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FLASH GENE
Symbol RANGAP1 contributors: mct/npt - updated : 10-01-2015
HGNC name Ran GTPase activating protein 1
HGNC id 9854
Location 22q13.2      Physical location : 41.641.615 - 41.682.216
Synonym name
  • segregation distorter homolog (Drosophila)
  • segregation distortion
  • Synonym symbol(s) KIAA1835, FUG1, KIAA1835, MGC20266, SD
    DNA
    TYPE functioning gene
    STRUCTURE 41.47 kb     16 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Map cen - D22S284 /GRAP2 - D22S423 - ADSL - MCHR1 - SLC25A17 - ST13 - RBX1 - RANGAP1 - TOB2 - ACO2 - PMM1 - XRCC6 - HMG17L2 - D22S276 - SREBF2 /D22S1157 - D22S1178 - NAGA NAGA - NDUFA6 - TCF20 - DIA1 - PACSIN2 - BIK - TSPO - C22orf1 - qter
    Authors Dunham (99)
    RNA
    TRANSCRIPTS type messenger
    text a SUMO1 modified form 90kDa
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    16 - 3907 63.4 587 - Zhang (2008)
    17 - 4368 - 587 - -
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Lymphoid/Immunethymus   highly
    Nervousnerve   highly
    Respiratoryrespiratory tractlarynx  highly
    Skin/Tegumentskin   predominantly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • six leucine rich repeats (LRR)
  • mono polymer homomer , dimer
    HOMOLOGY
    interspecies homolog to yeast rnap1
    homolog to murine Fug1
    Homologene
    FAMILY
  • RNA1 family
  • CATEGORY enzyme , regulatory , signaling , transport
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,mitotic spindle
    intracellular,nucleus
    intracellular,nuclear envelope,pore
    text
  • highly concentrated at its cytoplasmic periphery
  • modified form 90kd associated with the cytoplasmic fibers of NPC and mitotic spindle
  • basic FUNCTION
  • Ran GTPase activating protein 1, regulator of the Ran GTP/GDP cycle
  • required for various nuclear events including the bi-directional transport of proteins and ribonucleoproteins through the nuclear pore complex, spindle formation, and reassembly of the nuclear envelope
  • Ran specific GTPase activating protein
  • Ran GTPase-activating protein that plays a critical role in nuclear transport, was the first identified substrate for SUMO modification
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS cellular trafficking transport
    text bidirectional nucleocytoplasmic transport of macromolecular
    PATHWAY
    metabolism
    signaling
    a component
  • SUMO1 modified form of RANGAP1 constituent of the nuclear pore complex with RANBP2
  • forming a trimeric complex with RANBP1,RANBP7 or RANBP8
  • RANBP2 forms a stable complex with SUMO-modified RANGAP1 and UBE2I at the nuclear pore complex
  • KPNB1 interacts through its central region with NUP358/RANBP2, which in turn binds SUMO-conjugated RANGAP1 in nuclear pores
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • RANBP2
  • conjugation with UBC9
  • inhibitor of RCC1
  • interacting with RAN and RANBP1 (phosphorylation of RANGAP1 stabilizes its interaction with RAN and RANBP1) (Takeda 2005)
  • SUMO1-modified RANGAP1 forms a more stable, higher affinity complex with the nucleoporin RANBP2 that preferentially protects it from isopeptidases
  • RANBP2 forms a stable complex with SUMO-modified RANGAP1 and UBE2I at the nuclear pore complex
  • cellular RANBP2 is quantitatively associated with RANGAP1, indicating that complexed rather than free RANBP2 is the relevant E3 ligase
  • RANGAP1 is actively transported between the nuclear and cytoplasmic compartments, and the cytoplasmic and nuclear pore complex (NPC) localization of RANGAP1 is dependent on XPO1-mediated nuclear export
  • 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)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in NPM-ALK positive anaplastic large cells lymphoma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
  • promising diagnostic marker for aggressive B-cell lymphoma, especially diffuse large B-cell lymphoma (DLBCL)
  • Therapy target
    SystemTypeDisorderPubmed
    cancerhemopathy 
    promising therapeutic target for aggressive B-cell lymphoma, especially diffuse large B-cell lymphoma (DLBCL)
    ANIMAL & CELL MODELS