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Symbol NUP98 contributors: mct/pgu - updated : 05-06-2015
HGNC name nucleoporin 98kDa
HGNC id 8068
Corresponding disease
AMLT2 acute myeloid leukemia, with limited differentiation (FAB M2 and M4)
Location 11p15.4      Physical location : 3.696.240 - 3.819.022
Synonym name
  • GLFG-repeat containing nucleoporin
  • nuclear pore complex protein Nup98-Nup96
  • Synonym symbol(s) ADIR2, NUP196, HXAD, NU98, Nup98-Nup96, ADAR2, NUP96
    TYPE functioning gene
    STRUCTURE 126.71 kb     33 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Physical map
    CDKN1C 11p15.5 cyclin-dependent kinase inhibitor 1C (p57, Kip2) HSA404617 11p15.5 BWRT protein SLC22A1LS 11p15.5 solute carrier family 22 (organic cation transporter), member 1-like antisense SLC22A1L 11p15.5 solute carrier family 22 (organic cation transporter), member 1-like PHLDA2 11p15.5 pleckstrin homology-like domain, family A, member 2 NAP1L4 11p15.5 nucleosome assembly protein 1-like 4 PRO0943 11p15.5 hypothetical protein PRO0943 CARS 11p15.5 cysteinyl-tRNA synthetase OSBPL5 11p15.4 oxysterol binding protein-like 5 FLJ36102 11p15.5 hypothetical protein FLJ36102 MRGE 11p15.5 mas-related G protein-coupled MRGE ZNF195 11p15.5 zinc finger protein 195 OR7E12P 11p15.5 olfactory receptor, family 7, subfamily E, member 12 pseudogene LOC387744 11 similar to asparagine-linked glycosylation 1 homolog (yeast, beta-1,4-mannosyltransferase); beta-1,4 mannosyltransferase LOC387745 11 similar to hypothetical protein SB153 LOC387746 11 LOC387746 LOC341141 11p15.5 similar to seven transmembrane helix receptor TRPC2 11p15.4-p15.3 transient receptor potential cation channel, subfamily C, member 2 ART5 11p15.5 ADP-ribosyltransferase 5 ART1 11p15.5 ADP-ribosyltransferase 1 CHRNA10 11p15.5 cholinergic receptor, nicotinic, alpha polypeptide 10 NUP98 11p15.5 nucleoporin 98kDa FRAG1 11p15.5 nucleoporin 98kDa ARHG 11p15.5-p15.4 ras homolog gene family, member G (rho G) STIM1 11p15.5 stromal interaction molecule 1 RRM1 11p15.5 ribonucleotide reductase M1 polypeptide LOC390030 11 similar to olfactory receptor GA_x6K02T2PBJ9-5199377-5198367 LOC340977 11p15.4 similar to Chain A, Crystal Structure Of The Radixin Ferm Domain Complexed With Inositol-(1,4,5)-Triphosphate LOC196120 11p15.4 similar to RIKEN cDNA 1500011L16 LOC390031 11 similar to RIKEN cDNA 1500011L16 LOC256875 11p15.4 similar to RIKEN cDNA 1500011L16 LOC390032 11 similar to RIKEN cDNA 1500011L16 LOC143506 11p15.4 similar to RIKEN cDNA 1500011L16 LOC390033 11 similar to RIKEN cDNA 1500011L16 LOC143496 11p15.4 similar to Olfactory receptor 52B4 LOC387747 11 similar to seven transmembrane helix receptor SSA1 11p15.5 Sjogren syndrome antigen A1 (52kDa, ribonucleoprotein autoantigen SS-A/Ro) LOC390034 11 similar to olfactory receptor MOR16-1 LOC119775 11p15.4 similar to seven transmembrane helix receptor LOC119774 11p15.4 similar to Olfactory receptor 52K2
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    20 - 3908 96.1 920 - 2010 20237156
    32 splicing 6758 - 1726 - 2010 20237156
    33 splicing 6980 195.8 1800 - 2010 20237156
    20 splicing 3959 97.8 937 - 2010 20237156
    Type widely
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrinepancreas   highly
    Lymphoid/Immunelymph node   highly
     thymus   moderately
    Reproductivefemale systemuterus  highly
     male systemtestis  highly
    Respiratorylung   highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
  • N terminal docking site, which contains the nucleoporin FG/GLFG repeat motifs , a GLFG domain functioning in pore targeting through binding to NUP93, positioning the GLFG domain in the center of the nuclear pore complex and supporting a role for this nucleoporin in the permeability barrier
  • multiple FG repeats that function in nucleocytoplasmic transport
  • a Gle2-binding sequence (GLEBS), for interaction with RAE1
    interspecies homolog to C.elegans ZK328.5B
  • GLFG nucleoporin family
  • CATEGORY RNA associated , transport carrier
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,nuclear envelope,pore
    basic FUNCTION
  • multiple RNA export pathway
  • playing a role in the bidirectional transport across the nucleoporin complex
  • plays roles in several important biological events such as gene expression, mitotic checkpoint, and pathogenesis
  • nucleoporin characterized by multiple FG repeats that function in nucleocytoplasmic transport by interacting with import and export carriers
  • in germ cells, the function of NUP98 extends beyond transport at the nuclear pore (NPC) to include mRNA regulation in the cytoplasm
  • functions as a novel shuttling cofactor for XPO1-mediated nuclear export in conjunction with RANBP3
  • contribution of the nucleoporin NUP98 to mitotic spindle assembly through regulation of microtubule dynamics
  • mitotic phosphorylation of NUP98 is a rate-limiting step in mitotic NPC disassembly
  • major determinant of the permeability barrier of NPCs
  • function in regulating select TP53 target genes that is distinct from the well-characterized oncogenic properties of NUP98 fusion proteins
  • dynamically associates with the human genome during differentiation, revealing a role of a nuclear pore protein in regulating developmental gene expression programs (PMID
  • NUP98 binding to recently expressed promoters plays potentially a conserved role in promoting epigenetic transcriptional memory
    a component
  • essential component of the nuclear pore complex
  • RAE1/NUP98 complex has distinct and versatile roles at different stages of the cell cycle, including the nuclear export of mRNAs and the regulation of the cell cycle progression
    small molecule
  • KPNB2 (can dissociate from its substrate)
  • interaction with RAE1
  • only two of more than 20 nuclear pore core structure components, namely NUP93 and NUP98, are caspase targets
  • NUP98-IQCG fusion protein may play an essential role in leukemogenesis, but by itself may not be sufficient to induce leukemia
  • predominantly interacted with transcriptionally active genes inside the nucleoplasm, in particular those involved in developmental regulation and the cell cycle
  • combined action of CDK1, NEK6, NEK7, and likely other kinases contributes to hyperphosphorylation of NUP98 during mitosis
  • specifically regulates nucleus-cytoplasm transport of galectin-3, which is a beta-galactoside-binding protein affecting adhesion, migration, and cancer progression, and controls cell growth through the CTNNB1 signaling pathway in cancer cells
  • highly selective targeting of NUP98-fusion proteins to HOX cluster regions via prebound XPO1 induces the formation of higher order chromatin structures that causes aberrant HOX gene regulation (pMID: 26740045)
  • cell & other
    Other its phosphorylation presents a crucial step during nuclear pore complexes disassembly
    corresponding disease(s) AMLT2
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral fusion      
    t(6;11)(q24.1;p15.5) resulting in a NUP98-CCDC28A fusion in acute megakaryoblastic leukemia
    tumoral fusion      
    fused with HOXD11 in t(5;11)(q35;p15.5) in acute myeloid leukemia
    tumoral fusion      
    fused with TOP1 in t(11;20)(p15;q11) in therapy related myelodysplastic syndrome, and with HOXA13 int (7;11) (p15;p15)
    tumoral fusion      
    fused with WHSC1L1 in t(8;11) (p11.2-p15) in acute myeloid leukemia, with ADD3 in t(10;11)(q25;p15) in T-cell acute lymphoblastic leukemia
    tumoral fusion      
    with DDX10 in de novo or therapy related myeloid malignancies,with HOXD13 or FN1 in translocation t(2;11) (q35;p15) or t(2;11) (q31;p15) in acute myelomonocytic leukemia with inv11(p15-q22)
    tumoral fusion      
    with PMX1 in translocation t(1;11) (q23;p15),with RAP1GDS1 in t(4;11)(q21;p15) in T cell acute lymphocitic leukemia,with PSIP1 in t (9;11) (p22;p15) in acute myeloid leukemia
    tumoral fusion      
    to KDM5A in acute lymphoblastic leukemia
    tumoral     --over  
    of NUP98-HOXA9 represses myeloid-specific gene transcription, thereby contributing to differentiation block in leukemogenesis
    tumoral fusion      
    NUP98-HOXD13 (NHD13) fusion in patients with myelodysplastic syndrome (MDS) and acute nonlymphocytic leukemia (ANLL)
    tumoral   translocation    
    t(3;11)(q12;p15)/NUP98-LNP1 fusion transcript in acute myeloid leukemia
    tumoral fusion      
    NUP98/NSD1 identifies a previously unrecognized group of young acute myeloid leukemia with a distinct HOX gene expression pattern
    tumoral fusion      
    POU1F1 is a fusion partner of NUP98 in acute myeloid leukemia with t(3;11)(p11;p15) (
    tumoral fusion      
    of NUP98 to KDM5A (t(11;15)(p15;q35)in acute megakaryoblastic leukemia (AMKL)
    Variant & Polymorphism
    Candidate gene
    Therapy target