Publication: Regulation mechanisms for the heterodimeric transcription factor, PEBP2lCBF
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Date
1999
Authors
Bae, S.C. ; Ito, Y.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Members of the new PEBP2 (Polyomavirus
Enhancer Binding Protein 2) family of heterodimeric
transcriptional regulatory protein are composed of two
subunits, a and B. One of the genes encoding the a
subunit, AMLlIPEBP2aB, was identified at the
breakpoints of various chromosome translocations,
including t(8;21) and t(12;21) associated with acute
myeloid leukemia and acute lymphoblastic leukemia,
respectively. The gene encoding the B subunit
(PEBP2flCBFB) was also shown to be the target of the
inversion of chromosome 16, another chromosomal
anomaly associated with acute myeloid leukemia.
Targeted disruption of either the AmlllPebp2aB or
PebpZflICbfb gene resulted in strikingly similar
phenotypes such as lack of definitive hematopoiesis of
the fetal liver and accompanying hemorrhage of the
central nervous system. These observations suggest that
both a and l3 subunits of PEBP2 are indispensable for its
in vivo function. However, the heterodimerization of the
a and B subunit does not seem to occur readily
suggesting that their capacity to associate might be an
important rate limiting step in PEBP2 site-dependent
transcription regulation. In this review, we concentrate
on the possible regulatory mechanisms of PEBP2
activity in relation to leukemogenesis.
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