NUP98 gene fusions and hematopoietic malignancies: common themes and new biologic insights

Gough, Sheryl M., Slape, Christopher I. and Aplan, Peter D. (2011) NUP98 gene fusions and hematopoietic malignancies: common themes and new biologic insights. Blood, 118 24: 6247-6257. doi:10.1182/blood-2011-07-328880


Author Gough, Sheryl M.
Slape, Christopher I.
Aplan, Peter D.
Title NUP98 gene fusions and hematopoietic malignancies: common themes and new biologic insights
Formatted title
NUP98 gene fusions and hematopoietic malignancies: common themes and new biologic insights
Journal name Blood   Check publisher's open access policy
ISSN 0006-4971
1528-0020
Publication date 2011-12-08
Year available 2011
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1182/blood-2011-07-328880
Open Access Status Not yet assessed
Volume 118
Issue 24
Start page 6247
End page 6257
Total pages 11
Place of publication Washington, DC, United States
Publisher American Society of Hematology
Language eng
Formatted abstract
Structural chromosomal rearrangements of the Nucleoporin 98 gene (NUP98), primarily balanced translocations and inversions, are associated with a wide array of hematopoietic malignancies. NUP98 is known to be fused to at least 28 different partner genes in patients with hematopoietic malignancies, including acute myeloid leukemia, chronic myeloid leukemia in blast crisis, myelodysplastic syndrome, acute lymphoblastic leukemia, and bilineage/ biphenotypic leukemia. NUP98 gene fusions typically encode a fusion protein that retains the amino terminus of NUP98; in this context, it is important to note that several recent studies have demonstrated that the amino-terminal portion of NUP98 exhibits transcription activation potential. Approximately half of the NUP98 fusion partners encode homeodomain proteins, and at least 5 NUP98 fusions involve known histone-modifying genes. Several of the NUP98 fusions, including NUP98-homeobox (HOX)A9, NUP98-HOXD13, and NUP98-JARID1A, have been used to generate animal models of both lymphoid and myeloid malignancy; these models typically up-regulate HOXA cluster genes, including HOXA5, HOXA7, HOXA9, and HOXA10. In addition, several of the NUP98 fusion proteins have been shown to inhibit differentiation of hematopoietic precursors and to increase self-renewal of hematopoietic stem or progenitor cells, providing a potential mechanism for malignant transformation.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collection: School of Chemistry and Molecular Biosciences
 
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