Update in Pulmonary Vascular Diseases 2013

Complexity of macrophage migration inhibitory factor (MIF) and other angiogenic biomarkers profiling in pulmonary arterial hypertension

“…The interaction between inflammation, angiogenic factors and pulmonary vascular disease has been well established. 2 – 4 …”

“…The interaction between inflammation, angiogenic factors and pulmonary vascular disease has been well established. [2][3][4] Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine that is ubiquitously expressed in many cell types including the endothelium and several types of immune-related cells, including macrophages and T-cells, playing a significant role in regulation of innate immunity. This cytokine is constitutively expressed in macrophages and other innate immune cells, rapidly upregulating the production of inflammatory cytokines in the presence pathogen.…”

Therapeutic inhibition of HIF-2α reverses polycythemia and pulmonary hypertension in murine models of human diseases

“…Polycythemia, also known as erythrocytosis, can result from multiple causes, including mutations in proteins involved in oxygen sensing and erythropoiesis, such as in the erythropoietinerythropoietin receptor (EPO-EPOR), and mutations in hemoglobins (HBB, HBA), 2,3-bisphosphoglycerate mutase (BPGM), VHL, EGLN1 (prolyl hydroxylase domain-2 [PHD-2]), endothelial PAS-domain containing protein-1 (EPAS1, HIF-2a) and IRP1 genes that increase HIF-2a expression levels in renal tissues or endothelia. [1][2][3][4][5][6][7][8][9] Pulmonary hypertension can develop as a primary disease in which mutations in several disease genes have been identified, 10 including bone morphogenetic protein receptor 2 (BMPR2) 11 and VHL in Chuvash polycythemia, 12,13 but in many cases, the causes and pathophysiology are not well understood. Better therapies are needed for polycythemia 14 and pulmonary hypertension.…”