Endothelial transcription factor GATA-2 (UniProt: P23769; also known as GATA-binding protein 2) is encoded by the GATA2 gene (Gene ID: 2624) in human. GATA2 is a zinc-finger transcriptional activator that regulates endothelin-1 gene expression in endothelial cells. It binds to the consensus sequence 5'-AGATAG-3'. GATA2 is expressed in embryonic brain and liver, endothelial cells, and in erythroid cells. GATA2 mutations appear to cause loss of function of the mutated allele leading to haploinsufficiency, which results in immunodeficiency characterized by significantly reduced or absent monocytes, B-lymphocytes, natural killer lymphocytes, and circulating and tissue dendritic cells. However, it does not affect the T-cell number. This deficiency leads to increased susceptibility to mycobacterial, papillomavirus, and opportunistic fungal infections. Certain mutations in GATA2 are also linked to lymphedema that results in swelling of the extremities due to altered lymphatic flow.