Abstract
Pulmonary arterial hypertension (PAH) is characterized by significant and aberrant cellular proliferation of the endothelium and smooth muscle that results in an angioproliferative vasculopathy with complex involvement of apoptosis and inflammatory pathways. We previously demonstrated Pre-B-Cell Colony Enhancing Factor (PBEF) to be a biomarker and candidate gene in inflammatory lung injury (Ye S et al 2005; Hong et al 2008), with extracellular proinflammatory cytokine-like, NF-kB-mediated activity as well as intracellular enzymatic activity as a phosphoribosyltransferase which regulates intracellular NAD levels (NAMPT activity) and inhibits apoptosis. Thus, we hypothesize that PBEF might be a potential therapeutic target in PAH.
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