zinc finger protein 521 (early hematopoietic Zn+2 finger protein, LYST-interacting protein 3, ZNF521, EHZF, LIP3)
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Function
- transcription factor that can both act as an activator or a repressor depending on the context
- involved in BMP signaling & in the regulation of the immature compartment of the hematopoietic system
- associates with SMADs in response to BMP2 leading to activate transcription of BMP target genes
- acts as a transcriptional repressor via its interaction with EBF1, a transcription factor involved specification of B-cell lineage, thus preventing EBF1 binding of DNA & activation of target genes
- interacts with SMAD1 & SMAD4
Structure
- uses different DNA- & protein-binding zinc fingers to regulate the distinct BMP-Smad & hematopoietic system
- belongs to the krueppel C2H2-type zinc-finger protein family
- contains 30 C2H2-type zinc fingers
Compartment
Expression
- predominantly expressed in hematopoietic cells
- present in organs & tissues that contain stem & progenitor cells, myeloid &/or lymphoid: placenta, spleen, lymph nodes, thymus, bone marrow & fetal liver
- within the hematopoietic system, abundant in CD34+ cells but undetectable in mature peripheral blood leukocytes
- levels rapidly decrease during the differentiation of CD34+ cells in response to hemopoietins
Pathology
- chromosomal translocation t(9;18)(p13;q11.2) involving ZNF521 with PAX5 is found in acute lymphoblastic leukemia
translocation generates the PAX5-ZNF521 oncogene consisting of N-terminus of PAX5 & C-terminus of ZNF521