Relative mRNA expression of C1r, C1s, C2, CFB, C4A, CFH, SERPING1 and the protein level of C1r in supernatants of PXE and control fibroblasts. Fibroblasts from PXE patients (PXEF) and healthy control donors (NHDF) were cultivated for 72 h in 10% LPDS medium with DMSO as vehicle (untreated) or 1 µM BA. (A) Gene expression and (B) protein level of C1r in PXEF (black) (n = 4) and NHDF (white) (n = 4). mRNA expression of (C) C1s, (D) C2, (E) CFB, (F) C4A, (G) CFH, (H) and SERPING1 in PXEF (black) (n = 4) and NHDF (white) (n = 4). Data are shown as mean ± SEM. **** p ≤ 0.001, *** p ≤ 0.002, ** p ≤ 0.01, * p ≤ 0.05, ns p > 0.05.