Conference: 2010 International PHA Conference and Scientific Sessions
Release Date: 06.22.2010
Presentation Type: Scientific Sessions
Plexiform lesions are unique findings in the pulmonary blood vessels of patients with severe pulmonary hypertension (PH). Comprised of excessive numbers of endothelial cells, these plexiform lesions may form as a result of excessive activation of endothelial proliferation. During normal blood vessel development, different growth factors (including hedgehog, bone morphogenic proteins, fibroblast growth factors, and
vascular endothelial growth factors) regulate expansion of endothelial cell number, formation of endothelial tubes, and maturation of developing endothelial tubes into intact blood vessels. By studying how growth factors control normal blood vessel development, we can better understand how disruption of normal vascular homeostasis either by mutation or injury can lead to diseases such as PH. In this session, we will
discuss the growth factors and signaling pathways that regulate angiogenesis and blood vessel development from the earliest stages of embryogenesis to the formation of mature vessels late in development. We will then examine how defects in the normal angiogenesis program can lead to abnormalities in the circulatory system. Based on what we have learned from both experimental animal models and human studies, we
will then try to connect the molecular mechanisms of angiogenesis with the disease processes observed in patients with PH. By understanding the specific processes regulating both normal and disease-related blood vessel development at the molecular and cellular level, we may be able to design more directed therapies for prevention and treatment of PH and other vascular diseases.