Abstract
GATA transcription factors are zinc finger DNA binding proteins that regulate transcription during development and cell differentiation. The three important GATA transcription factors GATA1, GATA2 and GATA3 play essential roles in the development and maintenance of hematopoietic systems. GATA1 is required for the erythroid and megakaryocytic commitment during hematopoiesis. GATA2 is crucial for the proliferation and survival of early hematopoietic cells, and is also involved in lineage specific transcriptional regulation as the dynamic partner of GATA1. GATA3 plays an essential role in T lymphoid cell development and immune regulation. As a result, mutations in genes encoding the GATA transcription factors or alteration in the protein expression level or their function have been linked to a variety of human hematologic disorders. In this review, we summarized the current knowledge regarding the disrupted biologic function of GATA in various hematologic disorders.
Highlights
Hematopoiesis is a finely modulated process controlled by numerous transcriptional and signaling factors
The C terminal zinc finger binds to the GATA consensus sites, whereas the N terminal zinc finger promotes the interaction between GATA and specific DNA sequences through stabilizing the association with zinc finger protein cofactors [1, 2]
GATA2 mutations have been implicated in a group of complex clinical entities with overlapping features including familial myelodysplastic syndrome/acute myeloid leukemia (AML), Emberger syndrome, and MonoMAC syndrome characterized by peripheral monocytopenia, B- and NK-cell lymphocytopenia, increased susceptibility to mycobacterium infections and a predisposition to acute myeloid leukemia and myelodysplastic syndrome
Summary
Hematopoiesis is a finely modulated process controlled by numerous transcriptional and signaling factors. The interaction of GATA1 with N terminal zinc finger cofactors such as FOG-1 (Friend of GATA) is essential for megakaryocyte or erythroid development [6]. Mutations of GATA1 in TAM and AMKL are clustered in exon 2 and result in a truncated GATA1 protein from a premature stop codon that lacks the N-terminal activation domain.
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