@article{HankeGogokhiaFrederickZhangetal.2018,
  author    = {Hanke-Gogokhia, Christin and Frederick, Jeanne M. and Zhang, Houbin and Baehr, Wolfgang},
  title     = {Binary Function of ARL3-GTP Revealed by Gene Knockouts},
  series = {Retinal Degenerative Diseases : Mechanisms and Experimental Therapy},
  volume    = {1074},
  journal   = {Retinal Degenerative Diseases : Mechanisms and Experimental Therapy},
  publisher = {Springer},
  address   = {Cham},
  isbn      = {978-3-319-75402-4},
  issn      = {0065-2598},
  doi       = {10.1007/978-3-319-75402-4_39},
  pages     = {317 -- 325},
  year      = {2018},
  abstract  = {UNC119 and PDE delta are lipid-binding proteins and are thought to form diffusible complexes with transducin-alpha and prenylated OS proteins, respectively, to mediate their trafficking to photoreceptor outer segments. Here, we investigate mechanisms of trafficking which are controlled by Arf-like protein 3 (Arl3), a small GTPase. The activity of ARL3 is regulated by a GEF (ARL13b) and a GAP (RP2). In a mouse germline knockout of RP2, ARL3-GTP is abundant as its intrinsic GTPase activity is extremely low. High levels of ARL3-GTP impair binding and trafficking of cargo to the outer segment. Germline knockout of ARL3 is embryonically lethal generating a syndromic ciliopathy-like phenotype. Retina-and rod-specific knockout of ARL3 allow to determine the precise mechanisms leading to photoreceptor degeneration. The knockouts reveal binary functions of ARL3-GTP as a key molecule in late-stage photoreceptor ciliogenesis and cargo displacement factor.},
  language  = {en}
}