retinitis/glutation

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Interaction of ciliary disease protein retinitis pigmentosa GTPase regulator with nephronophthisis-associated proteins in mammalian retinas.

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OBJECTIVE Retinitis pigmentosa GTPase regulator (RPGR) is a cilia-centrosomal protein that frequently mutates in X-linked retinal degeneration and associated disorders. RPGR interacts with multiple ciliary proteins in the retina. Perturbations in the assembly of RPGR complexes are associated with

Erythrocyte and platelet fatty acids in retinitis pigmentosa.

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The fatty acid composition and the glutathione-peroxidase activity (GSH-Px) of erythrocytes and platelets, the production of malondialdehyde (MDA) by platelets and the activity of the main systems of transmembrane cation transport in erythrocyte have been studied in 12 patients (5 males and 7

Membrane fatty acids, glutathione-peroxidase activity, and cation transport systems of erythrocytes and malondialdehyde production by platelets in Laurence Moon Barter Biedl syndrome.

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The fatty acid composition of erythrocyte membrane, the glutathione-peroxidase activity of erythrocytes and platelets, the production of malondialdehyde by platelets and the activity of the main systems of transmembrane cation transport have been studied in 5 members of a family, 2 of whom affected

Relationships Between Serum Antioxidant and Oxidant Statuses and Visual Function in Retinitis Pigmentosa.

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To investigate the serum changes of antioxidant/oxidant markers and the relationship between these factors and visual function in patients with retinitis pigmentosa (RP).Fifty-two RP patients <40 years old and 25 controls were included. Serum samples

The X-linked retinitis pigmentosa protein RP2 facilitates G protein traffic.

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The X-linked retinitis pigmentosa protein RP2 is a GTPase activating protein (GAP) for the small GTPase Arl3 and both proteins are implicated in the traffic of proteins to the primary cilia. Here, we show that RP2 can facilitate the traffic of the Gβ subunit of transducin (Gβ1). Glutathione

Photoreceptor preservation in the S334ter model of retinitis pigmentosa by a novel estradiol analog.

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Belépés Regisztrálás

The cytoprotective activity of MITO-4565, a novel, non-hormonal, estradiol derivative, was evaluated in the S334ter transgenic model of retinitis pigmentosa (RP). Progressive blindness in RP is due to apoptotic death of the photoreceptors, a process mimicked by the animal models [Portera-Cailliau C,

Alterations in glutamate cysteine ligase content in the retina of two retinitis pigmentosa animal models.

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Belépés Regisztrálás

Retinitis Pigmentosa (RP) comprises a group of rare genetic retinal disorders in which one of several different mutations induces photoreceptor death. Oxidative stress and glutathione (GSH) alterations may be related to the pathogenesis of RP. GSH has been shown to be present in high concentrations

Neuroprotective actions of progesterone in an in vivo model of retinitis pigmentosa.

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Progesterone has been shown to have neuroprotective effects in experimental acute brain injury models, but little is known about the effects of steroid sex hormones in models of retinitis pigmentosa (RP). The aim of this study was to asses whether progesterone had a protective effect in one animal

Increased expression of glutathione peroxidase 4 strongly protects retina from oxidative damage.

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Oxidative damage contributes to cone cell death in retinitis pigmentosa and death of rods, cones, and retinal pigmented epithelial (RPE) cells in age-related macular degeneration. In this study, we explored the strategy of overexpressing components of the endogenous antioxidant defense system to

Is There Excess Oxidative Stress and Damage in Eyes of Patients with Retinitis Pigmentosa?

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Belépés Regisztrálás

Retinitis pigmentosa (RP) is a group of diseases in which a mutation in one of the large variety of genes causes death of rod photoreceptors. After rods die, cone photoreceptors gradually die resulting in constriction of visual fields and eventual blindness in many patients. Studies in animal models

Low glutathione peroxidase in rd1 mouse retina increases oxidative stress and proteases.

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Belépés Regisztrálás

Malondialdehyde, reduced glutathione, glutathione peroxidase, glutathione reductase and cysteine protease cathepsins at postnatal (PN) days 2, 7, 14, 21 and 28 in controls (wt) and the retinal degeneration 1 (rd1) mouse model for retinitis pigmentosa retinas were measured to determine oxidative

The mechanism of cone cell death in Retinitis Pigmentosa.

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Belépés Regisztrálás

Retinitis Pigmentosa (RP) is a group of diseases in which one of a large number of mutations causes death of rod photoreceptors. After rods die, cone photoreceptors slowly degenerate in a characteristic pattern. The mechanism of rod cell death varies depending upon the gene that is mutated and the

Influence of heredity on human sensitivity to environmental chemicals.

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Hereditary peculiarities in individual responses to environmental chemicals are a common occurrence in human populations. Genetic variation in glutathione S-transferase, CYP1A2, N-acetyltransferase, and paraoxonase exemplify the relationship of metabolic variation to individual susceptibility to

Lens opacity induced by lipid peroxidation products as a model of cataract associated with retinal disease.

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The cataractous lenses of patients with retinitis pigmentosa have been studied by electron microscopy. The posterior subcapsular opacities showed common ultrastructural features. Large areas of disruption of the lens fibre pattern were observed which showed an increase in the number of fibre

MPP5 recruits MPP4 to the CRB1 complex in photoreceptors.

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Belépés Regisztrálás

OBJECTIVE Mutations in the human Crumbs homologue 1 (CRB1) gene are a frequent cause of Leber congenital amaurosis (LCA) and various forms of retinitis pigmentosa. CRB1 is thought to organize an intracellular protein scaffold in the retina that is involved in photoreceptor polarity. This study was

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