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RANBP2 (RAN binding protein)

Identity

Other namesNUP358 (nucleoporin 358 kDa)
HGNC (Hugo) RANBP2
Location 2q13

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
  

External links

Nomenclature
HGNC (Hugo)RANBP2   9848
Entrez_Gene (NCBI)RANBP2  5903  RAN binding protein 2
Cards
AtlasRANBP2ID483
GeneCards (Weizmann)RANBP2
Ensembl (Hinxton)ENSG00000153201 [Gene_View]  RANBP2 [Vega]
AceView (NCBI)RANBP2
Genatlas (Paris)RANBP2
euGene (Indiana)5903
SOURCE (Stanford)NM_006267
Gene Expression (Array Express) ENSG00000153201
Genomic and cartography
GoldenPath (UCSC)RANBP2  -  2q13
EnsemblRANBP2 - [CytoView]
Mapping of homologs : NCBIRANBP2 [Mapview]
OMIM601181   608033   
Gene and transcription
Gene : Genbank (Entrez)AA971491 AB209483 AK025462 AK025711 AK026993
Reference sequence (RefSeq transcript) :SRSNM_006267
Reference transcript : EntrezNM_006267
RefSeq genomic : SRSAC_000045 AC_000134 NC_000002 NG_012210 NT_022171 NW_001838822 NW_921374
RefSeq genomic : EntrezAC_000045 AC_000134 NC_000002 NG_012210 NT_022171 NW_001838822 NW_921374
Consensus coding sequences : CCDS NCBIRANBP2
Cluster EST : UnigeneHs.715056 [ SRS ] Hs.715056 [ NCBI ]
Alternative Splicing : Fast-db (Paris)9351
Protein : pattern, domain, 3D structure
Protein : UniProt/SwissProtP49792 (SRS) P49792 (Expasy) P49792 (Uniprot)
With graphics : InterProP49792
Splice isoforms : VarSplice FASTAP49792(VarSplice FASTA)
Domaine pattern : Prosite (SRS)CSA_PPIASE_1 (PS00170)    CSA_PPIASE_2 (PS50072)    RANBD1 (PS50196)    TPR (PS50005)    TPR_REGION (PS50293)    ZF_RANBP2_1 (PS01358)    ZF_RANBP2_2 (PS50199)   
Domain 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 (SRS)Cyclophilin-like    IR1-M    Pep-Pro_Isoase_cyclophilin_CS    PH_type    PPIase_cyclophilin    RanBP    TPR-1    TPR-contain    TPR-like_helical    TPR_repeat    Znf_RanBP2   
Domains : Interpro (EBI)Cyclophilin-like    IR1-M    Pep-Pro_Isoase_cyclophilin_CS    PH_type    PPIase_cyclophilin    RanBP    TPR-1    TPR-contain    TPR-like_helical    TPR_repeat    Znf_RanBP2   
Related proteins : CluSTrP49792
Domain families : Pfam SRSIR1-M (PF12185)    Pro_isomerase (PF00160)    Ran_BP1 (PF00638)    TPR_1 (PF00515)    zf-RanBP (PF00641)   
Domain families : Pfam SangerIR1-M (PF12185)    Pro_isomerase (PF00160)    Ran_BP1 (PF00638)    TPR_1 (PF00515)    zf-RanBP (PF00641)   
Domain families : Pfam NCBIpfam12185    pfam00160    pfam00638    pfam00515    pfam00641   
Domain families : Smart EMBLRanBD (SM00160)  ZnF_RBZ (SM00547)  
Blocks (Seattle)P49792
Crystal structure of protein : PDB SRS1RRP    1XKE    1Z5S   
Crystal structure of protein : PDBSum1RRP    1XKE    1Z5S   
Crystal structure of protein : IMB1RRP    1XKE    1Z5S   
Crystal structure of protein : PDB RSDB1RRP    1XKE    1Z5S   
Human Protein AtlasENSG00000153201
HPRD03111
Protein Interaction databases
DIP (DOE-UCLA)P49792
IntAct (EBI)P49792
FunCoupENSG00000153201
Polymorphism : SNP, mutations, diseases
Single Nucleotide Polymorphism (SNP) : dbSNP NCBIRANBP2
SNP : GeneSNP UtahRANBP2
SNP : HGBaseRANBP2
Genetic variants : HAPMAPRANBP2
Somatic Mutations in Cancer : COSMICRANBP2 
Rearrangement : COSMICRANBP2 2q13  -  ALK 2p23.2
Translocation Breakpoints in Cancer : TICdbRANBP2 
Mutations and Diseases : HGMDRANBP2
Hereditary diseases : OMIM601181    608033   
Hereditary diseases : GENETests601181    608033   
Diseases : Genetic AssociationRANBP2
General knowledge
Homologs : HomoloGeneRANBP2
Homology/Alignments : Family Browser UCSCRANBP2
Phylogenetic Trees/Animal Genes : TreeFamRANBP2
Chemical/Protein Interactions : CTD5903
Keywords Ontology : AmiGOpeptidyl-prolyl cis-trans isomerase activity  protein binding  intracellular  nucleus  nuclear pore  cytosol  protein folding  protein import into nucleus  zinc ion binding  Ran GTPase binding  protein transport  isomerase activity  metal ion binding  intracellular transport  mRNA transport  transmembrane transport  
Keywords Ontology : EGO-EBIpeptidyl-prolyl cis-trans isomerase activity  protein binding  intracellular  nucleus  nuclear pore  cytosol  protein folding  protein import into nucleus  zinc ion binding  Ran GTPase binding  protein transport  isomerase activity  metal ion binding  intracellular transport  mRNA transport  transmembrane transport  
Pathways : BIOCARTAMechanism of Protein Import into the Nucleus [Genes]    Sumoylation by RanBP2 Regulates Transcriptional Repression [Genes]    Cycling of Ran in nucleocytoplasmic transport [Genes]   
Pathways : KEGG
Other databases
Probes
Probes : ImagenesRANBP2 Related clones (RZPD - Berlin)
Literature
PubMed69 Pubmed reference(s) in Entrez
PubGeneRANBP2

Bibliography

A giant nucleopore protein that binds Ran/TC4.
Yokoyama N, Hayashi N, Seki T, Pantˆ© 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, Kˆºbler 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. August 2003 .
URL : http://AtlasGeneticsOncology.org/Genes/RANBP2ID483.html

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