{"entryType":"UniProtKB reviewed (Swiss-Prot)","primaryAccession":"P52948","secondaryAccessions":["Q8IUT2","Q8WYB0","Q96E54","Q9H3Q4","Q9NT02","Q9UF57","Q9UHX0","Q9Y6J4","Q9Y6J5"],"uniProtkbId":"NUP98_HUMAN","entryAudit":{"firstPublicDate":"1996-10-01","lastAnnotationUpdateDate":"2026-09-02","lastSequenceUpdateDate":"2010-10-05","entryVersion":246,"sequenceVersion":4},"annotationScore":5.0,"organism":{"scientificName":"Homo sapiens","commonName":"Human","taxonId":9606,"lineage":["Eukaryota","Metazoa","Chordata","Craniata","Vertebrata","Euteleostomi","Mammalia","Eutheria","Euarchontoglires","Primates","Haplorrhini","Catarrhini","Hominidae","Homo"]},"proteinExistence":"1: Evidence at protein level","proteinDescription":{"recommendedName":{"fullName":{"evidences":[{"evidenceCode":"ECO:0000305"}],"value":"Nuclear pore complex protein Nup98-Nup96"},"ecNumbers":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18287282"}],"value":"3.4.21.-"}]},"contains":[{"recommendedName":{"fullName":{"value":"Nuclear pore complex protein Nup98"}},"alternativeNames":[{"fullName":{"value":"98 kDa nucleoporin"}},{"fullName":{"value":"Nucleoporin Nup98"},"shortNames":[{"value":"Nup98"}]}]},{"recommendedName":{"fullName":{"value":"Nuclear pore complex protein Nup96"}},"alternativeNames":[{"fullName":{"value":"96 kDa nucleoporin"}},{"fullName":{"value":"Nucleoporin Nup96"},"shortNames":[{"value":"Nup96"}]}]}],"flag":"Precursor"},"genes":[{"geneName":{"evidences":[{"evidenceCode":"ECO:0000312","source":"HGNC","id":"HGNC:8068"}],"value":"NUP98"},"synonyms":[{"value":"ADAR2"}]}],"comments":[{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15229283"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33097660"}],"value":"Plays a role in the nuclear pore complex (NPC) assembly and/or maintenance. NUP98 and NUP96 are involved in the bidirectional transport across the NPC (PubMed:33097660). May anchor NUP153 and TPR to the NPC. In cooperation with DHX9, plays a role in transcription and alternative splicing activation of a subset of genes (PubMed:28221134). Involved in the localization of DHX9 in discrete intranuclear foci (GLFG-body) (PubMed:28221134)"}],"commentType":"FUNCTION"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23523133"}],"value":"(Microbial infection) Interacts with HIV-1 capsid protein P24 and nucleocapsid protein P7 and may thereby promote the integration of the virus in the host nucleus (in vitro) (PubMed:23523133). Binding affinity to HIV-1 CA-NC complexes bearing the capsid change Asn-74-Asp is reduced (in vitro) (PubMed:23523133)"}],"commentType":"FUNCTION"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000250","source":"UniProtKB","id":"Q6PFD9"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10209021"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11684705"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15229283"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18287282"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20498086"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"28221134"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"30543681"}],"value":"Part of the nuclear pore complex (NPC) (PubMed:15229283, PubMed:18287282). Interacts directly with NUP96 (PubMed:12191480). Part of the Nup160 subcomplex in the nuclear pore which is composed of NUP160, NUP133, NUP107 and NUP96; this complex plays a role in RNA export and in tethering NUP98 and NUP153 to the nucleus (PubMed:11684705). Interacts with RAE1 (PubMed:10209021, PubMed:20498086). Does not interact with TPR (PubMed:11684705). Interacts with NUP88 (PubMed:30543681). Interacts directly with NUP88 and NUP214, subunits of the cytoplasmic filaments of the NPC (By similarity). Interacts (via N-terminus) with DHX9 (via DRBM, OB-fold and RGG domains); this interaction occurs in a RNA-dependent manner and stimulates DHX9-mediated ATPase activity (PubMed:28221134)"}],"commentType":"SUBUNIT"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23523133"}],"value":"(Microbial infection) Interacts with HIV-1 capsid protein P24 and nucleocapsid protein P7 (in vitro); the interaction may promote the integration of the virus in the host nucleus (in vitro)"}],"commentType":"SUBUNIT"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11106761"}],"value":"(Microbial infection) Interacts with vesicular stomatitis virus protein M (PubMed:11106761)"}],"commentType":"SUBUNIT"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33097660"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33360543"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33849972"}],"value":"(Microbial infection) Interacts with SARS coronavirus-2/SARS-CoV-2 ORF6 protein; the interaction blocks STAT1 nuclear translocation, antagonizes interferon signaling and blocks mRNA nuclear export (ex vivo)"}],"commentType":"SUBUNIT"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"34163069"}],"value":"(Microbial infection) Interacts with SARS coronavirus/SARS-CoV ORF6 protein"}],"commentType":"SUBUNIT"},{"commentType":"INTERACTION","interactions":[{"interactantOne":{"uniProtKBAccession":"P52948","intActId":"EBI-295727"},"interactantTwo":{"uniProtKBAccession":"P78406","geneName":"RAE1","intActId":"EBI-724495"},"numberOfExperiments":12,"organismDiffer":false},{"interactantOne":{"uniProtKBAccession":"P52948","intActId":"EBI-295727"},"interactantTwo":{"uniProtKBAccession":"P0DTC6","geneName":"6","intActId":"EBI-25475897"},"numberOfExperiments":11,"organismDiffer":true},{"interactantOne":{"uniProtKBAccession":"P52948","intActId":"EBI-295727"},"interactantTwo":{"uniProtKBAccession":"P59634","geneName":"6","intActId":"EBI-25489038"},"numberOfExperiments":6,"organismDiffer":true}]},{"commentType":"SUBCELLULAR LOCATION","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11106761"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11839768"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12802065"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"28221134"}],"value":"Localized to the nucleoplasmic side of the nuclear pore complex (NPC), at or near the nucleoplasmic basket (PubMed:11839768). Dissociates from the disassembled NPC structure early during prophase of mitosis (PubMed:12802065). Colocalized with NUP153 and TPR to the nuclear basket of NPC (PubMed:11839768). Colocalized with DHX9 in diffuse and discrete intranuclear foci (GLFG-body) (PubMed:11839768, PubMed:28221134)"}]},"subcellularLocations":[{"location":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10087256"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11106761"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11839768"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12802065"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15229283"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20407419"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"28221134"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33360543"}],"value":"Nucleus membrane","id":"SL-0182"},"topology":{"value":"Peripheral membrane protein","id":"SL-9903"},"orientation":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11839768"}],"value":"Nucleoplasmic side","id":"SL-9915"}},{"location":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11839768"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12802065"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"15229283"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"33097660"}],"value":"Nucleus, nuclear pore complex","id":"SL-0185"}},{"location":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"28221134"}],"value":"Nucleus, nucleoplasm","id":"SL-0190"}}]},{"commentType":"SUBCELLULAR LOCATION","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11106761"}],"value":"(Microbial infection) Remains localized to the nuclear membrane after poliovirus (PV) infection"}]},"subcellularLocations":[{"location":{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"11106761"}],"value":"Nucleus membrane","id":"SL-0182"}}]},{"commentType":"ALTERNATIVE PRODUCTS","events":["Alternative splicing"],"isoforms":[{"name":{"value":"1"},"synonyms":[{"value":"Nup98-Nup96 precursor"}],"isoformIds":["P52948-1"],"isoformSequenceStatus":"Displayed"},{"name":{"value":"2"},"synonyms":[{"value":"Nup98-Nup96 precursor splice variant 1"}],"isoformIds":["P52948-2"],"sequenceIds":["VSP_003619","VSP_007944"],"isoformSequenceStatus":"Described"},{"name":{"value":"3"},"synonyms":[{"value":"Nup98-specific 1"}],"isoformIds":["P52948-3"],"sequenceIds":["VSP_007942","VSP_007943"],"isoformSequenceStatus":"Described"},{"name":{"value":"4"},"isoformIds":["P52948-4"],"sequenceIds":["VSP_003619","VSP_007942","VSP_007943"],"isoformSequenceStatus":"Described"},{"name":{"value":"5"},"synonyms":[{"value":"Nup196"},{"value":"ADIR2"}],"isoformIds":["P52948-5"],"sequenceIds":["VSP_003619"],"isoformSequenceStatus":"Described"},{"name":{"value":"6"},"isoformIds":["P52948-6"],"sequenceIds":["VSP_038328"],"isoformSequenceStatus":"Described"}]},{"texts":[{"value":"Contains G-L-F-G repeats. The FG repeat domains in Nup98 have a direct role in the transport"}],"commentType":"DOMAIN"},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10087256"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18287282"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"20407419"}],"value":"Isoform 1 to isoform 4 are autoproteolytically cleaved to yield Nup98 and Nup96 or Nup98 only, respectively (PubMed:10087256, PubMed:12191480, PubMed:18287282, PubMed:20407419). Cleaved Nup98 is necessary for the targeting of Nup98 to the nuclear pore and the interaction with Nup96 (PubMed:12191480, PubMed:20407419)"}],"commentType":"PTM"},{"texts":[{"value":"Proteolytically degraded after poliovirus (PV) infection; degradation is partial and NCP- and TPR-binding domains withstand degradation"}],"commentType":"PTM"},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"17287853"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"34163069"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"8563753"}],"value":"Chromosomal aberrations involving NUP98 have been found in acute myeloid leukemia. Translocation t(7;11)(p15;p15) with HOXA9 (PubMed:8563753). The chimera includes NUP98 intrinsic disordered regions which contribute to aberrant liquid-liquid phase separation puncta of the chimera in the nucleus. This phase-separation enhances the chimera genomic targeting and induces organization of aberrant three-dimensional chromatin structures leading to tumorous transformation (PubMed:34163069). Translocation t(11;17)(p15;p13) with PHF23 (PubMed:17287853)"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16419055"}],"value":"A chromosomal aberration involving NUP98 has been found in M0 type acute myeloid leukemia. Translocation t(4;11)(q23;p15) with RAP1GDS1"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10477737"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10929031"}],"value":"A chromosomal aberration involving NUP98 has been found in T-cell acute lymphocytic leukemia. Translocation t(4;11)(q23;p15) with RAP1GDS1"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23531517"}],"value":"A chromosomal aberration involving NUP98 has been found in M5 type acute myeloid leukemia. Translocation t(11;12)(p15;p13) with KDM5A"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16028218"}],"value":"Chromosomal aberrations involving NUP98 have been found in childhood acute myeloid leukemia. Translocation t(5;11)(q35;p15.5) with NSD1. Translocation t(8;11)(p11.2;p15) with WHSC1L1"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16419055"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"23531517"}],"value":"Chromosomal aberrations involving NUP98 have been found in M7 type childhood acute myeloid leukemia. Translocation t(11;12)(p15;p13) with KDM5A"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16028218"}],"value":"A chromosomal aberration involving NUP98 is found in a form of therapy-related myelodysplastic syndrome. Translocation t(11;20)(p15;q11) with TOP1"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16028218"}],"value":"A chromosomal aberration involving NUP98 is found in a form of T-cell acute lymphoblastic leukemia (T-ALL). Translocation t(3;11)(q12.2;p15.4) with LNP1"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16028218"}],"value":"A chromosomal aberration involving NUP98 is associated with pediatric acute myeloid leukemia (AML) with intermediate characteristics between M2-M3 French-American-British (FAB) subtypes. Translocation t(9;11)(p22;p15) with PSIP1/LEDGF. The chimeric transcript is an in-frame fusion of NUP98 exon 8 to PSIP1/LEDGF exon 4"}]}},{"commentType":"DISEASE","note":{"texts":[{"evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16028218"}],"value":"A chromosomal aberration involving NUP98 has been identified in acute leukemias. Translocation t(6;11)(q24.1;p15.5) with CCDC28A. The chimeric transcript is an in-frame fusion of NUP98 exon 13 to CCDC28A exon 2. Ectopic expression of NUP98-CCDC28A in mouse promotes the proliferative capacity and self-renewal potential of hematopoietic progenitors and rapidly induced fatal myeloproliferative neoplasms and defects in the differentiation of the erythro-megakaryocytic lineage"}]}},{"texts":[{"evidences":[{"evidenceCode":"ECO:0000305"}],"value":"Belongs to the nucleoporin GLFG family"}],"commentType":"SIMILARITY"},{"commentType":"SEQUENCE CAUTION","sequenceCautionType":"Miscellaneous discrepancy","sequence":"AAD22395.1","note":"Contaminating sequence. Sequence of unknown origin in the C-terminal part.","evidences":[{"evidenceCode":"ECO:0000305"}]},{"commentType":"SEQUENCE CAUTION","sequenceCautionType":"Miscellaneous discrepancy","sequence":"AAD22396.1","note":"Contaminating sequence. Sequence of unknown origin in the C-terminal part.","evidences":[{"evidenceCode":"ECO:0000305"}]},{"commentType":"SEQUENCE CAUTION","sequenceCautionType":"Erroneous initiation","sequence":"AAF19342.1","note":"Truncated N-terminus.","evidences":[{"evidenceCode":"ECO:0000305"}]},{"commentType":"SEQUENCE CAUTION","sequenceCautionType":"Frameshift","sequence":"AAF19342.1","evidences":[{"evidenceCode":"ECO:0000305"}]},{"commentType":"WEB RESOURCE","resourceName":"Atlas of Genetics and Cytogenetics in Oncology and Haematology","resourceUrl":"https://atlasgeneticsoncology.org/gene/98/NUP","ftp":false}],"features":[{"type":"Chain","location":{"start":{"value":1,"modifier":"EXACT"},"end":{"value":880,"modifier":"EXACT"}},"description":"Nuclear pore complex protein Nup98","featureId":"PRO_0000019929"},{"type":"Chain","location":{"start":{"value":881,"modifier":"EXACT"},"end":{"value":1817,"modifier":"EXACT"}},"description":"Nuclear pore complex protein Nup96","featureId":"PRO_0000019930"},{"type":"Domain","location":{"start":{"value":738,"modifier":"EXACT"},"end":{"value":880,"modifier":"EXACT"}},"description":"Peptidase S59","evidences":[{"evidenceCode":"ECO:0000255","source":"PROSITE-ProRule","id":"PRU00765"}]},{"type":"Region","location":{"start":{"value":1,"modifier":"EXACT"},"end":{"value":156,"modifier":"EXACT"}},"description":"FG repeats 1"},{"type":"Region","location":{"start":{"value":157,"modifier":"EXACT"},"end":{"value":213,"modifier":"EXACT"}},"description":"GLEBS; interaction with RAE1","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10209021"}]},{"type":"Region","location":{"start":{"value":214,"modifier":"EXACT"},"end":{"value":480,"modifier":"EXACT"}},"description":"FG repeats 2"},{"type":"Region","location":{"start":{"value":512,"modifier":"EXACT"},"end":{"value":535,"modifier":"EXACT"}},"description":"Disordered","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Region","location":{"start":{"value":614,"modifier":"EXACT"},"end":{"value":633,"modifier":"EXACT"}},"description":"Disordered","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Region","location":{"start":{"value":662,"modifier":"EXACT"},"end":{"value":682,"modifier":"EXACT"}},"description":"Disordered","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Region","location":{"start":{"value":886,"modifier":"EXACT"},"end":{"value":937,"modifier":"EXACT"}},"description":"Disordered","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Compositional bias","location":{"start":{"value":525,"modifier":"EXACT"},"end":{"value":534,"modifier":"EXACT"}},"description":"Basic and acidic residues","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Compositional bias","location":{"start":{"value":670,"modifier":"EXACT"},"end":{"value":682,"modifier":"EXACT"}},"description":"Polar residues","evidences":[{"evidenceCode":"ECO:0000256","source":"SAM","id":"MobiDB-lite"}]},{"type":"Active site","location":{"start":{"value":881,"modifier":"EXACT"},"end":{"value":881,"modifier":"EXACT"}},"description":"Nucleophile","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18287282"}]},{"type":"Site","location":{"start":{"value":391,"modifier":"EXACT"},"end":{"value":392,"modifier":"EXACT"}},"description":"Breakpoint for translocation to form the NUP98-RAP1GDS1 fusion protein. Breakpoint for translocation to form the NUP98-RAP1GDS1 fusion protein","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10477737"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16419055"}]},{"type":"Site","location":{"start":{"value":486,"modifier":"EXACT"},"end":{"value":487,"modifier":"EXACT"}},"description":"Breakpoint for translocation to form the NUP98-HOXA9 fusion protein. Breakpoint for translocation to form the NUP98-RAP1GDS1 fusion protein","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10477737"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"8563753"}]},{"type":"Site","location":{"start":{"value":531,"modifier":"EXACT"},"end":{"value":532,"modifier":"EXACT"}},"description":"Breakpoint for translocation to form NUP98-CCDC28A"},{"type":"Site","location":{"start":{"value":531,"modifier":"EXACT"},"end":{"value":532,"modifier":"EXACT"}},"description":"Breakpoint for translocation to form NUP98-PHF23 oncogene"},{"type":"Site","location":{"start":{"value":531,"modifier":"EXACT"},"end":{"value":532,"modifier":"EXACT"}},"description":"Breakpoint for translocation to form the NUP98-KDM5A fusion protein","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"16419055"}]},{"type":"Site","location":{"start":{"value":880,"modifier":"EXACT"},"end":{"value":881,"modifier":"EXACT"}},"description":"Cleavage; by autolysis","evidences":[{"evidenceCode":"ECO:0000269","source":"PubMed","id":"10087256"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"12191480"},{"evidenceCode":"ECO:0000269","source":"PubMed","id":"18287282"}]},{"type":"Modified residue","location":{"start":{"value":524,"modifier":"EXACT"},"end":{"value":524,"modifier":"EXACT"}},"description":"Phosphoserine","evidences":[{"evidenceCode":"ECO:0007744","source":"PubMed","id":"20068231"}]},{"type":"Modified residue","location":{"start":{"value":603,"modifier":"EXACT"},"end":{"value":603,"modifier":"EXACT"}},"description":"N6-acetyllysine; alternate","evidences":[{"evidenceCode":"ECO:0007744","source":"PubMed","id":"19608861"}]},{"type":"Modified 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