Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease of unknown origin with limited treatment options. Research suggests that the signaling molecule WNT5A plays a key role in the pathogenic process. Now, a group of scientists from Helmholtz Zentrum München in Germany, working with colleagues from the University of Denver, has taken a further step towards uncovering the mechanisms responsible for the development of fibrosis: they have shown that IPF is associated with the increase of extracellular vesicles (EVs) (sometimes called exosomes)* that relay WNT5A signals to cells in the lungs. The study, published online on July 25, 2018 in the American Journal of Respiratory and Critical Care Medicine, proposes a further pharmacological biomarker (the EVs) and a possible therapeutic approach (reducing the number of these EVs). The article is titled “Increased Extracellular Vesicles Mediate WNT-5A Signaling in Idiopathic Pulmonary Fibrosis.” Pulmonary fibrosis is associated with the increased formation of connective tissue in the lungs, resulting in scarring (fibrosis) of functional lung tissue. This leads to a decrease in the inner surface of the extremely fine alveoli and the extensibility of the lungs, which, in turn, impedes the intake of oxygen and the release of carbon dioxide. The result is impaired lung function. IPF is a particularly aggressive form of pulmonary fibrosis that cannot yet be attributed to a specific cause. The symptoms worsen rapidly. Existing drugs can slow progression of the disease, but are unable to stop it permanently. Research is therefore continuing to focus on elucidating the mechanisms underlying the pathological tissue changes associated with IPF.
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