t(7;11)(p15;p15) NUP98/HOXA9

1999-01-01   Jean-Loup Huret  , Jean-Loup Huret  

1.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 IDYearTitleCitations
343837402021A genetic screen in Drosophila uncovers the multifaceted properties of the NUP98-HOXA9 oncogene.106
326644472020Disclosing the Interactome of Leukemogenic NUP98-HOXA9 and SET-NUP214 Fusion Proteins Using a Proteomic Approach.86
203394402010Effects of the NUP98-DDX10 oncogene on primary human CD34+ cells: role of a conserved helicase motif.83
348310742021The Retinoblastoma Tumor Suppressor Is Required for the NUP98-HOXA9-Induced Aberrant Nuclear Envelope Phenotype.81
238405802013Dynein Light Chain 1 (DYNLT1) Interacts with Normal and Oncogenic Nucleoporins.75
267400452016Chromatin-prebound Crm1 recruits Nup98-HoxA9 fusion to induce aberrant expression of Hox cluster genes.72
219374512011Amino-terminal enhancer of split (AES) interacts with the oncoprotein NUP98-HOXA9 and enhances its transforming ability.69
156492482005Retroviral insertional mutagenesis identifies oncogene cooperation.64
341630692021Phase separation drives aberrant chromatin looping and cancer development.61
196969242009Dissection of the transformation of primary human hematopoietic cells by the oncogene NUP98-HOXA9.61
247479722014Evi1 defines leukemia-initiating capacity and tyrosine kinase inhibitor resistance in chronic myeloid leukemia.60
208059922010In vitro transformation of primary human CD34+ cells by AML fusion oncogenes: early gene expression profiling reveals possible drug target in AML.60
234272682013In vivo analysis of human nucleoporin repeat domain interactions.57
284227132017SEL120-34A is a novel CDK8 inhibitor active in AML cells with high levels of serine phosphorylation of STAT1 and STAT5 transactivation domains.57
349036202022Phase Separation Mediates NUP98 Fusion Oncoprotein Leukemic Transformation.56
165255062006TGFbeta/BMP inhibits the bone marrow transformation capability of Hoxa9 by repressing its DNA-binding ability.55
98585991999CREB binding protein interacts with nucleoporin-specific FG repeats that activate transcription and mediate NUP98-HOXA9 oncogenicity.54
233263932013Hoxa9 transduction induces hematopoietic stem and progenitor cell activity through direct down-regulation of geminin protein.52
278891852016NUP98 Fusion Proteins Interact with the NSL and MLL1 Complexes to Drive Leukemogenesis.52
202337152010Inhibition of CRM1-mediated nuclear export of transcription factors by leukemogenic NUP98 fusion proteins.51
282025222017Histone Acetyltransferase Activity of MOF Is Required for MLL-AF9 Leukemogenesis.50
270551522016Hypoxia-Like Signatures Induced by BCR-ABL Potentially Alter the Glutamine Uptake for Maintaining Oxidative Phosphorylation.50
337245672021Reduction of leukemic burden via bone-targeted nanoparticle delivery of an inhibitor of C-chemokine (C-C motif) ligand 3 (CCL3) signaling.50
217684002011Graft-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
120323332002A murine model of CML blast crisis induced by cooperation between BCR/ABL and NUP98/HOXA9.45
391037512024Clinical features and prognosis of patients with myeloid neoplasms harboring t(7;11)(p15;p15) translocation: a single-center retrospective study.43
176019862007Leukemia stem cells in a genetically defined murine model of blast-crisis CML.43
183453532007Imaging hematopoietic precursor division in real time.40
111577422001NUP98-HOXA9 expression in hemopoietic stem cells induces chronic and acute myeloid leukemias in mice.39
285214132017Chromosome 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
345825592022The menin-MLL1 interaction is a molecular dependency in NUP98-rearranged AML.36
206398632010Regulation of myeloid leukaemia by the cell-fate determinant Musashi.36
113698632001The HoxB1 hexapeptide is a prefolded domain: implications for the Pbx1/Hox interaction.35
328109692020[Acute myeloid leukemia with co-expression of TEL-ABL1 and NUP98-HOXA9 fusion genes: a case report and literature review].31
393441462025Rare Non-Cryptic NUP98 Rearrangements Associated With Myeloid Neoplasms and Their Poor Prognostic Impact.25
270331632016Transient potential receptor melastatin-2 (Trpm2) does not influence murine MLL-AF9-driven AML leukemogenesis or in vitro response to chemotherapy.25
240216712013Essential role of PR-domain protein MDS1-EVI1 in MLL-AF9 leukemia.24
363043232022An Image Analysis Pipeline for Quantifying the Features of Fluorescently-Labeled Biomolecular Condensates in Cells.23
249711562014Blast crisis Ph+ chronic myeloid leukemia with NUP98/HOXA13 up-regulating MSI2.23
353816772022[A three-series case report and literature review of acute myeloid leukemia with t(7;11)(p15;p15)/NUP98-HOXA9].19
286304382017The molecular pathogenesis of the NUP98-HOXA9 fusion protein in acute myeloid leukemia.18
324992422021Epigenetic and functional changes imposed by NUP98-HOXA9 in a genetically engineered model of chronic myeloid leukemia progression.15
212528542010Colony forming cell (CFC) assay for human hematopoietic cells.7
282100052017MLL is essential for NUP98-HOXA9-induced leukemia.0
368617322023Validation 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
390178542024HOXA9 Regulome and Pharmacological Interventions in Leukemia.0
379951022023Acute myeloid leukemia with NUP98::HOXA9 mimicking acute promyelocytic leukemia.0
278383402017Human NUP98-HOXA9 promotes hyperplastic growth of hematopoietic tissues in Drosophila.0
145617642004The oncogene Nup98-HOXA9 induces gene transcription in myeloid cells.0
218100912011NUP98-HOXA9-transgenic zebrafish develop a myeloproliferative neoplasm and provide new insight into mechanisms of myeloid leukaemogenesis.0
120826122002Nup98-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
161057552005NUP98 fusion in human leukemia: dysregulation of the nuclear pore and homeodomain proteins.0
174427732007NUP98 dysregulation in myeloid leukemogenesis.0
168186362006NUP98-HOXA9 induces long-term proliferation and blocks differentiation of primary human CD34+ hematopoietic cells.0
214678412011RNA export factor RAE1 contributes to NUP98-HOXA9-mediated leukemogenesis.0
171788742006Enforced expression of NUP98-HOXA9 in human CD34(+) cells enhances stem cell proliferation.0
154544932005Identification of cooperative genes for NUP98-HOXA9 in myeloid leukemogenesis using a mouse model.0
375169642023Phase-separated nuclear bodies of nucleoporin fusions promote condensation of MLL1/CRM1 and rearrangement of 3D genome structure.0
121125332002The 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
85637531996Fusion of the nucleoporin gene NUP98 to HOXA9 by the chromosome translocation t(7;11)(p15;p15) in human myeloid leukaemia.0
264182292016NUP98-HOXA9 bearing therapy-related myeloid neoplasm involves myeloid-committed cell and induces HOXA5, EVI1, FLT3, and MEIS1 expression.0
123934332002Oncogenic interaction between BCR-ABL and NUP98-HOXA9 demonstrated by the use of an in vitro purging culture system.0
105832651999Molecular heterogeneity of the NUP98/HOXA9 fusion transcript in myelodysplastic syndromes associated with t(7;11)(p15;p15).0
157216372005NUP98 is fused to HOXA9 in a variant complex t(7;11;13;17) in a patient with AML-M2.0
398369822025Clinicopathologic and Molecular Characterization of NUP98-Rearranged Acute Leukemias.0
183881812008Leukemogenic mechanisms and targets of a NUP98/HHEX fusion in acute myeloid leukemia.0
325146252021Faggot cells in acute myeloid leukemia with t(7;11)(p15;p15) and NUP98-HOXA9 fusion.0
118304962002Single-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
85637541996The t(7;11)(p15;p15) translocation in acute myeloid leukaemia fuses the genes for nucleoporin NUP98 and class I homeoprotein HOXA9.0
159638482005Converging pathways in leukemogenesis and stem cell self-renewal.0
102213431999Low frequency of rearrangements of the homeobox gene HOXA9/t(7;11) in adult acute myeloid leukemia.0
260170322015Epigenetic therapy restores normal hematopoiesis in a zebrafish model of NUP98-HOXA9-induced myeloid disease.0
108488352000Expression of the NUP98/HOXA9 fusion transcript in the blast crisis of Philadelphia chromosome-positive chronic myelogenous leukaemia with t(7;11)(p15;p15).0
156818492005HOXA9 activates transcription of the gene encoding gp91Phox during myeloid differentiation.0
214715252011Smad4 binds Hoxa9 in the cytoplasm and protects primitive hematopoietic cells against nuclear activation by Hoxa9 and leukemia transformation.0
105653041999Chronic myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98/HOXA9 fusion.0
237547672013Atypical chronic myeloid leukemia harboring NUP98-HOXA9.0
295510432018[The expression of NUP98-HOXA9 in adult acute myeloid leukemia: a case report].0
208077972010Pathobiological aspects of the local bone marrow renin-angiotensin system: a review.0
112417952001NUP98 gene rearrangements and the clonal evolution of chronic myelogenous leukemia.0
190056242008Additional acquisition of t(1;21)(p32;q22) in a patient relapsing with acute myelogenous leukemia with NUP98-HOXA9.0
103977411999NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15).0
308353572019[Characteristics of a patient with myeloid neoplasm and co-existence of t(7;11)(p15;p15) and t(5;12)(q33;p13) translocations].0
238007962013Clinical and laboratory studies of 17 patients with acute myeloid leukemia harboring t(7;11)(p15;p15) translocation.0
291052432018Multiplex fusion gene testing in pediatric acute myeloid leukemia.0
192255392009Acute 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
265428932016ETV6-LPXN fusion transcript generated by t(11;12)(q12.1;p13) in a patient with relapsing acute myeloid leukemia with NUP98-HOXA9.0
97666501998NUP98-HOXD13 gene fusion in therapy-related acute myelogenous leukemia.0
270606782016NUP98-PHF23 fusion is recurrent in acute myeloid leukemia and shares gene expression signature of leukemic stem cells.0
166514082006Trans-repressive effect of NUP98-PMX1 on PMX1-regulated c-FOS gene through recruitment of histone deacetylase 1 by FG repeats.0
346274232021[Clinical Characteristics and Prognosis of Adult Acute Myeloid Leukemia with NUP98 Gene Rearrangement].0
90744071997Clinical, haematological and molecular studies in patients with chromosome translocation t(7;11): a study of four Chinese patients in Taiwan.0
109368662000Down's syndrome with myelodysplastic syndrome showing t(7;11)(p13;p14).0
303783132018[Molecular Characteristics and Clinical Features of Adults with NUP98 Fusions in Acute Myeloid Leukemia].0
115880242001Estrogen-dependent E2a/Pbx1 myeloid cell lines exhibit conditional differentiation that can be arrested by other leukemic oncoproteins.0
151825302004[HUP98-HOXA9 transgenic mice are susceptible to N-ethyl-N-nitrosourea stimulation in leukemogenesis].0
193380472009Juvenile myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98-HOXA11 fusion.0
118414132002t(7;11)(p15;p15) Chronic myeloid leukaemia developed into blastic transformation showing a novel NUP98/HOXA11 fusion.0
306267922018[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
121389012002A 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
Atlas Image
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/t(7;11)(p15;p15)-nup98-hoxa9