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RANBP2 (RAN binding protein)

Identity

Other namesNUP358 (nucleoporin 358 kDa)
HGNC (Hugo) RANBP2
LocusID (NCBI) 5903
Location 2q12.3
Location_base_pair Starts at 109335937 and ends at 109402267 bp from pter ( according to hg19-Feb_2009)  [Mapping]

Protein

Description 3224 amino acids, 358 kDa; is composed, from N-term to C-term, of: a leucine -rich region with a leucine zipper, 4 RAN-binding domains, , 8 Zn fingers, and an internal repeat region
Function nuclear export transporter; bind RAN-GTP and exportin-1

Implicated in

Entity Inflammatory myofibroblastic tumors with t(2;2)(p23;q13)
Disease rare soft tissue tumour found in children and young adults
Prognosis good prognosis
Hybrid/Mutated Gene 5' RANBP2 - 3' ALK
  

Other Leukemias implicated (Data extracted from papers in the Atlas)

Leukemias 11q23ChildAMLID1615

Other Solid tumors implicated (Data extracted from papers in the Atlas)

Solid Tumors AmeloblastomID5945 MedulloblastomaID5065 rhab5004

External links

Nomenclature
HGNC (Hugo)RANBP2   9848
Cards
AtlasRANBP2ID483
Entrez_Gene (NCBI)RANBP2  5903  RAN binding protein 2
GeneCards (Weizmann)RANBP2
Ensembl (Hinxton)ENSG00000153201 [Gene_View]  chr2:109335937-109402267 [Contig_View]  RANBP2 [Vega]
ICGC DataPortalENSG00000153201
cBioPortalRANBP2
AceView (NCBI)RANBP2
Genatlas (Paris)RANBP2
WikiGenes5903
SOURCE (Princeton)NM_006267
Genomic and cartography
GoldenPath (UCSC)RANBP2  -  2q12.3   chr2:109335937-109402267 +  2q13   [Description]    (hg19-Feb_2009)
EnsemblRANBP2 - 2q13 [CytoView]
Mapping of homologs : NCBIRANBP2 [Mapview]
OMIM601181   608033   
Gene and transcription
Genbank (Entrez)AA971491 AB209483 AK025462 AK025711 AK026993
RefSeq transcript (Entrez)NM_006267
RefSeq genomic (Entrez)AC_000134 NC_000002 NC_018913 NG_012210 NT_005403 NW_001838822 NW_004929303
Consensus coding sequences : CCDS (NCBI)RANBP2
Cluster EST : UnigeneHs.715056 [ NCBI ]
CGAP (NCI)Hs.715056
Alternative Splicing : Fast-db (Paris)GSHG0016756
Alternative Splicing GalleryENSG00000153201
Gene ExpressionRANBP2 [ NCBI-GEO ]     RANBP2 [ SEEK ]   RANBP2 [ MEM ]
Protein : pattern, domain, 3D structure
UniProt/SwissProtP49792 (Uniprot)
NextProtP49792  [Medical]
With graphics : InterProP49792
Splice isoforms : SwissVarP49792 (Swissvar)
Catalytic activity : Enzyme6.3.2.- [ Enzyme-Expasy ]   6.3.2.-6.3.2.- [ IntEnz-EBI ]   6.3.2.- [ BRENDA ]   6.3.2.- [ KEGG ]   
Domaine pattern : Prosite (Expaxy)CSA_PPIASE_1 (PS00170)    CSA_PPIASE_2 (PS50072)    RANBD1 (PS50196)    TPR (PS50005)    TPR_REGION (PS50293)    ZF_RANBP2_1 (PS01358)    ZF_RANBP2_2 (PS50199)   
Domains : Interpro (EBI)Cyclophilin-like_dom [organisation]   Cyclophilin-type_PPIase_CS [organisation]   Cyclophilin-type_PPIase_dom [organisation]   IR1-M [organisation]   PH_like_dom [organisation]   Ran_bind_dom [organisation]   TPR-contain_dom [organisation]   TPR-like_helical [organisation]   TPR_1 [organisation]   TPR_repeat [organisation]   Znf_RanBP2 [organisation]  
Related proteins : CluSTrP49792
Domain families : Pfam (Sanger)IR1-M (PF12185)    Pro_isomerase (PF00160)    Ran_BP1 (PF00638)    TPR_1 (PF00515)    zf-RanBP (PF00641)   
Domain families : Pfam (NCBI)pfam12185    pfam00160    pfam00638    pfam00515    pfam00641   
Domain families : Smart (EMBL)RanBD (SM00160)  ZnF_RBZ (SM00547)  
DMDM Disease mutations5903
Blocks (Seattle)P49792
PDB (SRS)1RRP    1XKE    1Z5S    3UIN    3UIO    3UIP    4GA0    4I9Y    4L6E    4LQW   
PDB (PDBSum)1RRP    1XKE    1Z5S    3UIN    3UIO    3UIP    4GA0    4I9Y    4L6E    4LQW   
PDB (IMB)1RRP    1XKE    1Z5S    3UIN    3UIO    3UIP    4GA0    4I9Y    4L6E    4LQW   
PDB (RSDB)1RRP    1XKE    1Z5S    3UIN    3UIO    3UIP    4GA0    4I9Y    4L6E    4LQW   
Human Protein AtlasENSG00000153201 [gene] [tissue] [antibody] [cell] [cancer]
Peptide AtlasP49792
HPRD03111
IPIIPI00221325   IPI00915960   IPI00888992   IPI00916333   
Protein Interaction databases
DIP (DOE-UCLA)P49792
IntAct (EBI)P49792
FunCoupENSG00000153201
BioGRIDRANBP2
InParanoidP49792
Interologous Interaction database P49792
IntegromeDBRANBP2
STRING (EMBL)RANBP2
Ontologies - Pathways
Ontology : AmiGOmitotic cell cycle  protein peptidyl-prolyl isomerization  RNA binding  peptidyl-prolyl cis-trans isomerase activity  protein binding  nuclear pore  mitochondrion  cytosol  carbohydrate metabolic process  protein folding  protein import into nucleus  mitotic nuclear envelope disassembly  zinc ion binding  Ran GTPase binding  hexose transport  regulation of glucose transport  glucose transport  membrane  viral process  ligase activity  protein sumoylation  protein sumoylation  cytokine-mediated signaling pathway  nuclear membrane  negative regulation of glucokinase activity  nuclear inclusion body  small molecule metabolic process  nuclear pore nuclear basket  mRNA transport  transmembrane transport  regulation of gluconeogenesis involved in cellular glucose homeostasis  
Ontology : EGO-EBImitotic cell cycle  protein peptidyl-prolyl isomerization  RNA binding  peptidyl-prolyl cis-trans isomerase activity  protein binding  nuclear pore  mitochondrion  cytosol  carbohydrate metabolic process  protein folding  protein import into nucleus  mitotic nuclear envelope disassembly  zinc ion binding  Ran GTPase binding  hexose transport  regulation of glucose transport  glucose transport  membrane  viral process  ligase activity  protein sumoylation  protein sumoylation  cytokine-mediated signaling pathway  nuclear membrane  negative regulation of glucokinase activity  nuclear inclusion body  small molecule metabolic process  nuclear pore nuclear basket  mRNA transport  transmembrane transport  regulation of gluconeogenesis involved in cellular glucose homeostasis  
Pathways : BIOCARTAMechanism of Protein Import into the Nucleus [Genes]    Sumoylation by RanBP2 Regulates Transcriptional Repression [Genes]    Cycling of Ran in nucleocytoplasmic transport [Genes]   
Pathways : KEGGRNA transport   
Protein Interaction DatabaseRANBP2
Wikipedia pathwaysRANBP2
Gene fusion - rearrangments
Rearrangement : COSMICRANBP2 [2q12.3]  -  ALK [2p23.2]  
  [COSF415] [COSF416] [COSF417] [COSF418] 
Rearrangement : TICdbRANBP2 [2q12.3]  -  ALK [9q34.13]
Polymorphisms : SNP, mutations, diseases
SNP Single Nucleotide Polymorphism (NCBI)RANBP2
snp3D : Map Gene to Disease5903
SNP (GeneSNP Utah)RANBP2
SNP : HGBaseRANBP2
Genetic variants : HAPMAPRANBP2
Exome VariantRANBP2
1000_GenomesRANBP2 
ICGC programENSG00000153201 
Somatic Mutations in Cancer : COSMICRANBP2 
CONAN: Copy Number AnalysisRANBP2 
Mutations and Diseases : HGMDRANBP2
Genomic VariantsRANBP2  RANBP2 [DGVbeta]
dbVarRANBP2
ClinVarRANBP2
Pred. of missensesPolyPhen-2  SIFT(SG)  SIFT(JCVI)  Align-GVGD  MutAssessor  Mutanalyser  
Pred. splicesGeneSplicer  Human Splicing Finder  MaxEntScan  
Diseases
OMIM601181    608033   
MedgenRANBP2
GENETestsRANBP2
Disease Genetic AssociationRANBP2
Huge Navigator RANBP2 [HugePedia]  RANBP2 [HugeCancerGEM]
General knowledge
Homologs : HomoloGeneRANBP2
Homology/Alignments : Family Browser (UCSC)RANBP2
Phylogenetic Trees/Animal Genes : TreeFamRANBP2
Chemical/Protein Interactions : CTD5903
Chemical/Pharm GKB GenePA34209
Clinical trialRANBP2
Cancer Resource (Charite)ENSG00000153201
Other databases
Probes
Litterature
PubMed146 Pubmed reference(s) in Entrez
CoreMineRANBP2
iHOPRANBP2
OncoSearchRANBP2

Bibliography

A giant nucleopore protein that binds Ran/TC4.
Yokoyama N, Hayashi N, Seki T, Pantłę N, Ohba T, Nishii K, Kuma K, Hayashida T, Miyata T, Aebi U
Nature. 1995 ; 376 (6536) : 184-188.
PMID 7603572
 
A small ubiquitin-related polypeptide involved in targeting RanGAP1 to nuclear pore complex protein RanBP2.
Mahajan R, Delphin C, Guan T, Gerace L, Melchior F
Cell. 1997 ; 88 (1) : 97-107.
PMID 9019411
 
Identification of a novel Ran binding protein 2 related gene (RANBP2L1) and detection of a gene cluster on human chromosome 2q11-q12.
Nothwang HG, Rensing C, Kł║bler M, Denich D, Brandl B, Stubanus M, Haaf T, Kurnit D, Hildebrandt F
Genomics. 1998 ; 47 (3) : 383-392.
PMID 9480752
 
Structure of a Ran-binding domain complexed with Ran bound to a GTP analogue: implications for nuclear transport.
Vetter IR, Nowak C, Nishimoto T, Kuhlmann J, Wittinghofer A
Nature. 1999 ; 398 (6722) : 39-46.
PMID 10078529
 
GTP hydrolysis links initiation and termination of nuclear import on the nucleoporin nup358.
Yaseen NR, Blobel G
The Journal of biological chemistry. 1999 ; 274 (37) : 26493-26502.
PMID 10473610
 
Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.
Ma Z, Hill DA, Collins MH, Morris SW, Sumegi J, Zhou M, Zuppan C, Bridge JA
Genes, chromosomes & cancer. 2003 ; 37 (1) : 98-105.
PMID 12661011
 
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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Contributor(s)

Written08-2003Jean-Loup Huret, Sylvie Senon

Citation

This paper should be referenced as such :
Huret, JL ; Senon, S
RANBP2 (RAN binding protein)
Atlas Genet Cytogenet Oncol Haematol. 2003;7(4):230-230.
Free online version   Free pdf version   [Bibliographic record ]
URL : http://AtlasGeneticsOncology.org/Genes/RANBP2ID483.html

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indexed on : Wed Jul 30 16:55:55 CEST 2014

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