Hanke-Gogokhia Christin, Zhang Houbin, Frederick Jeanne M, Baehr Wolfgang
Department of Physical Biochemistry, University of Potsdam, Karl-Liebknecht-Strasse 24-25, 14476, Potsdam, Germany.
Department of Ophthalmology, John A. Moran Eye Center, University of Utah Health Science Center, 65 Mario Capecchi Drive, 84132, Salt Lake City, UT, USA.
Adv Exp Med Biol. 2016;854:655-61. doi: 10.1007/978-3-319-17121-0_87.
Arf-like proteins (ARLs) are ubiquitously expressed small G proteins of the RAS superfamily. In photoreceptors, ARL2 and ARL3 participate in the trafficking of lipidated membrane-associated proteins and colocalize in the inner segment with UNC119A and PDEδ. UNC119A and PDEδ are acyl- and prenyl-binding proteins, respectively, involved in trafficking of acylated (transducin-α subunit, nephrocystin NPHP3) and prenylated proteins (GRK1, PDE6). Germline Arl3 knockout mice do not survive beyond postnatal day 21 and display ciliary defects in multiple organs (kidney, liver and pancreas) as well as retinal degeneration. Conditional knockouts will be necessary to delineate mechanisms of protein transport in retina disease.
Arf样蛋白（ARLs）是RAS超家族中普遍表达的小G蛋白。在光感受器中，ARL2和ARL3参与脂化膜相关蛋白的运输，并与UNC119A和PDEδ在内节中共定位。UNC119A和PDEδ分别是酰基结合蛋白和异戊二烯基结合蛋白，参与酰化蛋白（转导素-α亚基、肾囊肿蛋白NPHP3）和异戊二烯化蛋白（GRK1、PDE6）的运输。生殖系Arl3基因敲除小鼠出生后21天内无法存活，并在多个器官（肾脏、肝脏和胰腺）出现纤毛缺陷以及视网膜变性。有必要进行条件性基因敲除以阐明视网膜疾病中蛋白质运输的机制。
