Typical examples include mutations in PDGFB or PDGFRB growth factor/receptor causing primary familial brain calcification (idiopathic basal ganglia calcification, Fah’s disease), mutations in receptor NOTCH3 associated with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and tight junction OCLN in band-like calcification with simplified gyration and polymicrogyria (BLC-PMG), and mutations in basement membrane COL4A1 and COL4A2 underlying cerebral small vessel disease or LAMA2 in congenital muscular dystrophy 1A (MDC1A). This evidence concerns the gene OCLN and bilateral striopallidodentate calcinosis.