Description
Product Characteristics:
The nuclear pore complex protein, Ran-binding protein 2 (Ran BP-2 or Nup358), contains four Ran-binding domains. Ran BP-2 is a large scaffold cyclophilin-related protein expressed in photoreceptor cells. Ran BP-2 localization at cytoplasmic fibrils emanating from the nuclear pore complex and interaction with the Ran-GTPase support its role in nucleocytoplasmic transport processes. In humans, the Ran BP-2 gene is partially duplicated in a gene cluster and lies in a hot spot for recombination on chromosome 2q. This genetic heterogeneity renders further significance of this genomic region in human disease due to its possible involvement in genetically linked disorders such as juvenile nephronophthisis, congenital hepatic fibrosis and chorioretinal dysplasia.
Synonyms: Nuclear pore complex protein Nup358, Nucleoporin 358, Nucleoporin Nup358, NUP358, P270, RAN binding protein 2, Ran-binding protein 2, RANBP2, RBP2_HUMAN, Transformation related protein 2, TRP 1, TRP 2, TRP1, TRP2, 358 kDa nucleoporin, E3 SUMO-protein ligase RanBP2.
Target Information: 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]