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doi:10.1056/NEJMoa1301467

Best practice guidelines on genetic diagnostics of Facioscapulohumeral muscular dystrophy: Workshop 9th June 2010, LUMC, Leiden, The Netherlands

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Endogenous proteases, of which matrix metalloproteinases (MMPs) are the predominant kind in the lung, control the destruction of ECM ( 6.2 Transforming growth factor- is essential for fibrosis Transforming growth factor- is a cytokine known to increase ECM production ( in vitro expression of -SMA protein were markedly enhanced by SMAD3 overexpression ( 7 Oxidative stress and necroptosis interact to promote airway remodeling Cigarette-smoke induces numerous mechanisms of cell death, and the pattern of cell death dictates when inflammatory reactions start