K channel regulator in IPF

Pulmonary vascular disease, including its clinical manifestation as elevated blood pressure in the lungs referred to as pulmonary hypertension (PH) is a common complication of idiopathic pulmonary fibrosis (IPF), particularly in advanced stages of the disease. PH occurs when the blood vessels in the lungs become blocked, leading to increased pressure in the pulmonary arteries. This forces the right side of the heart to work harder to pump blood through the lungs, eventually weakening the heart muscle and leading to heart failure. PH is recognised as a significant and independent prognostic factor for survival in patients with pulmonary fibrosis. Research in other types of pulmonary hypertension has shown that transmembrane proteins, such as ion channels, play a critical role in the development and progression of arterial remodelling an important pathological feature of elevated blood pressure in the lungs. However, the exact mechanisms by which this occurs in the context of pulmonary vascular disease, particularly pulmonary fibrosis, are still unclear and require further investigation. Methods: To investigate these mechanisms, we will utilise our extensive database of human lung tissue and cells and apply a wide range of advanced techniques. These include state-of-the-art methods in cell physiology, molecular biology, structural biology and proteomics. We will also collaborate with international partners to pool expertise and resources to ensure a comprehensive approach to understanding the underlying processes. Innovation: Our research has the potential to uncover a novel molecular mechanism responsible for vascular remodelling in pulmonary fibrosis, which could lead to new therapeutic targets for the treatment of the disease. The project will greatly benefit from our established expertise in pulmonary vascular disease and access to a large pool of human lung tissue. In addition, we will utilise sophisticated techniques to investigate how ion channel function affects physiological outcomes in vivo, all of which will be performed at the Medical University of Graz. The collaboration will be further strengthened by the expertise in structural biology at the University of Regensburg and the University of Graz. By fostering this international collaboration, we aim to discover new insights and potential new therapeutic strategies.