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Gene Review

NUP98  -  nucleoporin 98kDa

Homo sapiens

Synonyms: ADAR2, ADIR2, NUP196, NUP96, Nuclear pore complex protein Nup98-Nup96
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Disease relevance of NUP98


High impact information on NUP98


Chemical compound and disease context of NUP98


Biological context of NUP98

  • Here we show that in three patients with t(7;11), the chromosome rearrangement creates a genomic fusion between the HOXA9 gene and the nucleoporin gene NUP98 on chromosome 11p15 [5].
  • We show that NUP98 is essential for mouse gastrulation, a developmental stage that is associated with rapid cell proliferation, but dispensable for basal cell growth [11].
  • These results indicate that NUP98 is a recurrent target in therapy-related malignancies, and that TOP1 is a previously unrecognized target for chromosomal translocations [2].
  • Here we demonstrate that the NUP98-HOXA9 fusion gene encodes two nuclear oncoproteins with either 19 or 37 NUP98 FG repeats fused to the DNA binding and PBX heterodimerization domains of the transcription factor HOXA9 [12].
  • This NUP98-derived activity is essential for transformation and can be replaced by the bona fide transactivation domain of VP16 [12].

Anatomical context of NUP98


Associations of NUP98 with chemical compounds

  • This region is always retained in the fusion transcript with the NH(2) terminus FG repeats of NUP98, suggesting an important role in the mechanism of leukemogenesis [17].
  • The NUP98-HOXC11 fusion protein consists of the NH2-terminal phenylalanine-glycine repeat motif of NUP98 and the COOH-terminal homeodomain of HOXC11 [18].
  • These observations raise a possibility that the NUP98/DDX10 fusion might be involved in imatinib resistance as well as in acute transformation of CML [9].

Physical interactions of NUP98

  • Interestingly, the same site within Nup98 is involved in binding to both Nup88 and Nup96 [19].
  • Nup98 was previously described to interact with the nuclear-oriented Nup160, 133, 107, 96 complex through direct binding to Nup96 [19].
  • We found that in vitro translated TPR bound to in vitro translated Nup98 and, via Nup98, to Nup96 [20].
  • VSV M protein binds the mRNA export factor Rae1 that is in complex with Nup98, resulting in nuclear retention of mRNAs [21].
  • In a solution binding assay, both beta1 and beta2 competed with each other for binding to immobilized repeat nucleoporin Nup98 [22].

Regulatory relationships of NUP98

  • Strikingly, a mutation to a TOP1 site to inactivate the isomerase activity essentially left unaltered the growth-promoting and leukemogenic effects of NUP98-TOP1 [23].
  • The antitumor effects of deguelin are related to upregulating the expression of Nup98 and downregulating the expression of Nup88 protein in U937 cells [24].

Other interactions of NUP98


Analytical, diagnostic and therapeutic context of NUP98


  1. A novel gene, NSD1, is fused to NUP98 in the t(5;11)(q35;p15.5) in de novo childhood acute myeloid leukemia. Jaju, R.J., Fidler, C., Haas, O.A., Strickson, A.J., Watkins, F., Clark, K., Cross, N.C., Cheng, J.F., Aplan, P.D., Kearney, L., Boultwood, J., Wainscoat, J.S. Blood (2001) [Pubmed]
  2. The t(11;20)(p15;q11) chromosomal translocation associated with therapy-related myelodysplastic syndrome results in an NUP98-TOP1 fusion. Ahuja, H.G., Felix, C.A., Aplan, P.D. Blood (1999) [Pubmed]
  3. NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15). Nakamura, T., Yamazaki, Y., Hatano, Y., Miura, I. Blood (1999) [Pubmed]
  4. The HOXD11 gene is fused to the NUP98 gene in acute myeloid leukemia with t(2;11)(q31;p15). Taketani, T., Taki, T., Shibuya, N., Ito, E., Kitazawa, J., Terui, K., Hayashi, Y. Cancer Res. (2002) [Pubmed]
  5. Fusion of the nucleoporin gene NUP98 to HOXA9 by the chromosome translocation t(7;11)(p15;p15) in human myeloid leukaemia. Nakamura, T., Largaespada, D.A., Lee, M.P., Johnson, L.A., Ohyashiki, K., Toyama, K., Chen, S.J., Willman, C.L., Chen, I.M., Feinberg, A.P., Jenkins, N.A., Copeland, N.G., Shaughnessy, J.D. Nat. Genet. (1996) [Pubmed]
  6. The t(7;11)(p15;p15) translocation in acute myeloid leukaemia fuses the genes for nucleoporin NUP98 and class I homeoprotein HOXA9. Borrow, J., Shearman, A.M., Stanton, V.P., Becher, R., Collins, T., Williams, A.J., Dubé, I., Katz, F., Kwong, Y.L., Morris, C., Ohyashiki, K., Toyama, K., Rowley, J., Housman, D.E. Nat. Genet. (1996) [Pubmed]
  7. The three-dimensional structure of the autoproteolytic, nuclear pore-targeting domain of the human nucleoporin Nup98. Hodel, A.E., Hodel, M.R., Griffis, E.R., Hennig, K.A., Ratner, G.A., Xu, S., Powers, M.A. Mol. Cell (2002) [Pubmed]
  8. Vesicular stomatitis virus matrix protein inhibits host cell gene expression by targeting the nucleoporin Nup98. von Kobbe C, n.u.l.l., van Deursen JM, n.u.l.l., Rodrigues, J.P., Sitterlin, D., Bachi, A., Wu, X., Wilm, M., Carmo-Fonseca, M., Izaurralde, E. Mol. Cell (2000) [Pubmed]
  9. Clonal evolution with inv(11)(p15q22) and NUP98/DDX10 fusion gene in imatinib-resistant chronic myelogenous leukemia. Yamamoto, M., Kakihana, K., Kurosu, T., Murakami, N., Miura, O. Cancer Genet. Cytogenet. (2005) [Pubmed]
  10. Role of nucleoporin induction in releasing an mRNA nuclear export block. Enninga, J., Levy, D.E., Blobel, G., Fontoura, B.M. Science (2002) [Pubmed]
  11. Disruption of the FG nucleoporin NUP98 causes selective changes in nuclear pore complex stoichiometry and function. Wu, X., Kasper, L.H., Mantcheva, R.T., Mantchev, G.T., Springett, M.J., van Deursen, J.M. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  12. CREB binding protein interacts with nucleoporin-specific FG repeats that activate transcription and mediate NUP98-HOXA9 oncogenicity. Kasper, L.H., Brindle, P.K., Schnabel, C.A., Pritchard, C.E., Cleary, M.L., van Deursen, J.M. Mol. Cell. Biol. (1999) [Pubmed]
  13. Identification of cooperative genes for NUP98-HOXA9 in myeloid leukemogenesis using a mouse model. Iwasaki, M., Kuwata, T., Yamazaki, Y., Jenkins, N.A., Copeland, N.G., Osato, M., Ito, Y., Kroon, E., Sauvageau, G., Nakamura, T. Blood (2005) [Pubmed]
  14. 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. Arai, Y., Hosoda, F., Kobayashi, H., Arai, K., Hayashi, Y., Kamada, N., Kaneko, Y., Ohki, M. Blood (1997) [Pubmed]
  15. NUP98 rearrangements in hematopoietic malignancies: a study of the Groupe Francophone de Cytogénétique Hématologique. Romana, S.P., Radford-Weiss, I., Ben Abdelali, R., Schluth, C., Petit, A., Dastugue, N., Talmant, P., Bilhou-Nabera, C., Mugneret, F., Lafage-Pochitaloff, M., Mozziconacci, M.J., Andrieu, J., Lai, J.L., Terre, C., Rack, K., Cornillet-Lefebvre, P., Luquet, I., Nadal, N., Nguyen-Khac, F., Perot, C., Van den Akker, J., Fert-Ferrer, S., Cabrol, C., Charrin, C., Tigaud, I., Poirel, H., Vekemans, M., Bernard, O.A., Berger, R. Leukemia (2006) [Pubmed]
  16. Transplantable cell lines generated with NUP98-Hox fusion genes undergo leukemic progression by Meis1 independent of its binding to DNA. Pineault, N., Abramovich, C., Humphries, R.K. Leukemia (2005) [Pubmed]
  17. NUP98 is fused to adducin 3 in a patient with T-cell acute lymphoblastic leukemia and myeloid markers, with a new translocation t(10;11)(q25;p15). Lahortiga, I., Vizmanos, J.L., Agirre, X., Vázquez, I., Cigudosa, J.C., Larrayoz, M.J., Sala, F., Gorosquieta, A., Perez-Equiza, K., Calasanz, M.J., Odero, M.D. Cancer Res. (2003) [Pubmed]
  18. Novel NUP98-HOXC11 fusion gene resulted from a chromosomal break within exon 1 of HOXC11 in acute myeloid leukemia with t(11;12)(p15;q13). Taketani, T., Taki, T., Shibuya, N., Kikuchi, A., Hanada, R., Hayashi, Y. Cancer Res. (2002) [Pubmed]
  19. Nup98 localizes to both nuclear and cytoplasmic sides of the nuclear pore and binds to two distinct nucleoporin subcomplexes. Griffis, E.R., Xu, S., Powers, M.A. Mol. Biol. Cell (2003) [Pubmed]
  20. The nucleoporin Nup98 associates with the intranuclear filamentous protein network of TPR. Fontoura, B.M., Dales, S., Blobel, G., Zhong, H. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  21. Nuclear export assays for poly(A) RNAs. Chakraborty, P., Satterly, N., Fontoura, B.M. Methods (2006) [Pubmed]
  22. Karyopherin beta2 mediates nuclear import of a mRNA binding protein. Bonifaci, N., Moroianu, J., Radu, A., Blobel, G. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  23. NUP98-topoisomerase I acute myeloid leukemia-associated fusion gene has potent leukemogenic activities independent of an engineered catalytic site mutation. Gurevich, R.M., Aplan, P.D., Humphries, R.K. Blood (2004) [Pubmed]
  24. Deguelin regulates nuclear pore complex proteins Nup98 and Nup88 in U937 cells in vitro. Liu, H.L., Chen, Y., Cui, G.H., Wu, Q.L., He, J., Chen, W.H., Zhou, J.F. Acta Pharmacol. Sin. (2005) [Pubmed]
  25. Nucleoporins nup98 and nup214 participate in nuclear export of human immunodeficiency virus type 1 Rev. Zolotukhin, A.S., Felber, B.K. J. Virol. (1999) [Pubmed]
  26. Fusion of the NUP98 gene and the homeobox gene HOXC13 in acute myeloid leukemia with t(11;12)(p15;q13). Panagopoulos, I., Isaksson, M., Billström, R., Strömbeck, B., Mitelman, F., Johansson, B. Genes Chromosomes Cancer (2003) [Pubmed]
  27. The chromosome translocation t(7;11)(p15;p15) in acute myeloid leukemia results in fusion of the NUP98 gene with a HOXA cluster gene, HOXA13, but not HOXA9. Taketani, T., Taki, T., Ono, R., Kobayashi, Y., Ida, K., Hayashi, Y. Genes Chromosomes Cancer (2002) [Pubmed]
  28. Single-translocation and double-chimeric transcripts: detection of NUP98-HOXA9 in myeloid leukemias with HOXA11 or HOXA13 breaks of the chromosomal translocation t(7;11)(p15;p15). Fujino, T., Suzuki, A., Ito, Y., Ohyashiki, K., Hatano, Y., Miura, I., Nakamura, T. Blood (2002) [Pubmed]
  29. t(9;11)(p22;p15) in acute myeloid leukemia results in a fusion between NUP98 and the gene encoding transcriptional coactivators p52 and p75-lens epithelium-derived growth factor (LEDGF). Ahuja, H.G., Hong, J., Aplan, P.D., Tcheurekdjian, L., Forman, S.J., Slovak, M.L. Cancer Res. (2000) [Pubmed]
  30. 11p15 translocations involving the NUP98 gene in childhood therapy-related acute myeloid leukemia/myelodysplastic syndrome. Nishiyama, M., Arai, Y., Tsunematsu, Y., Kobayashi, H., Asami, K., Yabe, M., Kato, S., Oda, M., Eguchi, H., Ohki, M., Kaneko, Y. Genes Chromosomes Cancer (1999) [Pubmed]
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