The role of NUP98 gene fusions in hematologic malignancy

Slape, C and Aplan, PD (2004) The role of NUP98 gene fusions in hematologic malignancy. Leukemia and Lymphoma, 45 7: 1341-1350. doi:10.1080/10428190310001659325


Author Slape, C
Aplan, PD
Title The role of NUP98 gene fusions in hematologic malignancy
Journal name Leukemia and Lymphoma   Check publisher's open access policy
ISSN 1042-8194
Publication date 2004
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1080/10428190310001659325
Volume 45
Issue 7
Start page 1341
End page 1350
Total pages 10
Language eng
Subject 1306 Cancer Research
2720 Hematology
2730 Oncology
Abstract Chromosomal aberrations occur with great frequency and some specificity in leukemia and other hematologic malignancies. The most common outcome of these rearrangements is the formation of a fusion gene, comprising portions of 2 genes normally present in the cell. These fusion proteins are presumed to be oncogenic; in many cases, animal models have proven them to be oncogenic. One of the most promiscuous fusion partner genes is the newly identified NUP98 gene, located on chromosome 11p15.5, which to date has been observed fused to 15 different fusion partners. NUP98 encodes a 98 kD protein that is an important component of the nuclear pore complex, which mediates nucleo-cytoplasmic transport of protein and RNA. The fusion partners of NUP98 form 2 distinct groups: homeobox genes and non-homeobox genes. All NUP98 fusions join the N-terminal GLFG repeats of NUP98 to the C-terminal portion of the partner gene, which, in the case of the homeobox gene partners, includes the homeodomain. Clinical findings are reviewed here, along with the findings of several in vivo and in vitro models have been employed to investigate the mechanisms by which NUP98 fusion genes contribute to the pathogenesis of leukemia.
Keyword 11p15.5 translocation
HOX
Leukemia
Myelodysplastic syndrome
NUP98
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Unknown

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
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