Finally, we show that elevated ET-1 protein in the adult human SVZ of Cathepsin A-related arteriopathy with strokes and leukoencephalopathy (CARASAL) patients correlates with increased numbers of astrocytes and OPCs, indicating that ET-1 signaling plays a similar role in the human SVZ. Here, EDN1 is linked to cathepsin a-related arteriopathy-strokes-leukoencephalopathy.