RNF213 regulates palmitate signaling through AAA+ and RZ-finger domains, a process enhanced by gain-of-function moyamoya disease-associated mutations.
RNF213 was originally identified as a susceptibility gene for Moyamoya disease (MMD), and its variants are also associated with other vascular occlusive disorders, including pulmonary arterial hypertension and large-artery atherosclerosis. RNF213 encodes a large protein with AAA+ ATPase and E3 ubiquitin ligase activiti...