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Aliases for RANBP2 Gene

Aliases for RANBP2 Gene

  • RAN Binding Protein 2 2 3
  • NUP358 3 4 6
  • Acute Necrotizing Encephalopathy 1 (Autosomal Dominant) 2 3
  • Nuclear Pore Complex Protein Nup358 3 4
  • Ran-Binding Protein 2 3 4
  • 358 KDa Nucleoporin 3 4
  • Nucleoporin Nup358 3 4
  • IIAE3 3 6
  • P270 3 4
  • ANE1 3 6
  • Transformation-Related Protein 2 3
  • E3 SUMO-Protein Ligase RanBP2 3
  • Nucleoporin 358 3
  • EC 63
  • EC 6.3.2.- 4
  • RanBP2 4
  • ADANE 3
  • TRP1 3
  • TRP2 3

External Ids for RANBP2 Gene

Previous Symbols for RANBP2 Gene

  • ANE1

Summaries for RANBP2 Gene

Entrez Gene Summary for RANBP2 Gene

  • 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]

GeneCards Summary for RANBP2 Gene

RANBP2 (RAN Binding Protein 2) is a Protein Coding gene. Diseases associated with RANBP2 include familial acute necrotizing encephalopathy and acute necrotizing encephalopathy of childhood. Among its related pathways are Disease and Cell Cycle, Mitotic. GO annotations related to this gene include ligase activity and Ran GTPase binding. An important paralog of this gene is PPIE.

UniProtKB/Swiss-Prot for RANBP2 Gene

  • E3 SUMO-protein ligase which facilitates SUMO1 and SUMO2 conjugation by UBE2I. Involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain which acts as a docking site for substrates. Binds single-stranded RNA (in vitro). May bind DNA. Component of the nuclear export pathway. Specific docking site for the nuclear export factor exportin-1. Sumoylates PML at Lys-490 which is essential for the proper assembly of PML-NB.

Gene Wiki entry for RANBP2 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for RANBP2 Gene

Genomics for RANBP2 Gene

Genomic Location for RANBP2 Gene

108,719,446 bp from pter
108,785,811 bp from pter
66,366 bases
Plus strand

Genomic View for RANBP2 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for RANBP2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for RANBP2 Gene

Regulatory Elements for RANBP2 Gene

Proteins for RANBP2 Gene

  • Protein details for RANBP2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    E3 SUMO-protein ligase RanBP2
    Protein Accession:
    Secondary Accessions:
    • Q13074
    • Q15280
    • Q53TE2
    • Q59FH7

    Protein attributes for RANBP2 Gene

    3224 amino acids
    Molecular mass:
    358199 Da
    Quaternary structure:
    • Forms a tight complex with RANBP1 and UBE2I. Interacts with SUMO1 but not SUMO2. Interacts with PARK2. Interacts with sumoylated RANGAP1. Interacts with CDCA8. Interacts with PML (isoform PML-4).

    Three dimensional structures from OCA and Proteopedia for RANBP2 Gene

neXtProt entry for RANBP2 Gene

Proteomics data for RANBP2 Gene at MOPED

Selected DME Specific Peptides for RANBP2 Gene

Post-translational modifications for RANBP2 Gene

  • Polyubiquitinated by PARK2, which leads to proteasomal degradation.
  • The the inner channel of the NPC has a different redox environment from the cytoplasm and allows the formation of interchain disulfide bonds between some nucleoporins, the significant increase of these linkages upon oxidative stress reduces the permeability of the NPC.
  • Modification sites at PhosphoSitePlus
  • Ubiquitination at Lys106, Lys798, Lys2061, Lys2125, Lys2156, Lys2181, Lys2358, Lys2396, Lys2424, Lys2522, and Lys2959

Other Protein References for RANBP2 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Domains for RANBP2 Gene

Gene Families for RANBP2 Gene

  • TTC :Tetratricopeptide (TTC) repeat domain containing

Suggested Antigen Peptide Sequences for RANBP2 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Contains a dozen F-X-F-G repeats in the C-terminal half.:
    • P49792
  • The PPIase cyclophilin-type domain has high structural similarity with PPIA, but has extremely low and barely detectable proline isomerase activity (in vitro) (PubMed:23353830). Only about half of the residues that surround the PPIA active site cleft are conserved.:
    • P49792
  • Contains 1 PPIase cyclophilin-type domain.:
    • P49792
  • Contains 4 RanBD1 domains.:
    • P49792
  • Contains 8 RanBP2-type zinc fingers.:
    • P49792
  • Contains 7 TPR repeats.:
    • P49792
genes like me logo Genes that share domains with RANBP2: view

Function for RANBP2 Gene

Molecular function for RANBP2 Gene

GENATLAS Biochemistry: Ran (Ras-related small nuclear protein)component of the nuclear pore complex (NPC),involved in cell-cycle progression,nucleocytoplasmic transport,pre-mRNA processing
UniProtKB/Swiss-Prot Function: E3 SUMO-protein ligase which facilitates SUMO1 and SUMO2 conjugation by UBE2I. Involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain which acts as a docking site for substrates. Binds single-stranded RNA (in vitro). May bind DNA. Component of the nuclear export pathway. Specific docking site for the nuclear export factor exportin-1. Sumoylates PML at Lys-490 which is essential for the proper assembly of PML-NB.

Enzyme Numbers (IUBMB) for RANBP2 Gene

Gene Ontology (GO) - Molecular Function for RANBP2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0003723 RNA binding IEA --
GO:0003755 peptidyl-prolyl cis-trans isomerase activity IEA --
GO:0005515 protein binding IPI 16314522
GO:0008270 zinc ion binding IEA --
GO:0008536 Ran GTPase binding TAS 7603572
genes like me logo Genes that share ontologies with RANBP2: view
genes like me logo Genes that share phenotypes with RANBP2: view

Animal Models for RANBP2 Gene

MGI Knock Outs for RANBP2:

No data available for Transcription Factor Targeting and HOMER Transcription for RANBP2 Gene

Localization for RANBP2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for RANBP2 Gene

Nucleus. Nucleus membrane. Nucleus, nuclear pore complex. Note=Detected in diffuse and discrete intranuclear foci. Cytoplasmic filaments.

Subcellular locations from

Jensen Localization Image for RANBP2 Gene COMPARTMENTS Subcellular localization image for RANBP2 gene
Compartment Confidence
nucleus 5
cytosol 4
cytoskeleton 1
golgi apparatus 1
peroxisome 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for RANBP2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005643 nuclear pore IDA 17098863
GO:0005739 mitochondrion IEA --
GO:0005829 cytosol TAS --
GO:0016020 membrane IDA 19946888
GO:0031965 nuclear membrane IDA 11839768
genes like me logo Genes that share ontologies with RANBP2: view

Pathways for RANBP2 Gene

SuperPathways for RANBP2 Gene

genes like me logo Genes that share pathways with RANBP2: view

Pathways by source for RANBP2 Gene

UniProtKB/Swiss-Prot P49792-RBP2_HUMAN

  • Pathway: Protein modification; protein sumoylation

Gene Ontology (GO) - Biological Process for RANBP2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000278 mitotic cell cycle TAS --
GO:0000413 protein peptidyl-prolyl isomerization IEA --
GO:0005975 carbohydrate metabolic process TAS --
GO:0006457 protein folding IEA --
GO:0006606 protein import into nucleus TAS 7603572
genes like me logo Genes that share ontologies with RANBP2: view

Compounds for RANBP2 Gene

(1) HMDB Compounds for RANBP2 Gene

Compound Synonyms Cas Number PubMed IDs
Guanosine triphosphate
  • 5'-GTP

(4) Novoseek inferred chemical compound relationships for RANBP2 Gene

Compound -log(P) Hits PubMed IDs
gppnhp 54.5 1
leptomycin b 54.4 1
gtp 51.6 7
zinc 5.08 7
genes like me logo Genes that share compounds with RANBP2: view

Transcripts for RANBP2 Gene

Unigene Clusters for RANBP2 Gene

RAN binding protein 2:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for RANBP2 Gene

ExUns: 1 ^ 2 ^ 3 ^ 4 ^ 5 ^ 6 ^ 7 ^ 8 ^ 9 ^ 10 ^ 11a · 11b ^ 12 ^ 13 ^ 14 ^ 15 ^ 16a · 16b · 16c ^ 17 ^ 18a · 18b ^ 19
SP1: - - -
SP2: - -

Relevant External Links for RANBP2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for RANBP2 Gene

mRNA expression in normal human tissues for RANBP2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for RANBP2 Gene

SOURCE GeneReport for Unigene cluster for RANBP2 Gene Hs.199561

genes like me logo Genes that share expressions with RANBP2: view

No data available for mRNA differential expression in normal tissues and mRNA Expression by UniProt/SwissProt for RANBP2 Gene

Orthologs for RANBP2 Gene

This gene was present in the common ancestor of animals and fungi.

Orthologs for RANBP2 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia RANBP2 35
  • 99.36 (n)
  • 98.88 (a)
  • 99 (a)
(Bos Taurus)
Mammalia RANBP2 36
  • 85 (a)
(Canis familiaris)
Mammalia RANBP2 35
  • 90.39 (n)
  • 89.82 (a)
  • 90 (a)
(Mus musculus)
Mammalia Ranbp2 16
Ranbp2 36
  • 87 (a)
(Monodelphis domestica)
Mammalia RANBP2 36
  • 78 (a)
(Ornithorhynchus anatinus)
Mammalia RANBP2 36
  • 73 (a)
(Gallus gallus)
Aves -- 36
  • 65 (a)
(Anolis carolinensis)
Reptilia -- 36
  • 68 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.7285 35
tropical clawed frog
(Silurana tropicalis)
Amphibia Str.1708 35
(Danio rerio)
Actinopterygii Dr.29687 35
fruit fly
(Drosophila melanogaster)
Insecta CG5071 36
  • 25 (a)
Cyp1 37
  • 66 (a)
(Caenorhabditis elegans)
Secernentea T22F3.12 36
  • 39 (a)
Y17G9B.4 36
  • 34 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes YRB1 38
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 22 (a)
Species with no ortholog for RANBP2:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rat (Rattus norvegicus)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for RANBP2 Gene

Gene Tree for RANBP2 (if available)
Gene Tree for RANBP2 (if available)

Paralogs for RANBP2 Gene

Paralogs for RANBP2 Gene

genes like me logo Genes that share paralogs with RANBP2: view

Variants for RANBP2 Gene

Sequence variations from dbSNP and Humsavar for RANBP2 Gene

SNP ID Clin Chr 02 pos Sequence Context AA Info Type MAF
rs12770 -, - 108,766,213(-) CACAG(C/G)GATGG reference, missense
rs700865 -- 108,757,292(-) TACCT(C/G)ATCTC intron-variant
rs700875 -- 108,772,668(+) TAATT(A/T)TCGAT intron-variant
rs700876 -- 108,772,390(-) CACAT(A/C/T)TGAAT intron-variant
rs749946 -- 108,773,660(-) CCCCC(A/C)AAAAA intron-variant

Structural Variations from Database of Genomic Variants (DGV) for RANBP2 Gene

Variant ID Type Subtype PubMed ID
esv2720498 CNV Deletion 23290073

Relevant External Links for RANBP2 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for RANBP2 Gene

Disorders for RANBP2 Gene

(1) OMIM Diseases for RANBP2 Gene (601181)


  • Encephalopathy, acute, infection-induced, 3 (IIAE3) [MIM:608033]: A rapidly progressive encephalopathy manifesting in susceptible individuals with seizures and coma. It can occur within days in otherwise healthy children after common viral infections such as influenza and parainfluenza, without evidence of viral infection of the brain or inflammatory cell infiltration. Brain T2-weighted magnetic resonance imaging reveals characteristic symmetric lesions present in the thalami, pons and brainstem. {ECO:0000269 PubMed:19118815}. Note=The disease is caused by mutations affecting the gene represented in this entry. Mutations in the RANBP2 gene predispose to IIAE3, but by themselves are insufficient to make the phenotype fully penetrant; additional genetic and environmental factors are required (PubMed:19118815). {ECO:0000269 PubMed:19118815}.

(2) Novoseek inferred disease relationships for RANBP2 Gene

Disease -log(P) Hits PubMed IDs
tumors 0 2
melanoma 0 1

Relevant External Links for RANBP2

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with RANBP2: view

Publications for RANBP2 Gene

  1. Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. (PMID: 19118815) Neilson D.E. … Warman M.L. (Am. J. Hum. Genet. 2009) 2 3 4 23
  2. A giant nucleopore protein that binds Ran/TC4. (PMID: 7603572) Yokoyama N. … Aebi U. (Nature 1995) 3 4 23
  3. Structure of a Ran-binding domain complexed with Ran bound to a GTP analogue: implications for nuclear transport. (PMID: 10078529) Vetter I.R. … Wittinghofer A. (Nature 1999) 3 4 23
  4. The nucleoporin RanBP2 has SUMO1 E3 ligase activity. (PMID: 11792325) Pichler A. … Melchior F. (Cell 2002) 3 4 23
  5. Unique binding interactions among Ubc9, SUMO and RanBP2 reveal a mechanism for SUMO paralog selection. (PMID: 15608651) Tatham M.H. … Hay R.T. (Nat. Struct. Mol. Biol. 2005) 3 4 23

Products for RANBP2 Gene

Sources for RANBP2 Gene

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