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2013 ; 3
(9
): a015412
Nephropedia Template TP
Katsumura KR
; DeVilbiss AW
; Pope NJ
; Johnson KD
; Bresnick EH
Cold Spring Harb Perspect Med
2013[Sep]; 3
(9
): a015412
PMID23838521
show ga
The physiological switch in expression of the embryonic, fetal, and adult ?-like
globin genes has garnered enormous attention from investigators interested in
transcriptional mechanisms and the molecular basis of hemoglobinopathies. These
efforts have led to the discovery of cell type-specific transcription factors,
unprecedented mechanisms of transcriptional coregulator function, genome biology
principles, unique contributions of nuclear organization to transcription and
cell function, and promising therapeutic targets. Given the vast literature
accrued on this topic, this article will focus on the master regulator of
erythroid cell development and function GATA-1, its associated proteins, and its
frontline role in controlling hemoglobin synthesis. GATA-1 is a crucial regulator
of genes encoding hemoglobin subunits and heme biosynthetic enzymes.
GATA-1-dependent mechanisms constitute an essential regulatory core that
nucleates additional mechanisms to achieve the physiological control of
hemoglobin synthesis.
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