Recombinant Human Nuclear pore complex protein Nup98-Nup96(NUP98) ,partial

1. NHA9 (NUP98-HOXA9 fusion protein) deregulates the expression of key leukemic genes, including MEIS1-HOXA9-PBX3 complex, through the enhancer binding and the direct interaction of the fusion protein with HDAC and p300 transcriptional regulators PMID: 28630438
2. human NUP98-IQCG fusion protein could induce fatal and transplantable acute myelomonocytic leukemia in a mouse model PMID: 26675333
3. Importantly, binding of Nup98 to DHX9 stimulates the ATPase activity of DHX9, and a transcriptional reporter assay suggests Nup98 supports DHX9-stimulated transcription. PMID: 28221134
4. The second FG repeat domain of the NUP98 moiety of the NUP98-HOXA9 fusion protein is important for its cell immortalization and leukemogenesis activities. NUP98-HOXA9 interacts with mixed lineage leukemia (MLL) via this FG repeat domain and that, in MLL-null mice, NUP98-HOXA9-induced cell immortalization and leukemogenesis are severely inhibited. PMID: 28210005
5. DYNLT1 interacts with nucleoporins and plays a role in the dysregulation of gene expression and induction of hematopoietic cell proliferation by the leukemogenic nucleoporin fusion, NUP98-HOXA9 PMID: 23840580
6. the mutation order in the NUP98-rearranged pediatric AML begins with the NUP98 rearrangement leading to epigenetic dysregulations then followed by mutations of critical hematopoietic transcription factors and finally, activation of the FLT3 signaling pathway. PMID: 27694926
7. These results provide novel insight into the mechanisms underlying the aberrant capability of NUP98 oncoproteins to interact with APC/C(Cdc20) and to interfere with its function. PMID: 27097363
8. Our results suggest that NA10HD increases the number of gamma-globin-transduced HSCs that engraft, leading to an elevated number of fetal hemoglobin-containing red cells. PMID: 28190779
9. NUP98-HOXA9 ability to induce blood cell expansion is evolutionarily conserved. PMID: 27838340
10. The study demonstrated the association of NUP98-IQCG with CRM1, and found that NUP98-IQCG expression inhibits the CRM1-mediated nuclear export of p65 and enhances the transcriptional activity of nuclear factor-kappaB. Moreover, IQCG could be entrapped in the nucleus by NUP98-IQCG, and the fusion protein interacts with calmodulin via the IQ motif in a calcium-independent manner. PMID: 27864780
11. The results indicate that highly selective targeting of Nup98-fusion proteins to Hox cluster regions via prebound Crm1 induces the formation of higher order chromatin structures that causes aberrant Hox gene regulation. PMID: 26740045
12. Despite the difference in localization, all tested Nup98 chimera provoked morphological alterations in the nuclear envelope (NE), in particular affecting the nuclear lamina and the lamina-associated polypeptide 2alpha. PMID: 27031510
13. NUP98-HOXA9 expression induces myeloid disease. PMID: 26017032
14. Overall, these results demonstrate a novel role for FOXK1 in regulating the expression of antiviral genes via Nup98, from insects to humans. PMID: 25852164
15. deregulation of the retinoid/rexinoid signaling pathway has a major role and may represent a potential therapeutic target for NUP98-RARG-mediated transformation PMID: 25510432
16. Acute myeloid leukemia can be reproduced in mice by transducing mouse mesenchymal stem cells with the human NUP98-NSD1 fusion and the FLT3-ITD mutated constracts. PMID: 24951466
17. These data reveal an emergent Kap-centric barrier mechanism that may underlie mechanistic and kinetic control in the nuclear pore complex. PMID: 24739174
18. Vesiculoviral matrix (M) protein occupies nucleic acid binding site at nucleoporin pair (Rae1 * Nup98). PMID: 24927547
19. NUP98 oncoproteins predispose myeloid cells to oncogenic transformation or malignant progression by promoting whole chromosome instability. PMID: 24371226
20. NUP98/JARID1A is a novel recurrent genetic abnormality in pediatric AMKL PMID: 23531517
21. Data indicate increased levels of reactive oxygen species (ROS) were detected in bone marrow nucleated cells (BMNC) that express CD71 in in NUP98-HOXD13 (NHD13) transgenic mice, a murine model for myelodysplastic syndromes (MDS). PMID: 23958061
22. support to the adoption of screening for NUP98-NSD1 in pediatric AML without otherwise favorable genetic markers PMID: 23999921
23. The repeat domain of Nup98 is substantially more cohesive than repeat domains from other nucleoporins. PMID: 23427268
24. Dominant negative Nup98 fusion proteins disrupt the transcriptional activity of wild-type Nup98 in the nucleoplasm to drive acute myeloid leukemia. PMID: 23246429
25. Nup98 RNA interference significantly suppresses downstream mRNA expression in the ss-catenin pathway, while nuclear galectin-3 binds to ss-catenin to inhibit transcriptional activity. PMID: 23541576
26. This work demonstrates for the first time that NUP98 dynamically associates with the human genome during differentiation, revealing a role of a nuclear pore protein in regulating developmental gene expression programs. PMID: 23468646
27. The roles of NUP153 and nup98 in the integration and replication of HIV-1 in human Jurkat lymphocytes are reported. PMID: 23523133
28. vesicular stomatitis virus M protein interacted efficiently with Rae1-Nup98 complexes associated with the chromatin fraction of host nuclei, consistent with an effect on host transcription PMID: 23028327
29. NUP98/NSD1 fusions are rare in adult acute myeloid leukemia. PMID: 22929522
30. Positive NUP98-NSD1 fusion is associated with acute myeloid leukemia. PMID: 22945772
31. Nup98 functions as a potential tumor suppressor and regulates posttranscriptional expression of select p53 target genes. PMID: 23102701
32. findings show that expression of NUP98-HOXD13 impairs class switch recombination and reduces the antibody-mediated immune response, in addition to its role in leukemia PMID: 22613470
33. Human Nup98 intereacted with Nup82 through a reciprocal exchange of loop structures. Strikingly, the same Nup98 groove promiscuously interacted with Nup82 and Nup96 in a mutually excusive fashion. PMID: 22480613
34. the recurrent NUP98-CCDC28A is an oncogene that induces a rapid and transplantable myeloid neoplasm in recipient mice. They also provide additional evidence for an alternative leukemogenic mechanism for NUP98 oncogenes. PMID: 22058212
35. The fusion genes combining NUP98 exon 11/12 with PSIP1 exon 8 may be implicated in the pathogenesis of myelodysplastic syndrome (MDS). PMID: 22103895
36. RAE1 transgene may be directly involved in NUP98 fusion-mediated leukemogenesis. PMID: 21467841
37. Structural chromosomal rearrangements of NUP98, primarily balanced translocations and inversions, are associated with wide array of hematopoietic malignancies; NUP98 is known to be fused to at least 28 different partner genes in patients. [REVIEW] PMID: 21948299
38. NUP98/NSD1 identifies a previously unrecognized group of young AML patients, with distinct characteristics and dismal prognosis, for whom new treatment strategies are urgently needed. PMID: 21813447
39. Ectopic expression of NA10 (Nup98-HoxA10) augments erythroid differentiation of human embryonic stem cells. PMID: 21328509
40. Study identified mitotic phosphorylation of Nup98 as a rate-limiting step in mitotic nuclear pore complexes disassembly. PMID: 21335236
41. NUP98/RARG gene rearrangement is associated with acute myeloid leukemia. PMID: 20935257
42. results suggest high frequency of cell proliferation gene mutations may contribute to leukemogenesis in NUP98-related leukemia; simultaneous occurrence of FLT3-ITD and WT1 aberrations may have an important role in outcome of NUP98-related leukemia PMID: 20861915
43. characterized a novel recurrent chromosomal aberration, inv(11)(p15q23) in 2 patients with acute myeloid leukemia. This aberration is predicted to result in the expression of a NUP98-MLL fusion protein that is unable to interact with menin. PMID: 20558618
44. Mapping tests further demonstrated that aa 22-53 in the N-terminal region of H5N1 virus NS2 and the glycine-leucine-phenylalanine-glycine (GLFG) repeat domain (aa 1-511) of human Nup98 are crucial for the interaction of these two proteins. PMID: 20554795
45. Findings suggest new possibilities for misregulation by which Nup98 translocations may contribute to cellular transformation and leukemogenesis. PMID: 20237156
46. present the crystal structure of human Rae1 in complex with the Gle2-binding sequence (GLEBS) of Nup98 at 1.65 A resolution. Rae1 forms a seven-bladed beta-propeller with several extensive surface loops. PMID: 20498086
47. NUP98-DDX10 dramatically increases proliferation and results in leukemogenesis. PMID: 20339440
48. Oxidative stress up-regulated the binding of Crm1 to Ran and affected multiple repeat-containing nucleoporins by changing their localization, phosphorylation, O-glycosylation, or interaction with other transport components. PMID: 19828735
49. effects of NUP98-HOXA9 on gene transcription and cell transformation are mediated by two distinct mechanisms: promoter binding through homeodomain with direct transcriptional activation, and another depending on NUP98 moiety not involving DNA binding PMID: 19696924
50. These results are consistent with a specific proteolysis of Nup98 by 2A protease to prevent de novo mRNA traffic in poliovirus-infected cells. PMID: 19789179
51. The HOXD11 gene is fused to the NUP98 gene in acute myeloid leukemia with t(2;11)(q31;p15). PMID: 11782354
52. increased Nup98 and Nup96 expression constitutes an IFN-mediated mechanism that reverses M protein-mediated inhibition of gene expression PMID: 11809937
53. the fusion gene NUP98-HOXA9 is an important gene in myeloid leukemogenesis. PMID: 11830496
54. Nup98 aids in direction of RNAs to the nuclear pore and provide the first potential mechanism for the role of a mobile nucleoporin. PMID: 11950939
55. Nup98-HoxA9 immortalizes myeloid progenitors, enforces expression of Hoxa9, Hoxa7 and Meis1, and alters cytokine-specific responses in a manner similar to that induced by retroviral co-expression of Hoxa9 and Meis1. PMID: 12082612
56. The chromosome translocation t(7;11)(p15;p15) in acute myeloid leukemia results in fusion of the NUP98 gene with cluster gene HOXA13. PMID: 12112533
57. The NUP98/HOXA9 FUSION transcript was detected by PC at exon A and not exon B of NUP98. PMID: 12138901
58. The three-dimensional structure of the autoproteolytic, nuclear pore-targeting domain of the human nucleoporin Nup98. PMID: 12191480
59. Nup98 interacts with both faces of the nuclear pore, a localization in keeping with its previously described nucleocytoplasmic shuttling activity PMID: 12589057
60. fused with NSD1 and frequently transcribed in childhool AML PMID: 12931227
61. Major form of NUP98/HOXC11 fusion in adult acute myeloid leukemia with t(11;12)(p15;q13) translocation exhibits aberrant trans-regulatory activity PMID: 12970787
62. Sec13 stably interacts with Nup96 at the NPC during interphase and the shuttling of Sec13 between the nucleus and the cytoplasm may couple and regulate functions between these two compartments PMID: 14517296
63. Specific and potent interactor of the HIV-1 Rev nuclear export signal. This suggests a physiological role for hNup98 in modulating Rev-dependent RNA export during HIV infection PMID: 14554087
64. Nup98-HOXA9 has a role in inducing gene transcription in myeloid cells PMID: 14561764
65. Nup153 and Nup98 have distinct domains to mediate transcription-dependent mobility PMID: 14718558
66. Data report novel nucleoporin 98 fusions with homeobox (HOX)A10, HOXB3 and HOXB4, and describe the results of coexpression of these proteins with the Hox cofactor Meis1 in leukemic induction. PMID: 14966272
67. Nup98 on the nuclear pore complex specifically participates in the nuclear entry of HIV-1 cDNA following HIV-1 entry. PMID: 15207818
68. Review. The newly identified NUP98 gene, located on chromosome 11p15.5, has been observed fused to 15 different partners. It encodes a 98 kD component of the nuclear pore complex, which mediates nucleo-cytoplasmic transport of protein and RNA. PMID: 15359631
69. Dnalc4, Fcgr2b, Fcrl, and Con1 genes cooperated with NUP98-HOXA9 in transforming NIH 3T3 cells. PMID: 15454493
70. CYBB is a common target gene repressed by HoxA10 and activated by HoxA9, and Meis1 and Nup98-hoxA9 have roles in repressing myeloid-specific gene transcription PMID: 15681849
71. A novel fusion of the NUP98 and LEDGF genes in a pediatric case of acute myeloid leukemia. PMID: 15725483
72. NUP98 has a role in hematopoietic malignancies with 11p15 aberrations PMID: 15951287
73. Caspases target only Nup93 and Nup96 within the core structure of the nuclear pore complex PMID: 16286466
74. synergism between NUP98-HOX and wt-Flt3 is the result of the ability of Flt3 to increase proliferation of myeloid progenitors blocked in differentiation by NUP98-HOX fusions and, revealing a direct role for wt-Flt3 in the pathobiology of AML. PMID: 16861351
75. The t(7;11)(p15;p15) translocation, observed in acute myelogenous leukemia and myelodysplastic syndrome, generates a chimeric gene where the 5' portion of the sequence encoding the human nucleoporin NUP98 protein is fused to the 3' region of HOXA9. PMID: 17178874
76. NUP98 is dysregulated in myeloid leukemogenesis [review] PMID: 17442773
77. study shows that NUP98-NSD1 induces acute myeloid leukemia in vivo, sustains self-renewal of myeloid stem cells in vitro, and enforces expression of the HoxA7, HoxA9, HoxA10 and Meis1 proto-oncogenes PMID: 17589499
78. Targeting of interferon-inducible NPC proteins, such as Nup98, may be an additional weapon in the arsenal of poliovirus and perhaps other picornaviruses to overcome host defense mechanisms. PMID: 18045934
79. A new fusion gene NUP98-IQCG identified in an acute T-lymphoid/myeloid leukemia with a t(3;11)(q29q13;p15)del(3)(q29) translocation PMID: 18084320
80. High-resolution crystal structures reveal that three highly conserved residues, Tyr866, Gly867, and Leu868, provide most of the interactions between the autoproteolytic domain and the C-terminal tail of Nup98. PMID: 18287282
81. translocation involving nucleoporin 98 (NUP98) fused to the DNA-binding domain of the hematopoietically expressed homeobox gene found in acute myeloid leukemia PMID: 18388181
82. transgenic mice expressing NUP98-HOXD13 (NHD13) fusion gene develop myelodysplastic syndrome, and more than half eventually progress to acute leukemia PMID: 18566322
83. Novel evidence of the contributory role of the NUP98 sequence in conferring leukemogenic properties on a partner gene. PMID: 18604245
84. Nup96 levels regulate differential gene expression in a phase-specific manner, setting the stage for proper cell cycle progression. PMID: 19000832
85. To our knowledge, this is the first case of t(11;12)(p15;q13) in de novo AML-M4 in association with FLT3 ITD mutation. Coexistence of NUP98-HOXC13 fusion and FLT3 ITD mutation is likely relevant in the process of leukemogenesis PMID: 19665070

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HGNC: 8068

OMIM: 601021

KEGG: hsa:4928

STRING: 9606.ENSP00000316032

UniGene: Hs.524750