Increased hyaluronic acid content in idiopathic pulmonary arterial hypertension
作者： E. Papakonstantinou , F. M. Kouri , G. Karakiulakis , I. Klagas , O. Eickelberg
DOI: 10.1183/09031936.00159507
关键词:
摘要: Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease characterised by elevated blood pressure in the circulation. Initial vasoconstriction, proliferation of smooth muscle cells (PASMC) and increased deposition extracellular matrix (ECM) contribute to pathological remodelling arterioles IPAH. Glycosaminoglycans (GAGs), components ECM, control cellular differentiation, but their expression IPAH remains elusive. In present study, GAG was investigated lungs patients with or transplant donors, localisation GAG-metabolising enzymes were analysed vivo vitro. A significant increase hyaluronic acid (HA) detected lungs, associated hyaluronan synthase (Has)1 decreased hyaluronoglucosaminidase 1 gene expression, as assessed quantitative RT-PCR Western blotting. HAS1 protein localised PASMC HA observed remodelled arteries Transforming growth factor-beta1, profibrotic factor, led secretion primary PASMC. The results demonstrate an content idiopathic expression. Synergistic regulation glycosaminoglycan-metabolising favour accumulation may, thus, regulate vascular lungs.
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