Increased SRC activity in HPAH patient-derived LO-EPCs.
A, LO-EPCs express endothelial cell markers. Patient-derived LO-EPCs are positive for two endothelial cell markers, CD146 and CD31, and negative for leukocyte and macrophage markers CD45 and CD14 as analyzed by FACS. B, Dio-Ac-LDL uptake. LO-EPCs take up endothelium-specific Dio-Ac-LDL and exhibit cobblestone-like morphology. Bovine aortic smooth muscle cells (SMCs) serve as negative controls. C, increased SRC activity in HPAH patient-derived LO-PECs. A Western blot depicts basal protein expression of Tyr(P)14- and total CAV-1 and Tyr(P)416- and total SRC in LO-PECs from two disease-free controls (C1 and C2), one HPAH patient (BMPR2 V299 FsX1), and one IPAH patient negative for BMPR2 mutations. β-Actin serves as a loading control. Gels were run and probed as follows: Gel 1, Tyr(P)14-CAV-1, Tyr(P)416-SRC, and β-actin; Gel 2, CAV-1 and SRC. DIC, differential interference contrast.