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FLASH GENE
Symbol RANBP2 contributors: mct - updated : 02-07-2016
HGNC name RAN binding protein 2
HGNC id 9848
Corresponding disease
ANE1 acute necrotizing encephalopathy 1
Location 2q12.3      Physical location : 109.335.936 - 109.402.266
Synonym name
  • nuclear pore complex protein NUP358
  • nucleoporin 358
  • transformation-related protein 2
  • E3 SUMO-protein ligase RanBP2
  • Synonym symbol(s) NUP358, P270, RBP2, TRP1, TRP2, ANE1, IIAE3
    DNA
    TYPE functioning gene
    SPECIAL FEATURE component of a cluster
    STRUCTURE 66.33 kb     29 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Physical map
    LOC391415 2 similar to ribosomal protein S21; 40S ribosomal protein S21 LOC391416 2 similar to hypothetical protein MGC4614 LOC389016 2 similar to RAN-binding protein 2-like 1 isoform 1; sperm membrane protein BS-63; RAN-binding protein 2-like 1 LOC391417 2 similar to anaphase-promoting complex 1 (meiotic checkpoint regulator) CD8B2 2q12 CD8 antigen, beta polypeptide 2, pseudogene (p37) LOC389017 2 similar to Elongation factor 1-alpha 1 (EF-1-alpha-1) (Elongation factor 1 A-1) (eEF1A-1) (Elongation factor Tu) (EF-Tu) LOC389018 2 similar to Elongation factor 1-alpha 1 (EF-1-alpha-1) (Elongation factor 1 A-1) (eEF1A-1) (Elongation factor Tu) (EF-Tu) ST6GalII 2q11.2-q12.1 beta-galactoside alpha-2,6-sialyltransferase II LOC339819 2q12.3 similar to protein phosphatase 1, regulatory (inhibitor) subunit 2 DKFZp686P0288 2q12.3 similar to Ran-binding protein 2 SLC5A7 2q12 solute carrier family 5 (choline transporter), member 7 LOC391418 2 similar to phenol sulfotransferase SULT1C1 2q11.1-q11.2 sulfotransferase family, cytosolic, 1C, member 1 LOC391419 2 similar to sulfotransferase 1C SULT1C2 2q11.2 sulfotransferase family, cytosolic, 1C, member 2 GCC2 2q12-q13 GRIP and coiled-coil domain-containing 2 MGC10701 2q12.3 hypothetical protein MGC10701 LIMS1 2q12 LIM and senescent cell antigen-like domains 1 LOC391420 2 similar to 60S ribosomal protein L10 (QM protein) (Tumor suppressor QM) (Laminin receptor homolog) RANBP2 2q11-q13 RAN binding protein 2 FLJ32745 2q12.3 hypothetical protein FLJ32745 EDAR 2q11-q13 ectodysplasin 1, anhidrotic receptor LOC389019 2 LOC389019 FLJ20019 2q13 hypothetical protein FLJ20019 FLJ00204 2q13 FLJ00204 protein SEPT10 2q13 septin 10 FLJ21870 2q13 hypothetical protein FLJ21870 LOC391421 2 similar to Ribosome biogenesis protein BMS1 homolog LOC391422 2 similar to ribosomal protein L22 RANBP2L1 2q12.3 RAN binding protein 2-like 1 LOC339692 2q13 hypothetical protein LOC339692 GPAA1P1 2q12-q14 glycosylphosphatidylinositol anchor attachment 1 pseudogene 1 LOC391423 2 similar to zinc finger protein 499 LOC343938 2q13 similar to zinc finger protein BENE 2q13 BENE protein NPHP1 2q12-q13 nephronophthisis 1 (juvenile) LOC205251 2q13 hypothetical protein LOC205251 FLJ38359 2q13 hypothetical protein FLJ38359 LOC96626 2q14-q21 pinch-2 LOC391424 2 similar to ribosomal protein L22
    regionally located located in a hot spot of recombination on chromosome 2q, 3Mb distal to RANBP2L1
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    29 - 11711 - 3224 - - PMID: 178879
    EXPRESSION
    Type restricted
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestivestomach    
    Urinarykidney    
    Visualeye     Homo sapiens
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Visualcone photoreceptor
    Visualganglion cell
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a short N-terminal RANBP2 fragment harboring the NPC-binding domain, three phenylalanine-glycine motifs, and one Ran-binding domain (RBD) corrected all transport defects and restored viability
  • a leucine-rich region (LRR)
  • a TPR domain
  • four potential RAN binding sites
  • eight zinc finger motifs
  • a kinesin-binding domain (KBD)
  • an internal repeat domain (IR1-M-IR2) that catalyzes E3 ligase activity and forms a stable complex with SUMO-modified RANGAP1 and UBE2I at the nuclear pore complex
  • a C terminal cyclophylin A homologous domain and nucleoporin repeat motifs (FXFG, GLFG, XXFG)
  • HOMOLOGY
    interspecies ortholog to murine RanBP2
    intraspecies homolog to Ras-related small nuclear protein
    homolog to large scaffold cyclophilin related protein
    Homologene
    FAMILY
  • RAS superfamily
  • CATEGORY chaperone/stress , transport
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    intracellular,nucleus
    intracellular,nuclear envelope,pore
    text
  • cytoplasmic filaments of NPC
  • resides on the cytoplasmic face of the interphase nuclear pore complex
  • localizes to metaphase spindles and kinetochores in a microtubule-dependent manner
  • located on the cytoplasmic surface of the nuclear pore
  • ligase E3, RANBP2 was exclusively localized in the nucleus with homogeneous distribution in ocular cell lines
  • basic FUNCTION
  • nucleocytoplasmic transport, pre-mRNA processing, involved in cell-cycle progression, stimulator of UBL1 modification (sumoylation SUMO E3 ligase activity)
  • playing a role in regulating interphase microtubules
  • having a kinesin-dependent role in mitochondria transport and function
  • activate the ATPase activity of KIF5B approximately 30-fold in the presence of microtubules and ATP
  • during interphase, its nuclear envelope-associated functions include facilitation of protein import and export and sumoylation of protein cargoes
  • cooperates with kinesin-2 to regulate the localization of APC to the cell cortex through a nuclear-transport-independent mechanism
  • host factor that is involved in the nuclear import of HIV-1 PIC (DNA), but is not critical to the nuclear export of the viral mRNAs or nucleo-cytoplasmic shuttling of Rev
  • potentially playing a role in myogenesis
  • acts as a negative regulator of cAMP signaling through RAPGEF3, a cAMP-regulated guanine nucleotide exchange factor for Rap
  • critical function of RANBP2 is to capture recycling RANGTP-KPNB1 complexes at cytoplasmic fibrils to allow for adequate classical nuclear localization signal-mediated cargo import
  • CELLULAR PROCESS cell cycle, division, mitosis
    cell life, differentiation
    nucleotide, repair
    nucleotide, transcription, maturation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • constituent of the nuclear pore complex (NPC) with RANBP1, RANGAP1, UBE2I, exportin XPO1, importin beta, KPNB3, KIF5 motor proteins components of the 19S cap of 26S proteasome and opsin
  • RANBP2 forms a stable complex with SUMO-modified RANGAP1 and UBE2I at the nuclear pore complex
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • exportin (XPO1)
  • the E2 enzyme UBE2I (UBC9)
  • modifying HDAC4 and HDAC-1 and -6 deacetylases
  • modifying MITR
  • interacts with interphase microtubules through its N-terminal region and modulates their stability
  • through kinesin-binding domain (KBD) associated selectively in the central nervous system (CNS), and directly, with the ubiquitous and CNS-specific kinesins, KIF5B and KIF5C, respectively, but not with the highly homologous KIF5A
  • APC interacts with RANBP2, a microtubule-binding nucleoporin
  • with RAN, anchor RAPGEF3 to the nuclear pore, permitting cAMP signals to activate RAP1A at the nuclear envelope)
  • KPNB1 having functions at kinetochores exerted via RANBP2 and opposed by XPO1
  • cellular RANBP2 is quantitatively associated with RANGAP1, indicating that complexed rather than free RANBP2 is the relevant E3 ligase
  • nuclear transport receptors KPNB1 and XPO1 play essential roles in localising the RANBP2-SUMO-RANGAP1 complex away from, or at kinetochores, respectively
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) ANE1
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    translocated with ALK in t(2;2) (p23;q32) in inflammatory myofibroblastic tumor
    tumoral fusion      
    with CLTC in t(2;17)(p23;q23)in inflammatory myofibroblastic tumors
    Susceptibility to acute necrotizing encephalopathy
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    immunologyinfectious 
    could be a potential target for efficient inhibition of HIV
    ANIMAL & CELL MODELS