NOTCH3 and CADASIL: Cys-sparing mutations are contradictory to the hypothesis that Cys-changing mutations in NOTCH3 are responsible for the disease mechanism in CADASIL; however, multiple case studies have identified Cys-sparing mutations in NOTCH3 (p.R61W, p.R75P, p.R213K, p.A1020P and p.T1098S) as a cause of CADASIL [35–40].