t(7;11)(p15;p15) NUP98/HOXA9
1999-01-01 Jean-Loup Huret , Jean-Loup Huret Affiliation1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
Clinics and Pathology
Disease
AML mostly; occasionally: CML-like cases without t(9;22), or CMLin blast crisis (with t(9;22))
Phenotype stem cell origin
M2 or M4 AML mainly; involving maturingleukemic cells in AML cases, might affect trilineage progenitors inCML-like cases
Epidemiology
most cases have been found in Japan; balanced sex ratio
Cytology
auer rods; low alkaline phosphatase scores; CML likeblood features
Prognosis
CR in most cases; but patients tend to relapse; mean survival: 15mths
Genes Involved and Proteins
Gene name
HOXA9 (homeobox A9)
Location
7p15.2
Protein description
encodes a class I homeodomain protein potentiallycontains repeated motifs and a RNA binding motif;
Gene name
NUP98 (nucleoporin 98 kDa)
Location
11p15.4
Dna rna description
alternate splicings
Protein description
nucleoporin: role in nucleo-cytoplasmic transport
Result of the Chromosomal Anomaly
Description
5 NUP98 - 3 HOXA9fuses the N-term GLFG repeat domains of NUP98 to the HOXA9 3homeobox
Oncogenesis
may promote leukaemogenesis through inhibition ofHOXA9-mediated terminaldifferentiation and/or aberrant nucleocytoplasmic transport
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 34383740 | 2021 | A genetic screen in Drosophila uncovers the multifaceted properties of the NUP98-HOXA9 oncogene. | 106 |
| 32664447 | 2020 | Disclosing the Interactome of Leukemogenic NUP98-HOXA9 and SET-NUP214 Fusion Proteins Using a Proteomic Approach. | 86 |
| 20339440 | 2010 | Effects of the NUP98-DDX10 oncogene on primary human CD34+ cells: role of a conserved helicase motif. | 83 |
| 34831074 | 2021 | The Retinoblastoma Tumor Suppressor Is Required for the NUP98-HOXA9-Induced Aberrant Nuclear Envelope Phenotype. | 81 |
| 23840580 | 2013 | Dynein Light Chain 1 (DYNLT1) Interacts with Normal and Oncogenic Nucleoporins. | 75 |
| 26740045 | 2016 | Chromatin-prebound Crm1 recruits Nup98-HoxA9 fusion to induce aberrant expression of Hox cluster genes. | 72 |
| 21937451 | 2011 | Amino-terminal enhancer of split (AES) interacts with the oncoprotein NUP98-HOXA9 and enhances its transforming ability. | 69 |
| 15649248 | 2005 | Retroviral insertional mutagenesis identifies oncogene cooperation. | 64 |
| 34163069 | 2021 | Phase separation drives aberrant chromatin looping and cancer development. | 61 |
| 19696924 | 2009 | Dissection of the transformation of primary human hematopoietic cells by the oncogene NUP98-HOXA9. | 61 |
| 24747972 | 2014 | Evi1 defines leukemia-initiating capacity and tyrosine kinase inhibitor resistance in chronic myeloid leukemia. | 60 |
| 20805992 | 2010 | In vitro transformation of primary human CD34+ cells by AML fusion oncogenes: early gene expression profiling reveals possible drug target in AML. | 60 |
| 23427268 | 2013 | In vivo analysis of human nucleoporin repeat domain interactions. | 57 |
| 28422713 | 2017 | SEL120-34A is a novel CDK8 inhibitor active in AML cells with high levels of serine phosphorylation of STAT1 and STAT5 transactivation domains. | 57 |
| 34903620 | 2022 | Phase Separation Mediates NUP98 Fusion Oncoprotein Leukemic Transformation. | 56 |
| 16525506 | 2006 | TGFbeta/BMP inhibits the bone marrow transformation capability of Hoxa9 by repressing its DNA-binding ability. | 55 |
| 9858599 | 1999 | CREB binding protein interacts with nucleoporin-specific FG repeats that activate transcription and mediate NUP98-HOXA9 oncogenicity. | 54 |
| 23326393 | 2013 | Hoxa9 transduction induces hematopoietic stem and progenitor cell activity through direct down-regulation of geminin protein. | 52 |
| 27889185 | 2016 | NUP98 Fusion Proteins Interact with the NSL and MLL1 Complexes to Drive Leukemogenesis. | 52 |
| 20233715 | 2010 | Inhibition of CRM1-mediated nuclear export of transcription factors by leukemogenic NUP98 fusion proteins. | 51 |
| 28202522 | 2017 | Histone Acetyltransferase Activity of MOF Is Required for MLL-AF9 Leukemogenesis. | 50 |
| 27055152 | 2016 | Hypoxia-Like Signatures Induced by BCR-ABL Potentially Alter the Glutamine Uptake for Maintaining Oxidative Phosphorylation. | 50 |
| 33724567 | 2021 | Reduction of leukemic burden via bone-targeted nanoparticle delivery of an inhibitor of C-chemokine (C-C motif) ligand 3 (CCL3) signaling. | 50 |
| 21768400 | 2011 | Graft-versus-leukemia (GVL) against mouse blast-crisis chronic myelogenous leukemia (BC-CML) and chronic-phase chronic myelogenous leukemia (CP-CML): shared mechanisms of T cell killing, but programmed death ligands render CP-CML and not BC-CML GVL resistant. | 50 |
| 12032333 | 2002 | A murine model of CML blast crisis induced by cooperation between BCR/ABL and NUP98/HOXA9. | 45 |
| 39103751 | 2024 | Clinical features and prognosis of patients with myeloid neoplasms harboring t(7;11)(p15;p15) translocation: a single-center retrospective study. | 43 |
| 17601986 | 2007 | Leukemia stem cells in a genetically defined murine model of blast-crisis CML. | 43 |
| 18345353 | 2007 | Imaging hematopoietic precursor division in real time. | 40 |
| 11157742 | 2001 | NUP98-HOXA9 expression in hemopoietic stem cells induces chronic and acute myeloid leukemias in mice. | 39 |
| 28521413 | 2017 | Chromosome t(7;11)(p15;p15) translocation in acute myeloid leukemia coexisting with multilineage dyspoiesis and mutations in NRAS and WT1: A case report and literature review. | 37 |
| 34582559 | 2022 | The menin-MLL1 interaction is a molecular dependency in NUP98-rearranged AML. | 36 |
| 20639863 | 2010 | Regulation of myeloid leukaemia by the cell-fate determinant Musashi. | 36 |
| 11369863 | 2001 | The HoxB1 hexapeptide is a prefolded domain: implications for the Pbx1/Hox interaction. | 35 |
| 32810969 | 2020 | [Acute myeloid leukemia with co-expression of TEL-ABL1 and NUP98-HOXA9 fusion genes: a case report and literature review]. | 31 |
| 39344146 | 2025 | Rare Non-Cryptic NUP98 Rearrangements Associated With Myeloid Neoplasms and Their Poor Prognostic Impact. | 25 |
| 27033163 | 2016 | Transient potential receptor melastatin-2 (Trpm2) does not influence murine MLL-AF9-driven AML leukemogenesis or in vitro response to chemotherapy. | 25 |
| 24021671 | 2013 | Essential role of PR-domain protein MDS1-EVI1 in MLL-AF9 leukemia. | 24 |
| 36304323 | 2022 | An Image Analysis Pipeline for Quantifying the Features of Fluorescently-Labeled Biomolecular Condensates in Cells. | 23 |
| 24971156 | 2014 | Blast crisis Ph+ chronic myeloid leukemia with NUP98/HOXA13 up-regulating MSI2. | 23 |
| 35381677 | 2022 | [A three-series case report and literature review of acute myeloid leukemia with t(7;11)(p15;p15)/NUP98-HOXA9]. | 19 |
| 28630438 | 2017 | The molecular pathogenesis of the NUP98-HOXA9 fusion protein in acute myeloid leukemia. | 18 |
| 32499242 | 2021 | Epigenetic and functional changes imposed by NUP98-HOXA9 in a genetically engineered model of chronic myeloid leukemia progression. | 15 |
| 21252854 | 2010 | Colony forming cell (CFC) assay for human hematopoietic cells. | 7 |
| 28210005 | 2017 | MLL is essential for NUP98-HOXA9-induced leukemia. | 0 |
| 36861732 | 2023 | Validation of the prognostic significance of the 2022 European LeukemiaNet risk stratification system in intensive chemotherapy treated aged 18 to 65 years patients with de novo acute myeloid leukemia. | 0 |
| 39017854 | 2024 | HOXA9 Regulome and Pharmacological Interventions in Leukemia. | 0 |
| 37995102 | 2023 | Acute myeloid leukemia with NUP98::HOXA9 mimicking acute promyelocytic leukemia. | 0 |
| 27838340 | 2017 | Human NUP98-HOXA9 promotes hyperplastic growth of hematopoietic tissues in Drosophila. | 0 |
| 14561764 | 2004 | The oncogene Nup98-HOXA9 induces gene transcription in myeloid cells. | 0 |
| 21810091 | 2011 | NUP98-HOXA9-transgenic zebrafish develop a myeloproliferative neoplasm and provide new insight into mechanisms of myeloid leukaemogenesis. | 0 |
| 12082612 | 2002 | 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. | 0 |
| 16105755 | 2005 | NUP98 fusion in human leukemia: dysregulation of the nuclear pore and homeodomain proteins. | 0 |
| 17442773 | 2007 | NUP98 dysregulation in myeloid leukemogenesis. | 0 |
| 16818636 | 2006 | NUP98-HOXA9 induces long-term proliferation and blocks differentiation of primary human CD34+ hematopoietic cells. | 0 |
| 21467841 | 2011 | RNA export factor RAE1 contributes to NUP98-HOXA9-mediated leukemogenesis. | 0 |
| 17178874 | 2006 | Enforced expression of NUP98-HOXA9 in human CD34(+) cells enhances stem cell proliferation. | 0 |
| 15454493 | 2005 | Identification of cooperative genes for NUP98-HOXA9 in myeloid leukemogenesis using a mouse model. | 0 |
| 37516964 | 2023 | Phase-separated nuclear bodies of nucleoporin fusions promote condensation of MLL1/CRM1 and rearrangement of 3D genome structure. | 0 |
| 12112533 | 2002 | 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. | 0 |
| 8563753 | 1996 | Fusion of the nucleoporin gene NUP98 to HOXA9 by the chromosome translocation t(7;11)(p15;p15) in human myeloid leukaemia. | 0 |
| 26418229 | 2016 | NUP98-HOXA9 bearing therapy-related myeloid neoplasm involves myeloid-committed cell and induces HOXA5, EVI1, FLT3, and MEIS1 expression. | 0 |
| 12393433 | 2002 | Oncogenic interaction between BCR-ABL and NUP98-HOXA9 demonstrated by the use of an in vitro purging culture system. | 0 |
| 10583265 | 1999 | Molecular heterogeneity of the NUP98/HOXA9 fusion transcript in myelodysplastic syndromes associated with t(7;11)(p15;p15). | 0 |
| 15721637 | 2005 | NUP98 is fused to HOXA9 in a variant complex t(7;11;13;17) in a patient with AML-M2. | 0 |
| 39836982 | 2025 | Clinicopathologic and Molecular Characterization of NUP98-Rearranged Acute Leukemias. | 0 |
| 18388181 | 2008 | Leukemogenic mechanisms and targets of a NUP98/HHEX fusion in acute myeloid leukemia. | 0 |
| 32514625 | 2021 | Faggot cells in acute myeloid leukemia with t(7;11)(p15;p15) and NUP98-HOXA9 fusion. | 0 |
| 11830496 | 2002 | 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). | 0 |
| 8563754 | 1996 | The t(7;11)(p15;p15) translocation in acute myeloid leukaemia fuses the genes for nucleoporin NUP98 and class I homeoprotein HOXA9. | 0 |
| 15963848 | 2005 | Converging pathways in leukemogenesis and stem cell self-renewal. | 0 |
| 10221343 | 1999 | Low frequency of rearrangements of the homeobox gene HOXA9/t(7;11) in adult acute myeloid leukemia. | 0 |
| 26017032 | 2015 | Epigenetic therapy restores normal hematopoiesis in a zebrafish model of NUP98-HOXA9-induced myeloid disease. | 0 |
| 10848835 | 2000 | Expression of the NUP98/HOXA9 fusion transcript in the blast crisis of Philadelphia chromosome-positive chronic myelogenous leukaemia with t(7;11)(p15;p15). | 0 |
| 15681849 | 2005 | HOXA9 activates transcription of the gene encoding gp91Phox during myeloid differentiation. | 0 |
| 21471525 | 2011 | Smad4 binds Hoxa9 in the cytoplasm and protects primitive hematopoietic cells against nuclear activation by Hoxa9 and leukemia transformation. | 0 |
| 10565304 | 1999 | Chronic myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98/HOXA9 fusion. | 0 |
| 23754767 | 2013 | Atypical chronic myeloid leukemia harboring NUP98-HOXA9. | 0 |
| 29551043 | 2018 | [The expression of NUP98-HOXA9 in adult acute myeloid leukemia: a case report]. | 0 |
| 20807797 | 2010 | Pathobiological aspects of the local bone marrow renin-angiotensin system: a review. | 0 |
| 11241795 | 2001 | NUP98 gene rearrangements and the clonal evolution of chronic myelogenous leukemia. | 0 |
| 19005624 | 2008 | Additional acquisition of t(1;21)(p32;q22) in a patient relapsing with acute myelogenous leukemia with NUP98-HOXA9. | 0 |
| 10397741 | 1999 | NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15). | 0 |
| 30835357 | 2019 | [Characteristics of a patient with myeloid neoplasm and co-existence of t(7;11)(p15;p15) and t(5;12)(q33;p13) translocations]. | 0 |
| 23800796 | 2013 | Clinical and laboratory studies of 17 patients with acute myeloid leukemia harboring t(7;11)(p15;p15) translocation. | 0 |
| 29105243 | 2018 | Multiplex fusion gene testing in pediatric acute myeloid leukemia. | 0 |
| 19225539 | 2009 | Acute myeloid leukemia bearing t(7;11)(p15;p15) is a distinct cytogenetic entity with poor outcome and a distinct mutation profile: comparative analysis of 493 adult patients. | 0 |
| 26542893 | 2016 | ETV6-LPXN fusion transcript generated by t(11;12)(q12.1;p13) in a patient with relapsing acute myeloid leukemia with NUP98-HOXA9. | 0 |
| 9766650 | 1998 | NUP98-HOXD13 gene fusion in therapy-related acute myelogenous leukemia. | 0 |
| 27060678 | 2016 | NUP98-PHF23 fusion is recurrent in acute myeloid leukemia and shares gene expression signature of leukemic stem cells. | 0 |
| 16651408 | 2006 | Trans-repressive effect of NUP98-PMX1 on PMX1-regulated c-FOS gene through recruitment of histone deacetylase 1 by FG repeats. | 0 |
| 34627423 | 2021 | [Clinical Characteristics and Prognosis of Adult Acute Myeloid Leukemia with NUP98 Gene Rearrangement]. | 0 |
| 9074407 | 1997 | Clinical, haematological and molecular studies in patients with chromosome translocation t(7;11): a study of four Chinese patients in Taiwan. | 0 |
| 10936866 | 2000 | Down's syndrome with myelodysplastic syndrome showing t(7;11)(p13;p14). | 0 |
| 30378313 | 2018 | [Molecular Characteristics and Clinical Features of Adults with NUP98 Fusions in Acute Myeloid Leukemia]. | 0 |
| 11588024 | 2001 | Estrogen-dependent E2a/Pbx1 myeloid cell lines exhibit conditional differentiation that can be arrested by other leukemic oncoproteins. | 0 |
| 15182530 | 2004 | [HUP98-HOXA9 transgenic mice are susceptible to N-ethyl-N-nitrosourea stimulation in leukemogenesis]. | 0 |
| 19338047 | 2009 | Juvenile myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98-HOXA11 fusion. | 0 |
| 11841413 | 2002 | t(7;11)(p15;p15) Chronic myeloid leukaemia developed into blastic transformation showing a novel NUP98/HOXA11 fusion. | 0 |
| 30626792 | 2018 | [Significance of monitoring cerebrospinal fluid leukocyte counts in managing central nervous system disease of acute myeloid leukemia in patients presenting with intracerebral hemorrhage upon initial examination]. | 0 |
| 12138901 | 2002 | A case of acute myeloid leukemia with t(7;11)(p15;p15) mimicking myeloid crisis of chronic myelogenous leukemia. | 0 |
Bibliography
No bibliography items were found for this article.
Summary
Fusion gene
NUP98/HOXA9 NUP98 (11p15.4) HOXA9 (7p15.2) M t(7;11)(p15;p15)|NUP98/HOXA9 NUP98 (11p15.4) HOXA9 (7p15.2) TIC
t(7;11)(p15;p15) G-banding - Courtesy Diane H. Norback, Eric B. Johnson, and Sara Morrison-Delap, UW Cytogenetic Services
Citation
Jean-Loup Huret ; Jean-Loup Huret
t(7;11)(p15;p15) NUP98/HOXA9
Atlas Genet Cytogenet Oncol Haematol. 1999-01-01
Online version: http://atlasgeneticsoncology.org/haematological/1016/new-content/meetings/
