RANBP2
Available structures PDB Human UniProt search: PDBe RCSB List of PDB id codes 1RRP, 1XKE, 1Z5S, 3UIN, 3UIO, 3UIP, 4I9Y, 4L6E, 4LQW, 5CLL, 5CLQ, 2LAS
Identifiers
Aliases	RANBP2, ADANE, ANE1, IIAE3, NUP358, TRP1, TRP2, RAN binding protein 2
External IDs	OMIM: 601181 HomoloGene: 136803 GeneCards: RANBP2
Gene location (Human) Chr. Chromosome 2 (human)[1] Band 2q13 Start 108,719,482 bp[1] End 108,785,809 bp[1]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in endothelial cell sperm tibia visceral pleura superficial temporal artery seminal vesicula germinal epithelium parietal pleura cavity of mouth biceps brachii n/a More reference expression data BioGPS More reference expression data
Gene ontology Molecular function peptidyl-prolyl cis-trans isomerase activity metal ion binding ligase activity protein binding RNA binding SUMO transferase activity GTPase activator activity protein-containing complex binding cyclosporin A binding transferase activity Cellular component cytosol nuclear membrane membrane nuclear pore nuclear inclusion body nuclear pore nuclear basket nucleus nuclear envelope lamellae anulatae intracellular membrane-bounded organelle cytoplasm centrosome mitochondrion nuclear pore cytoplasmic filaments cytoplasmic periphery of the nuclear pore complex host cell Biological process mRNA transport viral transcription intracellular transport protein sumoylation protein peptidyl-prolyl isomerization mitotic nuclear membrane disassembly protein folding regulation of cellular response to heat protein transport viral process NLS-bearing protein import into nucleus intracellular transport of virus mRNA export from nucleus tRNA export from nucleus sister chromatid cohesion centrosome localization G1 phase RNA export from nucleus ubiquitin-dependent protein catabolic process spindle organization positive regulation of GTPase activity positive regulation of mitotic centrosome separation response to amphetamine positive regulation of glucokinase activity regulation of gene silencing by miRNA regulation of glycolytic process transport regulation of gluconeogenesis Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 5903 n/a Ensembl ENSG00000153201 n/a UniProt P49792 n/a RefSeq (mRNA) NM_006267 n/a RefSeq (protein) NP_006258 n/a Location (UCSC) Chr 2: 108.72 – 108.79 Mb n/a PubMed search [2] n/a
Wikidata
View/Edit Human

RAN binding protein 2 (RANBP2) is protein which in humans is encoded by the RANBP2 gene.^[3] It is also known as nucleoporin 358 (Nup358) since it is a member nucleoporin family that makes up the nuclear pore complex. RanBP2 has a mass of 358 kDa.

Function

RAN is a small GTP-binding protein of the RAS superfamily. Ran GTPase is a master regulatory switch, which among other functions, controls the shuttling of proteins between the nuclear and cytoplasm compartments of the cell. Ran GTPase controls a variety of cellular functions through its interactions with other proteins. The RanBP2 gene encodes a very large RAN-binding protein that localizes to cytoplasmic filaments emanating from the nuclear pore complex. RanBP2/Nup358 is a giant scaffold and mosaic cyclophilin-related nucleoporin implicated in controlling selective processes of the Ran-GTPase cycle. RanBP2 is composed of multiple domains. Each domain of RanBP2 selectively and directly interacts with distinct proteins such as Ran GTPase, importin-beta, exportin-1/CRM1, red opsin, subunits of the proteasome, cox11 and the kinesin-1 isoforms, KIF5B and KIF5C. Another partner of RanBP2 is the E2 enzyme UBC9. RanBP2 strongly enhances SUMO1 transfer from UBC9 to the SUMO1 target SP100. Another target for SUMOylation is RanGAP which is the GTPase activating protein for Ran. SUMO-RanGAP interacts with a domain near the carboxyl terminus of RanBP2. 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. The pleiotropic (multifunctional) role of RanBP2 reflects its interaction with multiple partners, each presenting distinct cellular or molecular functions. This gene is partially duplicated in a gene cluster that lies in a hot spot for recombination on human chromosome 2q.

Clinical significance

Insufficiency of RanBP2 is directly linked to carcinogenesis, aneuploidy, and neuroprotection of photoreceptor neurons to light-elicited stress and aging. Human missense mutations in RanBP2 were identified in its leucine-rich domain and they cause autosomal dominant necrotizing encephalopathy (ADNE).^[4]

Interactions

RANBP2 has been shown to interact with KPNB1^[5][6][7] and UBE2I.^[8][9]

References

Further reading