Nucleoporin 98kDa

PDB rendering based on 1ko6.
Symbols NUP98; ADIR2; NUP196; NUP96
External IDs OMIM601021 HomoloGene35472 GeneCards: NUP98 Gene
RNA expression pattern
PBB GE NUP98 203194 s at tn.png
PBB GE NUP98 203195 s at tn.png
More reference expression data
Species Human Mouse
Entrez 4928 n/a
Ensembl ENSG00000110713 n/a
UniProt P52948 n/a
RefSeq (mRNA) NM_005387.5 n/a
RefSeq (protein) NP_005378.4 n/a
Location (UCSC) Chr 11:
3.69 – 3.82 Mb
PubMed search [1] n/a

Nuclear pore complex protein Nup98-Nup96 is a protein that in humans is encoded by the NUP98 gene.[1]

Signal-mediated nuclear import and export proceed through the nuclear pore complex (NPC), which is composed of approximately 50 unique proteins collectively known as nucleoporins. The 98 kD nucleoporin is generated through a biogenesis pathway that involves synthesis and proteolytic cleavage of a 186 kD precursor protein. This cleavage results in the 98 kD nucleoporin as well as a 96 kD nucleoporin, both of which are localized to the nucleoplasmic side of the NPC. Rat studies show that the 98 kD nucleoporin functions as one of several docking site nucleoporins of transport substrates. The human gene has been shown to fuse to several genes following chromosome translocatons in acute myelogenous leukemia (AML) and T-cell acute lymphocytic leukemia (T-ALL). This gene is one of several genes located in the imprinted gene domain of 11p15.5, an important tumor-suppressor gene region. Alterations in this region have been associated with the Beckwith-Wiedemann syndrome, Wilms tumor, rhabdomyosarcoma, adrenocortical carcinoma, and lung, ovarian, and breast cancer. Alternative splicing of this gene results in several transcript variants; however, not all variants have been fully described.[2]


NUP98 has been shown to interact with NUP88,[3] KPNB1,[4][5] RAE1,[6] CREB-binding protein[7] and TNPO2.[8]


  1. ^ Arai Y, Hosoda F, Kobayashi H, Arai K, Hayashi Y, Kamada N, Kaneko Y, Ohki M (Jun 1997). "The inv(11)(p15q22) chromosome translocation of de novo and therapy-related myeloid malignancies results in fusion of the nucleoporin gene, NUP98, with the putative RNA helicase gene, DDX10". Blood 89 (11): 3936–44. PMID 9166830. 
  2. ^ "Entrez Gene: NUP98 nucleoporin 98kDa". 
  3. ^ Griffis, Eric R; Xu Songli, Powers Maureen A (Feb. 2003). "Nup98 localizes to both nuclear and cytoplasmic sides of the nuclear pore and binds to two distinct nucleoporin subcomplexes". Mol. Biol. Cell (United States) 14 (2): 600–10. doi:10.1091/mbc.E02-09-0582. ISSN 1059-1524. PMC 149995. PMID 12589057. 
  4. ^ Moroianu, J; Blobel G, Radu A (Sep. 1997). "RanGTP-mediated nuclear export of karyopherin alpha involves its interaction with the nucleoporin Nup153". Proc. Natl. Acad. Sci. U.S.A. (UNITED STATES) 94 (18): 9699–704. doi:10.1073/pnas.94.18.9699. ISSN 0027-8424. PMC 23253. PMID 9275187. 
  5. ^ Bonifaci, N; Moroianu J, Radu A, Blobel G (May. 1997). "Karyopherin beta2 mediates nuclear import of a mRNA binding protein". Proc. Natl. Acad. Sci. U.S.A. (UNITED STATES) 94 (10): 5055–60. doi:10.1073/pnas.94.10.5055. ISSN 0027-8424. PMC 24630. PMID 9144189. 
  6. ^ Pritchard, C E; Fornerod M, Kasper L H, van Deursen J M (Apr. 1999). "RAE1 is a shuttling mRNA export factor that binds to a GLEBS-like NUP98 motif at the nuclear pore complex through multiple domains". J. Cell Biol. (UNITED STATES) 145 (2): 237–54. doi:10.1083/jcb.145.2.237. ISSN 0021-9525. PMC 2133102. PMID 10209021. 
  7. ^ Kasper, L H; Brindle P K, Schnabel C A, Pritchard C E, Cleary M L, van Deursen J M (Jan. 1999). "CREB binding protein interacts with nucleoporin-specific FG repeats that activate transcription and mediate NUP98-HOXA9 oncogenicity". Mol. Cell. Biol. (UNITED STATES) 19 (1): 764–76. ISSN 0270-7306. PMC 83933. PMID 9858599. 
  8. ^ Fontoura, B M; Blobel G, Yaseen N R (Oct. 2000). "The nucleoporin Nup98 is a site for GDP/GTP exchange on ran and termination of karyopherin beta 2-mediated nuclear import". J. Biol. Chem. (UNITED STATES) 275 (40): 31289–96. doi:10.1074/jbc.M004651200. ISSN 0021-9258. PMID 10875935. 

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