cystine/atrophy

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Antioxidant supplements prevent oxidation of cysteine/cystine redox in patients with age-related macular degeneration.

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OBJECTIVE Determine whether antioxidant supplements alter the plasma glutathione and/or cysteine redox potential in age-related macular degeneration (AMD) patients. METHODS This was an ancillary study to the Age-Related Eye Disease Study (AREDS), where subset of AREDS subjects at two sites were

Role of amino acids in cochlear degeneration: deprivation of cystine induces death of cochlear hair cells of guinea pigs in vitro.

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Redox regulation reportedly plays a role in maintaining cochlear homeostasis. However, little is known about the roles of oxidation-reduction systems in the cochlea. We examined the role of the cystine/cysteine oxidation-reduction system in survival of cochlea hair cells in vitro. The survival of

Cystine/glutamate antiporter blockage induces myelin degeneration.

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The cystine/glutamate antiporter is a membrane transport system responsible for the uptake of extracellular cystine and release of intracellular glutamate. It is the major source of cystine in most cells, and a key regulator of extrasynaptic glutamate in the CNS. Because cystine is the limiting

[Microchemical studies on the nervous system. VIII. Variations of level of cystine of nerve during degeneration].

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Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex.

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Cystinosis is a disorder associated with lysosomal cystine accumulation caused by defective cystine efflux. Cystine accumulation provokes a variable degree of symptoms depending on the involved tissues. Adult patients may present brain cortical atrophy. However, the mechanisms by which cystine is

Oxidative deterioration of the muscle proteins during nutritional muscular dystrophy in chicks.

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Nutritional muscular dystrophy in the chick results from the simultaneous deficiency of vitamin E and cystine. Being a biological antioxidant, vitamin E might be functional in maintaining a proper redox state of the sulfur-containing amino acid in the proteins. The analyses of protein-bound

Glial loss of the metallo β-lactamase domain containing protein, SWIP-10, induces age- and glutamate-signaling dependent, dopamine neuron degeneration.

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Across phylogeny, glutamate (Glu) signaling plays a critical role in regulating neural excitability, thus supporting many complex behaviors. Perturbed synaptic and extrasynaptic Glu homeostasis in the human brain has been implicated in multiple neuropsychiatric and neurodegenerative disorders

Enhancement of methylmercury toxicity by L-cystine in cultured mouse blastocysts.

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Expanded mouse blastocysts incubated with 1 to 2 microM methylmercury (MeHg) in modified Eagle's basal medium (BME + AA), which contains amino acids, collapsed and degenerated within 24 h. In contrast, blastocysts incubated with the same concentration of MeHg in egg culture medium (ECM), which does

Gyrate atrophy of the choroid and retina with hyperornithinemia, cystinuria and lysinuria responsive to vitamin B6.

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In this report, an 8-year-old girl is presented with the complaint of progressive night blindness. The authors have performed eye funduscopy, which showed chorioretinal atrophy in gyrate shape. A high level of plasma ornithine was determined. Urinary excretion of ornithine as well as lysine and

Differential regulation of cell proliferation in neurogenic zones in mice lacking cystine transport by xCT.

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The cystine/glutamate exchanger (xCT) supplies intracellular cyst(e)ine for the production of glutathione, a major cellular anti-oxidant. xCT is enriched in brain regions associated with neurogenesis. Previous studies have shown that the malfunction of this protein greatly attenuates cell

Idebenone attenuates neuronal degeneration induced by intrastriatal injection of excitotoxins.

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Previous studies with the N18-RE-105 neuronal-like cell line and primary cortical cultures demonstrate that glutamate can produce a calcium-dependent, delayed form of neuronal degeneration that results from its competitive inhibition of cystine transport, which leads to cellular glutathione

Plasma biomarkers of oxidative stress and genetic variants in age-related macular degeneration.

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OBJECTIVE To compare plasma levels of oxidative stress biomarkers in patients with age-related macular degeneration (AMD) and controls and to evaluate a potential relationship between biochemical markers of oxidative stress and AMD susceptibility genotypes. METHODS Prospective case-control

Reversible leukoencephalopathy with acute neurological deterioration and permanent residua in classical homocystinuria: A case report.

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We report a 24-year-old patient with underlying classical homocystinuria who developed acute neurological deterioration apparently induced by malnutrition secondary to poor compliance with treatment and pancreatitis. Neurological examination revealed stupor, tremor of the upper extremities,

Effects of a Lactobacillus reuteri BR11 mutant deficient in the cystine-transport system in a rat model of inflammatory bowel disease.

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BACKGROUND Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the gastrointestinal tract associated with altered composition of the gut microbiota. Lactobacillus reuteri BR11 (BR11) has recently been reported to reduce the severity of experimental IBD because of its probiotic

The mechanism of glutamate-induced degeneration of cultured Huntington's disease and control fibroblasts.

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Human cultured skin fibroblasts undergo rapid cellular degeneration and cell death when exposed to moderate levels of glutamate (10-30 mM). Huntington's disease (HD) skin fibroblast cultures are more sensitive to the toxic effects of glutamate (Gray et al. 1980) and elucidation of the toxic

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