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果蝇脂肪酸转运蛋白调节视紫红质-1 的代谢,是感光神经元存活所必需的。

Drosophila fatty acid transport protein regulates rhodopsin-1 metabolism and is required for photoreceptor neuron survival.

机构信息

Laboratory of Molecular Biology of the Cell, UMR5239 CNRS/Ecole Normale Supérieure de Lyon, UMS 344 Biosciences Lyon Gerland, Université de Lyon, Lyon, France.

出版信息

PLoS Genet. 2012;8(7):e1002833. doi: 10.1371/journal.pgen.1002833. Epub 2012 Jul 26.

Abstract

Tight regulation of the visual response is essential for photoreceptor function and survival. Visual response dysregulation often leads to photoreceptor cell degeneration, but the causes of such cell death are not well understood. In this study, we investigated a fatty acid transport protein (fatp) null mutation that caused adult-onset and progressive photoreceptor cell death. Consistent with fatp having a role in the retina, we showed that fatp is expressed in adult photoreceptors and accessory cells and that its re-expression in photoreceptors rescued photoreceptor viability in fatp mutants. The visual response in young fatp-mutant flies was abnormal with elevated electroretinogram amplitudes associated with high levels of Rhodopsin-1 (Rh1). Reducing Rh1 levels in rh1 mutants or depriving flies of vitamin A rescued photoreceptor cell death in fatp mutant flies. Our results indicate that fatp promotes photoreceptor survival by regulating Rh1 abundance.

摘要

视觉反应的严格调控对光感受器功能和存活至关重要。视觉反应失调常导致光感受器细胞变性,但这种细胞死亡的原因尚不清楚。在这项研究中,我们研究了一种脂肪酸转运蛋白 (fatp) 缺失突变,该突变导致成年后进行性光感受器细胞死亡。与 fatp 在视网膜中发挥作用一致,我们表明 fatp 在成年光感受器和辅助细胞中表达,并且其在光感受器中的重新表达挽救了 fatp 突变体中的光感受器活力。年轻的 fatp 突变果蝇的视觉反应异常,与高水平 Rhodopsin-1 (Rh1) 相关的视网膜电图幅度升高。在 rh1 突变体中降低 Rh1 水平或剥夺果蝇维生素 A 可挽救 fatp 突变果蝇中的光感受器细胞死亡。我们的结果表明,fatp 通过调节 Rh1 的丰度来促进光感受器的存活。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a4f/3405995/d643cfbe0040/pgen.1002833.g001.jpg

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