t(3;11)(q29;p15) NUP98/IQCG
2009-02-01 Li-Ting Chen , Sai-Juan Chen Affiliation1.Shanghai Institute of Hematology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, P.R. China
Clinics and Pathology
Phenotype stem cell origin
Only one case available; BM showed 82.5% blast cells mostly with myeloid features. Immunophenotype: CD34+ cells count 35.6%, CD13+ 25.9%, CD33+ 85.4%, MPO+ 20.6% and CD3+ 65.9%, CD7+ 96.5%.
Clinics
Only one case available; the patient was a 42-year-old female at diagnosis. After the chemotherapy with vincristine, cytarabine and mitoxantrone, she achieved complete remission (CR), but relapsed after 4 months, and died from intracranial hemorrhage at the 22nd months.
Prognosis
The prognostic implication of t(3;11)(q29q13;p15)del(3)(q29) remains to be defined.
Genes Involved and Proteins
Gene name
NUP98 (nucleoporin 98 kDa)
Location
11p15.4
Protein description
Nucleoporin 98, a 98 kDa component of the nuclear pore complex, which mediates nucleo-cytoplasmic transport of protein and RNA.
Gene name
IQCG (IQ motif containing G)
Location
3q29
Protein description
443 amino acids.
Result of the Chromosomal Anomaly
Description
5 NUP98 - 3 IQCG; Molecular analysis showed that exon 13 of NUP98 is fused in-frame to exon 10 of IQCG.It fuses the GLFG repeat domains of NUP98 to one coiled-coil domain and IQ motif of IQCG.
Expression localisation
Nuclear localization.
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 27864780 | 2016 | Inhibition of the nuclear export of p65 and IQCG in leukemogenesis by NUP98-IQCG. | 0 |
| 18084320 | 2008 | 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. | 0 |
| 26675333 | 2016 | Human NUP98-IQCG fusion protein induces acute myelomonocytic leukemia in mice by dysregulating the Hox/Pbx3 pathway. | 0 |
| 24787902 | 2014 | Functional and molecular features of the calmodulin-interacting protein IQCG required for haematopoiesis in zebrafish. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 18084320 | 2008 | 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. | Pan Q et al |
Summary
Fusion gene
NUP98/IQCG NUP98 (11p15.4) IQCG (3q29) M t(3;11)(q29;p15)|NUP98/IQCG NUP98 (11p15.4) IQCG (3q29) TIC
Complex translocation t(3;11)(q29q13;p15)del(3)(q29). A: Red arrow indicates der(3); green arrow indicates der(11). The chromosomes displaying only red color or green color (yellow arrows) are normal chromosomes 11 and 3, respectively. Insert: Representative partial karyotype showing t(3;11).
Citation
Li-Ting Chen ; Sai-Juan Chen
t(3;11)(q29;p15) NUP98/IQCG
Atlas Genet Cytogenet Oncol Haematol. 2009-02-01
Online version: http://atlasgeneticsoncology.org/haematological/1508/t(3;11)(q29;p15)-nup98-iqcg
