drosophila fatty acid transport protein regulates rhodopsin-1 metabolism and is required for photoreceptor neuron survival果蝇脂肪酸运输蛋白调节rhodopsin-1需要新陈代谢,光感受器神经元生存.pdfVIP

Drosophila Fatty Acid Transport Protein Regulates Rhodopsin-1 Metabolism and Is Required for Photoreceptor Neuron Survival 1 1 1 1 1 Pierre Dourlen *, Benjamin Bertin , Gilles Chatelain , Marion Robin , Francesco Napoletano , 2 1 Michel J. Roux , Bertrand Mollereau * ´ ´ 1 Laboratory of Molecular Biology of the Cell, UMR5239 CNRS/Ecole Normale Superieure de Lyon, UMS 344 Biosciences Lyon Gerland, Universite de Lyon, Lyon, France, ´ 2 Translational Medicine and Neurogenetics, IGBMC, UMR7104 CNRS/Universite de Strasbourg/Inserm U964, Strasbourg, France 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 indi