473 research outputs found

    Coenzyme Q10 as a therapeutic candidate for treating inherited photoreceptor degeneration

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    Inherited photoreceptor degeneration (IPD): The human retina is a highly specialised tissue that enables the perception of light across a range of intensities and colours. It covers about 65% of the inner surface of the eye and contains three layers of cells: the outer nuclear layer (ONL) containing the cell bodies and nuclei of the light-sensitive rod and cone photoreceptors whose photopigment-containing outer segments form the photoreceptor layer; the inner nuclear layer (INL) containing bipolar, horizontal and amacrine cells; and the ganglion cell layer (GCL) from which the optic nerve arises. There are two layers of synaptic connections between these three layers: the photoreceptors synapse with second order neurons, mainly bipolar cells, in the outer plexiform layer (OPL), while in turn the bipolar cells form connections in the inner plexiform layer (IPL) with ganglion cells. The retinal pigment epithelium (RPE) lies directly behind the photoreceptor layer, is heavily pigmented to reduce scattering of light, and is essential for the nourishment, maintenance and metabolism of photoreceptors

    The first Chinese student space shuttle getaway special program

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    The first Chinese Getaway Special program is described. Program organization, the student proposal evaluation procedure, and the objectives of some of the finalist's experiments are covered. The two experiments selected for eventual flight on the space shuttle are described in detail. These include: (1) the control of debris in the cabin of the space shuttle; and (2) the solidification of two immiscible liquids in space

    Therapeutic potential of co-enzyme Q10 in retinal diseases

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    Coenzyme Q10 (CoQ10) plays a critical role in mitochondrial oxidative phosphorylation by serving as an electron carrier in the respiratory electron transport chain. CoQ10 also functions as a lipid-soluble antioxidant by protecting lipids, proteins and DNA damaged by oxidative stress. CoQ10 deficiency has been associated with a number of human diseases including mitochondrial diseases, neurodegenerative disorders, cardiovascular diseases, diabetes, cancer, and with the ageing process. In many of these conditions CoQ10 supplementation therapy has been effective in slowing or reversing pathological changes. Oxidative stress is a major contributory factor in the process of retinal degeneration. In this brief review, we summarize the functions of CoQ10 and highlight its use in the treatment of age-related macular degeneration and glaucoma. In light of these data we propose that CoQ10 could have therapeutic potential for other retinal diseases

    Unraveling the unusually high electrical conductivity of the delafossite metal PdCoO2_2

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    The prototypical delafossite metal PdCoO2_2 has been the subject of intense interest for hosting exotic transport properties. Using first-principles transport calculations and theoretical modeling, we reveal that the high electrical conductivity of PdCoO2_2 at room temperature originates from the contributions of both high Fermi velocities, enabled by Pd 4dz25s4d_{z^2}-5s hybridization, and exceptionally weak electron-phonon coupling, which leads to a coupling strength (λ=0.057\lambda=0.057) that is nearly an order of magnitude smaller than those of common metals. The abnormally weak electron-phonon coupling in PdCoO2_2 results from a low electronic density of states at the Fermi level, as well as the large and strongly facetted Fermi surface with suppressed Umklapp electron-phonon matrix elements. We anticipate that our work will inform the design of unconventional metals with superior transport properties.Comment: 6 pages, 3 figure

    The role of RPGR and its interacting proteins in ciliopathies

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    Ciliopathies encompass a group of genetic disorders characterized by defects in the formation, maintenance, or function of cilia. Retinitis pigmentosa (RP) is frequently one of the clinical features presented in diverse ciliopathies. RP is a heterogeneous group of inherited retinal disorders, characterized by the death of photoreceptors and affecting more than one million individuals worldwide. The retinitis pigmentosa GTPase regulator (RPGR) gene is mutated in up to 20% of all RP patients. RPGR protein has different interacting partners to function in ciliary protein trafficking. In this review, we specifically focus on RPGR and its two interacting proteins: RPGRIP1 and RPGRIP1L. We summarize the function of the three proteins and highlight recent studies that provide insight into the cellular function of those proteins
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