期刊
TRENDS IN NEUROSCIENCES
卷 36, 期 11, 页码 652-660出版社
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tins.2013.08.003
关键词
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资金
- Houston Laboratory and Population Science Training Program in Gene Environment Interaction from Burroughs Wellcome Fund (BWF) [1008200]
- National Institutes of Health (NIH) [1RC4GM096355-01]
Rhodopsins (Rh) are G protein-coupled receptors that function as light-sensors in photoreceptors. In humans, Rh mutations cause retinitis pigmentosa (RP), a degenerative disease that ultimately results in blindness. Studies in Drosophila have provided many insights into basic Rh biology and have identified pathways that lead to retinal degeneration. It has been shown that, because Rh is very abundant in photoreceptors, its accumulation in numerous organelles induces severe stress and results in degeneration of these cells. Moreover, genetic lesions that affect proper activation of membrane-bound Rh lead to disruption in Ca2+ homeostasis which also causes photoreceptor degeneration. We review here the molecular signals involved in Rh homeostasis and the mechanisms underlying retinal degeneration in flies, and discuss possible links to human diseases.
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