PDGF-B and its receptor PDGF-Rβ, two more culprits for PFBC (Keller et al., 2013; Nicolas et al., 2013), are mainly and separately expressed in brain ECs and pericytes, and they play an essential role in regulating the functions of BBB-ECs. This evidence concerns the gene PDGFB and bilateral striopallidodentate calcinosis.