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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Rhodopsin C terminus, the site of mutations causing retinal disease, regulates trafficking by binding to ADP-ribosylation factor 4 (ARF4)
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Rhodopsin C terminus, the site of mutations causing retinal disease, regulates trafficking by binding to ADP-ribosylation factor 4 (ARF4)

机译：视紫红质C末端是引起视网膜疾病的突变位点，通过与ADP-核糖基化因子4（ARF4）结合来调节运输

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摘要

The maintenance of photoreceptor cell polarity is compromised by the rhodopsin mutations causing the human disease autosomal dominant retinitis pigmentosa. The severe form mutations occur in the C-terminal sorting signal of rhodopsin, VXPX-COOH. Here, we report that this sorting motif binds specifically to the small GTPase ARF4, a member of the ARF family of membrane budding and protein sorting regulators. The effects of blocking ARF4 action were functionally equivalent to the effects of blocking the rhodopsin C-terminal sorting signal. ARF4 was essential for the generation of post-Golgi carriers targeted to the rod outer segments of retinal photoreceptors. Thus, the severe retinitis pigmentosa alleles that affect the rhodopsin sorting signal interfere with interactions between ARF4 and rhodopsin, leading to aberrant trafficking and initiation of retinal degeneration.
机译：视紫红质突变会损害感光细胞极性的维持，导致人类疾病常染色体显性遗传性视网膜色素变性。严重形式的突变发生在视紫红质VXPX-COOH的C端分选信号中。在这里，我们报告此排序基序专门绑定到小的GTPase ARF4，膜芽和蛋白质分选调节剂的ARF家族的成员。阻断ARF4作用的作用在功能上等同于阻断视紫红质C端分选信号的作用。 ARF4对于产生针对视网膜感光器杆外节的高尔基后载体至关重要。因此，影响视紫红质分选信号的严重的视网膜色素变性等位基因干扰ARF4和视紫红质之间的相互作用，导致异常运输和视网膜变性的开始。

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