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
206398632010Regulation of myeloid leukaemia by the cell-fate determinant Musashi.160
183453532007Imaging hematopoietic precursor division in real time.127
98585991999CREB binding protein interacts with nucleoporin-specific FG repeats that activate transcription and mediate NUP98-HOXA9 oncogenicity.122
85637531996Fusion of the nucleoporin gene NUP98 to HOXA9 by the chromosome translocation t(7;11)(p15;p15) in human myeloid leukaemia.92
85637541996The t(7;11)(p15;p15) translocation in acute myeloid leukaemia fuses the genes for nucleoporin NUP98 and class I homeoprotein HOXA9.86
176019862007Leukemia stem cells in a genetically defined murine model of blast-crisis CML.73
120323332002A murine model of CML blast crisis induced by cooperation between BCR/ABL and NUP98/HOXA9.69
111577422001NUP98-HOXA9 expression in hemopoietic stem cells induces chronic and acute myeloid leukemias in mice.65
278891852016NUP98 Fusion Proteins Interact with the NSL and MLL1 Complexes to Drive Leukemogenesis.59
168186362006NUP98-HOXA9 induces long-term proliferation and blocks differentiation of primary human CD34+ hematopoietic cells.59
97666501998NUP98-HOXD13 gene fusion in therapy-related acute myelogenous leukemia.46
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 Meis1.44
183881812008Leukemogenic mechanisms and targets of a NUP98/HHEX fusion in acute myeloid leukemia.36
341630692021Phase separation drives aberrant chromatin looping and cancer development.34
145617642004The oncogene Nup98-HOXA9 induces gene transcription in myeloid cells.33
284227132017SEL120-34A is a novel CDK8 inhibitor active in AML cells with high levels of serine phosphorylation of STAT1 and STAT5 transactivation domains.31
171788742006Enforced expression of NUP98-HOXA9 in human CD34(+) cells enhances stem cell proliferation.31
218100912011NUP98-HOXA9-transgenic zebrafish develop a myeloproliferative neoplasm and provide new insight into mechanisms of myeloid leukaemogenesis.30
156818492005HOXA9 activates transcription of the gene encoding gp91Phox during myeloid differentiation.28
202337152010Inhibition of CRM1-mediated nuclear export of transcription factors by leukemogenic NUP98 fusion proteins.27
174427732007NUP98 dysregulation in myeloid leukemogenesis.27
166514082006Trans-repressive effect of NUP98-PMX1 on PMX1-regulated c-FOS gene through recruitment of histone deacetylase 1 by FG repeats.27
203394402010Effects of the NUP98-DDX10 oncogene on primary human CD34+ cells: role of a conserved helicase motif.25
154544932005Identification of cooperative genes for NUP98-HOXA9 in myeloid leukemogenesis using a mouse model.25
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.23
103977411999NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15).23
267400452016Chromatin-prebound Crm1 recruits Nup98-HoxA9 fusion to induce aberrant expression of Hox cluster genes.22
159638482005Converging pathways in leukemogenesis and stem cell self-renewal.21
234272682013In vivo analysis of human nucleoporin repeat domain interactions.20
282100052017MLL is essential for NUP98-HOXA9-induced leukemia.19
247479722014Evi1 defines leukemia-initiating capacity and tyrosine kinase inhibitor resistance in chronic myeloid leukemia.19
214678412011RNA export factor RAE1 contributes to NUP98-HOXA9-mediated leukemogenesis.19
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.19
123934332002Oncogenic interaction between BCR-ABL and NUP98-HOXA9 demonstrated by the use of an in vitro purging culture system.18
282025222017Histone Acetyltransferase Activity of MOF Is Required for MLL-AF9 Leukemogenesis.16
112417952001NUP98 gene rearrangements and the clonal evolution of chronic myelogenous leukemia.16
286304382017The molecular pathogenesis of the NUP98-HOXA9 fusion protein in acute myeloid leukemia.14
260170322015Epigenetic therapy restores normal hematopoiesis in a zebrafish model of NUP98-HOXA9-induced myeloid disease.14
208059922010In vitro transformation of primary human CD34+ cells by AML fusion oncogenes: early gene expression profiling reveals possible drug target in AML.14
214715252011Smad4 binds Hoxa9 in the cytoplasm and protects primitive hematopoietic cells against nuclear activation by Hoxa9 and leukemia transformation.13
212528542010Colony forming cell (CFC) assay for human hematopoietic cells.13
196969242009Dissection of the transformation of primary human hematopoietic cells by the oncogene NUP98-HOXA9.13
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).13
108488352000Expression of the NUP98/HOXA9 fusion transcript in the blast crisis of Philadelphia chromosome-positive chronic myelogenous leukaemia with t(7;11)(p15;p15).13
165255062006TGFbeta/BMP inhibits the bone marrow transformation capability of Hoxa9 by repressing its DNA-binding ability.12
161057552005NUP98 fusion in human leukemia: dysregulation of the nuclear pore and homeodomain proteins.12
115880242001Estrogen-dependent E2a/Pbx1 myeloid cell lines exhibit conditional differentiation that can be arrested by other leukemic oncoproteins.12
208077972010Pathobiological aspects of the local bone marrow renin-angiotensin system: a review.11
278383402017Human NUP98-HOXA9 promotes hyperplastic growth of hematopoietic tissues in Drosophila.10
270551522016Hypoxia-Like Signatures Induced by BCR-ABL Potentially Alter the Glutamine Uptake for Maintaining Oxidative Phosphorylation.10
238007962013Clinical and laboratory studies of 17 patients with acute myeloid leukemia harboring t(7;11)(p15;p15) translocation.10
233263932013Hoxa9 transduction induces hematopoietic stem and progenitor cell activity through direct down-regulation of geminin protein.10
193380472009Juvenile myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98-HOXA11 fusion.10
113698632001The HoxB1 hexapeptide is a prefolded domain: implications for the Pbx1/Hox interaction.8
349036202022Phase Separation Mediates NUP98 Fusion Oncoprotein Leukemic Transformation.7
240216712013Essential role of PR-domain protein MDS1-EVI1 in MLL-AF9 leukemia.7
156492482005Retroviral insertional mutagenesis identifies oncogene cooperation.7
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 HOXA9.7
219374512011Amino-terminal enhancer of split (AES) interacts with the oncoprotein NUP98-HOXA9 and enhances its transforming ability.6
105832651999Molecular heterogeneity of the NUP98/HOXA9 fusion transcript in myelodysplastic syndromes associated with t(7;11)(p15;p15).6
90744071997Clinical, haematological and molecular studies in patients with chromosome translocation t(7;11): a study of four Chinese patients in Taiwan.6
345825592022The menin-MLL1 interaction is a molecular dependency in NUP98-rearranged AML.5
326644472020Disclosing the Interactome of Leukemogenic NUP98-HOXA9 and SET-NUP214 Fusion Proteins Using a Proteomic Approach.5
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.5
265428932016ETV6-LPXN fusion transcript generated by t(11;12)(q12.1;p13) in a patient with relapsing acute myeloid leukemia with NUP98-HOXA9.5
264182292016NUP98-HOXA9 bearing therapy-related myeloid neoplasm involves myeloid-committed cell and induces HOXA5, EVI1, FLT3, and MEIS1 expression.5
118414132002t(7;11)(p15;p15) Chronic myeloid leukaemia developed into blastic transformation showing a novel NUP98/HOXA11 fusion.5
102213431999Low frequency of rearrangements of the homeobox gene HOXA9/t(7;11) in adult acute myeloid leukemia.5
337245672021Reduction of leukemic burden via bone-targeted nanoparticle delivery of an inhibitor of C-chemokine (C-C motif) ligand 3 (CCL3) signaling.4
249711562014Blast crisis Ph+ chronic myeloid leukemia with NUP98/HOXA13 up-regulating MSI2.4
105653041999Chronic myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98/HOXA9 fusion.4
291052432018Multiplex fusion gene testing in pediatric acute myeloid leukemia.3
270331632016Transient potential receptor melastatin-2 (Trpm2) does not influence murine MLL-AF9-driven AML leukemogenesis or in vitro response to chemotherapy.3
238405802013Dynein Light Chain 1 (DYNLT1) Interacts with Normal and Oncogenic Nucleoporins.3
270606782016NUP98-PHF23 fusion is recurrent in acute myeloid leukemia and shares gene expression signature of leukemic stem cells.2
237547672013Atypical chronic myeloid leukemia harboring NUP98-HOXA9.2
157216372005NUP98 is fused to HOXA9 in a variant complex t(7;11;13;17) in a patient with AML-M2.2
346274232021[Clinical Characteristics and Prognosis of Adult Acute Myeloid Leukemia with NUP98 Gene Rearrangement].1
121389012002A case of acute myeloid leukemia with t(7;11)(p15;p15) mimicking myeloid crisis of chronic myelogenous leukemia.1
109368662000Down's syndrome with myelodysplastic syndrome showing t(7;11)(p13;p14).1
353816772022[A three-series case report and literature review of acute myeloid leukemia with t(7;11)(p15;p15)/NUP98-HOXA9].0
325146252021Faggot cells in acute myeloid leukemia with t(7;11)(p15;p15) and NUP98-HOXA9 fusion.0
348310742021The Retinoblastoma Tumor Suppressor Is Required for the NUP98-HOXA9-Induced Aberrant Nuclear Envelope Phenotype.0
343837402021A genetic screen in Drosophila uncovers the multifaceted properties of the NUP98-HOXA9 oncogene.0
324992422021Epigenetic and functional changes imposed by NUP98-HOXA9 in a genetically engineered model of chronic myeloid leukemia progression.0
328109692020[Acute myeloid leukemia with co-expression of TEL-ABL1 and NUP98-HOXA9 fusion genes: a case report and literature review].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
303783132018[Molecular Characteristics and Clinical Features of Adults with NUP98 Fusions in Acute Myeloid Leukemia].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
295510432018[The expression of NUP98-HOXA9 in adult acute myeloid leukemia: a case report].0
190056242008Additional acquisition of t(1;21)(p32;q22) in a patient relapsing with acute myelogenous leukemia with NUP98-HOXA9.0
151825302004[HUP98-HOXA9 transgenic mice are susceptible to N-ethyl-N-nitrosourea stimulation in leukemogenesis].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/favicon/deep-insight-explorer/css/template-card.css