementation of glutathione (GSH), γ-L-glutamyl-L-cysteinyl-glycine, on human blood GSH levels. Healthy human volunteers were orally supplemented with GSH (50 mg/kg body weight). Venous blood was collected from the cubital vein before and after ingestion. Plasma was mixed with 3 volumes of ethanol. The supernatant and precipitate were used for the...

Non-small cell lung cancer (NSCLC) is the leading cause of cancer death in the United States. Because NSCLC is highly chemoresistant, it is, usually not treatable. Altered glutathione (GSH) metabolism is thought to be one major mechanism of chemoresistance, and GSH levels are reported to be elevated in NSCLC. The main objective of this study is to delineate the potential mechanisms involved in ...

For imitating the active site of antioxidant selenoenzyme glutathione peroxidase (GPx), an artificial enzyme selenosubtilisin was employed as a scaffold for reconstructing substrate glutathione (GSH) specific binding sites by a bioimprinting strategy. GSH was first covalently linked to selenosubtilisin to form a covalent complex GSH-selenosubtilisin through a Se-S bond, then the GSH molecule wa...

The physiologically occurring copper-glutathione complex, [Cu(I)-[GSH](2)], has the ability to react continually with oxygen, generating superoxide anions (O(2) (∙-)). We addressed here the effects that superoxide removal has on the redox state of Cu(I) and GSH present in such complex and assessed the formation of Cu(II)-GSSG as a final oxidation product. In addition, we investigated the potent...