Figure 3.

Actin-bundling protein expression in fibroblasts and osteoblast-like cells. The expression of PLS3, ACTN1, ACTN4, and FSCN1 was measured in cell lines from 5 healthy donors and 6 PLS3-variant patients on day 21. Protein expression was detected in whole-cell lysates of (A) primary fibroblasts (FB) and (B) osteoblast-like cells (OB) by Western blotting. (C,D) Quantification of Western blot results; the PLS3 expression of P6 is indicated separately. (E–H) Relative gene expression was measured by qPCR and results were normalized based on the expression of GAPDH (error bars indicate standard deviation per group, * p < 0.05, ** p < 0.01, *** p < 0.001, **** p < 0.0001).