background:
CP2 family gene products are reported to be involved in a-globin gene expression. Mouse CP2c (also known as a-CP2/CP2/LSF/UBP-1) is a homologue of human LBP-1c with 96% amino acid sequence identity. CP2c was initially identified as an activator of the mouse a-globin gene, which binds to the consensus DNA-binding sequence CNRG-N6-CNR(G/C) within a promoter element overlapping the CCAAT box. The transcriptional activity of CP2c was increased during induced differentiation of MEL cells and associated globin gene transcription. CP2c is also likely to be involved in regulation of non-globin erythroid-specific genes. It was reported that congenital erythropoietic porphyria was caused by the mutations in GATA-1 and CP2c binding sites within the promoter of the uroporphyrinogen III synthase gene, the fourth enzyme in the heme biosynthetic pathway.
Function:
Binds a variety of cellular and viral promoters including fibrinogen, alpha-globin, SV40 and HISLV1 promoters. Activation of the alpha-globin promoter in erythroid cells is via synergistic interaction with UBP1. Functions as part of the SSP (stage selector protein) complex. Facilitates the interaction of the gamma-globin genes with enhancer elements contained in the locus control region in fetal erythroid cells. Interacts by binding to the stage selector element (SSE) in the proximal gamma-globin promoter.
Subunit:
Binds to DNA as a dimer, isoform 3 does not bind to DNA or affect the binding of isoform 1 to DNA. Interacts with UBP1 and PIAS1, and is probably part of a complex containing TFCP2, UBP1 and PIAS1. Component of the SSP (stage selector protein) complex, which appears to be a heteromer of TFCP2 and 2 copies of NFE4.
Subcellular Location:
Nuclear.
Tissue Specificity:
Ubiquitous. Expressed in brain, ovary, kidney, thymus, spleen, liver, adrenal, heart and lung (at protein level).
Similarity:
Belongs to the grh/CP2 family. CP2 subfamily.
Database links:
Entrez Gene: 7024 Human
Entrez Gene: 21422 Mouse
Entrez Gene: 315309 Rat
Omim: 189889 Human
SwissProt: Q7T2U9 Chicken
SwissProt: Q2560 Human
SwissProt: Q9ERA0 Mouse
Unigene: 48849 Human
Unigene: 219040 Mouse
Unigene: 202290 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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