Cell Autonomous and Non-cell Autonomous Regulation of SMC Progenitors in Pulmonary Hypertension.

ABSTRACT: Pulmonary hypertension is a devastating disease characterized by excessive vascular muscularization. We previously demonstrated primed platelet-derived growth factor receptor β⁺ (PDGFR-β⁺)/smooth muscle cell (SMC) marker⁺ progenitors at the muscular-unmuscular arteriole border in the normal lung, and in hypoxia-induced pulmonary hypertension, a single primed cell migrates distally and expands clonally, giving rise to most of the pathological smooth muscle coating of small arterioles. Little is known regarding the molecular mechanisms underlying this process. Herein, we show that primed cell expression of Kruppel-like factor 4 and hypoxia-inducible factor 1-α (HIF1-α) are required, respectively, for distal migration and smooth muscle expansion in a sequentia

SUBMITTER: Sheikh AQ 

PROVIDER: S-EPMC5959296 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature