RAN is a small GTP-binding protein of the RAS superfamily that is associated with the nuclear membrane and is thought to control a variety of cellular functions through its interactions with other proteins. This gene encodes a very large RAN-binding protein that immunolocalizes to the nuclear pore complex. The protein is a giant scaffold and mosaic cyclophilin-related nucleoporin implicated in the Ran-GTPase cycle. The encoded protein directly interacts with the E2 enzyme UBC9 and strongly enhances SUMO1 transfer from UBC9 to the SUMO1 target SP100. These findings place sumoylation at the cytoplasmic filaments of the nuclear pore complex and suggest that, for some substrates, modification and nuclear import are linked events. This gene is partially duplicated in a gene cluster that lies in a hot spot for recombination on chromosome 2q. [provided by RefSeq, Jul 2008]
RANBP2; RAN binding protein 2; ANE1; TRP1; TRP2; ADANE; IIAE3; NUP358; E3 SUMO-protein ligase RanBP2; P270; nucleoporin 358; nucleoporin Nup358; 358 kDa nucleoporin; ran-binding protein 2; transformation-related protein 2; nuclear pore complex protein Nup358; acute necrotizing encephalopathy 1 (autosomal dominant);
Senecal, JL; Isabelle, C; et al. An Autoimmune Myositis-Overlap Syndrome Associated With Autoantibodies to Nuclear Pore Complexes Description and Long-Term Follow-up of the Anti-Nup Syndrome. MEDICINE 93:361-372(2014).
Splinter, D; Tanenbaum, ME; et al. Bicaudal D2, Dynein, and Kinesin-1 Associate with Nuclear Pore Complexes and Regulate Centrosome and Nuclear Positioning during Mitotic Entry. PLOS BIOLOGY 8:-(2010).