cystinuria/glutathione

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Metabolic consequences of cystinuria.

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Ielogoties Reģistrēties

Cystinuria is an inherited disorder of renal amino acid transport that causes recurrent nephrolithiasis and significant morbidity in humans. It has an incidence of 1 in 7000 worldwide making it one of the most common genetic disorders in man. We phenotypically characterized a mouse

The role of glutathione turnover in the apparent renal secretion of cystine.

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Ielogoties Reģistrēties

Previous studies with cystinuric dogs and humans have demonstrated that the amount of cystine excreted in the urine is, in some cases, larger than the amount of cystine removed from the plasma by glomerular filtration. It was concluded that the kidney must secrete cystine into the renal tubule. The

A case of cystinuria presenting with cerebellar ataxia and dementia.

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Ielogoties Reģistrēties

Cystinuria normally manifests as recurrent urinary stones and renal dysfunction, but can present to neurologists with ataxia, posterior column impairment, intellectual deficiency and pyramidal and extrapyramidal signs; the neuroradiological features include cerebellar, brainstem and cerebral

Sulfur amino acid metabolism in cystinuria: a biochemical and clinical study of patients.

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Ielogoties Reģistrēties

Sulfur amino acid metabolism was studied in 26 homozygotic cystinuric patients, some of whom received D-penicillamine, 2-mercaptopropionylglycine or N-acetylcysteine treatments in order to evaluate signs of cyst(e)ine deficiency. Decreased leukocyte glutathione and taurine levels, plasma cyst(e)ine

Developmental pattern of cystine transport in isolated rat renal tubules.

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Ielogoties Reģistrēties

Isolated renal cortical tubule fragments from rats ranging in age from less than 48 h to 15 weeks were used to examine the pattern of cystine uptake with development. Immature tubules took up cystine with a faster initial rate than mature tubules and did not reach a steady state by 60 min. By eight

Cellular accumulation of L-cystine in rat kidney cortex in vivo.

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Ielogoties Reģistrēties

Cellular accumulation of L-cystine in rat kidney cortex in vivo has been studied using L-[(35)S]cystine. The L-[(35)S]cystine radioactivity in plasma decreases to less than 10% of the initially calculated value by 15 min. Four (35)S-containing intracellular products of L-cystine metabolism were

Cystine and dibasic amino acid uptake by opossum kidney cells.

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Ielogoties Reģistrēties

The characteristics of the uptake of L-cystine by the continuous opossum kidney cell line, OK, were examined. Uptake of cystine is rapid and, in contrast to other continuous cultured cell lines, these cells retain the cystine/dibasic amino acid transport system which is found in vivo and in freshly

D-penicillamine combined with inhibitors of hydroperoxide metabolism enhances lung and breast cancer cell responses to radiation and carboplatin via H2O2-mediated oxidative stress.

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Ielogoties Reģistrēties

D-penicillamine (DPEN), a copper chelator, has been used in the treatment of Wilson's disease, cystinuria, and rheumatoid arthritis. Recent evidence suggests that DPEN in combination with biologically relevant copper (Cu) concentrations generates H2O2 in cancer cell cultures, but the effects of this

D-Penicillamine targets metastatic melanoma cells with induction of the unfolded protein response (UPR) and Noxa (PMAIP1)-dependent mitochondrial apoptosis.

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Ielogoties Reģistrēties

D-Penicillamine (3,3-dimethyl-D-cysteine; DP) is an FDA-approved redox-active D-cysteine-derivative with antioxidant, disulfide-reducing, and metal chelating properties used therapeutically for the control of copper-related pathology in Wilson's disease and reductive cystine-solubilization in

Capillary electrophoresis for diagnosis and studies of human disease, particularly metabolic disorders.

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Ielogoties Reģistrēties

High-performance capillary electrophoresis (HPCE) has been used in a multicomponent analytical system designed to diagnose and study human diseases, particularly metabolic disorders. Comparative analyses, using HPCE, high-performance liquid chromatography (HPLC) and an automated amino acid analyser,

Plasma D-penicillamine redox state evaluation by capillary electrophoresis with laser-induced fluorescence.

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Ielogoties Reģistrēties

D-Penicillamine (D-Pen) is a thiol drug used in the treatment of Wilson's disease, rheumatoid arthritis, metal intoxication and cystinuria. We have recently described a new capillary electrophoresis (CE) method to measure physiological thiols, in which separation of total plasma homocysteine,

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