C/EBPβ suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL

William T. Choi, Matthew R. Folsom, Mohammed F. Azim, Claus Meyer, Eric Kowarz, Rolf Marschalek, Nikolai A. Timchenko, Rizwan C. Naeem, Dean A. Lee

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Translocations involving the mixed-lineage leukemia gene (MLL) confer a poor prognosis in acute leukemias. In t(1;11)(q21;q23), MLL is fused reciprocally with AF1q. Here we describe a t(1;11)(q21;q23) with a secondary event involving insertion of the telomeric portion of MLL into the p arm of chromosome 11 (11p11). We show that this latter event interrupts the CUG triplet repeat binding protein-1 (CUGBP1) gene, a translational enhancer of C/EBPβ. We then showed that these cells have reduced expression of CUGBP1 and C/EBPβ when compared to other AML blasts. This is the first report to describe insertional disruption of the CUGBP1 gene and to suggest a role for the CUGBP1-C/EBPβ pathway in leukemogenesis.

Original languageEnglish (US)
Pages (from-to)108-114
Number of pages7
JournalCancer Genetics and Cytogenetics
Volume177
Issue number2
DOIs
StatePublished - Sep 2007
Externally publishedYes

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Trinucleotide Repeats
Carrier Proteins
Leukemia
Genes
Protein C
Chromosomes, Human, Pair 11

ASJC Scopus subject areas

  • Cancer Research
  • Genetics
  • Molecular Biology

Cite this

Choi, W. T., Folsom, M. R., Azim, M. F., Meyer, C., Kowarz, E., Marschalek, R., ... Lee, D. A. (2007). C/EBPβ suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL. Cancer Genetics and Cytogenetics, 177(2), 108-114. https://doi.org/10.1016/j.cancergencyto.2007.07.002

C/EBPβ suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL. / Choi, William T.; Folsom, Matthew R.; Azim, Mohammed F.; Meyer, Claus; Kowarz, Eric; Marschalek, Rolf; Timchenko, Nikolai A.; Naeem, Rizwan C.; Lee, Dean A.

In: Cancer Genetics and Cytogenetics, Vol. 177, No. 2, 09.2007, p. 108-114.

Research output: Contribution to journalArticle

Choi, WT, Folsom, MR, Azim, MF, Meyer, C, Kowarz, E, Marschalek, R, Timchenko, NA, Naeem, RC & Lee, DA 2007, 'C/EBPβ suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL', Cancer Genetics and Cytogenetics, vol. 177, no. 2, pp. 108-114. https://doi.org/10.1016/j.cancergencyto.2007.07.002
Choi, William T. ; Folsom, Matthew R. ; Azim, Mohammed F. ; Meyer, Claus ; Kowarz, Eric ; Marschalek, Rolf ; Timchenko, Nikolai A. ; Naeem, Rizwan C. ; Lee, Dean A. / C/EBPβ suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL. In: Cancer Genetics and Cytogenetics. 2007 ; Vol. 177, No. 2. pp. 108-114.
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AB - Translocations involving the mixed-lineage leukemia gene (MLL) confer a poor prognosis in acute leukemias. In t(1;11)(q21;q23), MLL is fused reciprocally with AF1q. Here we describe a t(1;11)(q21;q23) with a secondary event involving insertion of the telomeric portion of MLL into the p arm of chromosome 11 (11p11). We show that this latter event interrupts the CUG triplet repeat binding protein-1 (CUGBP1) gene, a translational enhancer of C/EBPβ. We then showed that these cells have reduced expression of CUGBP1 and C/EBPβ when compared to other AML blasts. This is the first report to describe insertional disruption of the CUGBP1 gene and to suggest a role for the CUGBP1-C/EBPβ pathway in leukemogenesis.

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