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Background and purpose: Moyamoya disease (MMD) is a unique cerebrovascular occlusive disease that is characterized by progressive stenosis and negative remodeling of the distal internal carotid artery (ICA). We hypothesized that caveolin-1, a protein that controls the regulation of endothelial vesicular trafficking and signal transduction, is associated with negative remodeling in MMD.Methods: We prospectively recruited 77 consecutive patients with MMD (49 bilateral and 28 unilateral MMD) diagnosed by conventional angiography. Seventeen patients with intracranial atherosclerotic stroke and no RNF213 mutation served as controls. Distal ICA outer diameters were examined with high-resolution MRI. We evaluated whether the degree of negative remodeling in MMD patients was associated with RNF213 polymorphism, caveolin-1 levels, or various clinical and vascular risk factors.Results: The RNF213 variant was observed in 49 (63.6%) patients with MMD. The serum caveolin-1 (ng/mL) level was lower in MMD patients than in controls (0.47±0.29 vs. 0.86±0.68, P=0.034). The mean ICA diameter was 2.48±0.98 mm (range 0.00-4.76) for the 126 affected distal ICAs in MMD patients and 3.84±0.42 mm for asymptomatic ICAs in controls. After adjusting for possible confounders, male sex (coefficient, 0.396; P=0.029), clinical presentation with ischemic stroke (coefficient, -0.733; P<0.001), and caveolin-1 level (coefficient, 1.018; P<0.001) were independently associated with distal ICA diameter in MMD patients.Conclusion: Our findings suggest that caveolin-1 may play a major role in arterial negative remodeling in MMD patients. Future studies exploring caveolin-1 as a therapeutic target in MMD are warranted.