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ID 112440
Morimoto, Takaaki Kyoto University
Enmi, Jun-ichiro National Cerebral and Cardiovascular Center
Hattori, Yorito National Cerebral and Cardiovascular Center
Iguchi, Satoshi National Cerebral and Cardiovascular Center
Saito, Satoshi National Cerebral and Cardiovascular Center
Harada, Kouji H. Kyoto University
Okuda, Hiroko Kyoto University
Mineharu, Yohei Kyoto University
Youssefian, Shohab Kyoto University
Iida, Hidehiro National Cerebral and Cardiovascular Center
Miyamoto, Susumu Kyoto University
Ihara, Masafumi National Cerebral and Cardiovascular Center
Kobayashi, Hatasu Kyoto University|Chubu University
Koizumi, Akio Kyoto University
Content Type
Journal Article
RNF213 is a susceptibility gene for moyamoya disease, yet its exact functions remain unclear. To evaluate the role of RNF213 in adaptation of cerebral blood flow (CBF) under cerebral hypoperfusion, we performed bilateral common carotid artery stenosis surgery using external microcoils on Rnf213 knockout (KO) and vascular endothelial cell-specific Rnf213 mutant (human p.R4810K orthologue) transgenic (EC-Tg) mice. Temporal CBF changes were measured by arterial spin-labelling magnetic resonance imaging. In the cortical area, no significant difference in CBF was found before surgery between the genotypes. Three of eight (37.5%) KO mice died after surgery but all wild-type and EC-Tg mice survived hypoperfusion. KO mice had a significantly more severe reduction in CBF on day 7 than wild-type mice (KO, 29.7% of baseline level; wild-type, 49.3%; p = 0.038), while CBF restoration on day 28 was significantly impaired in both KO (50.0%) and EC-Tg (56.1%) mice compared with wild-type mice (69.5%; p = 0.031 and 0.037, respectively). Changes in the subcortical area also showed the same tendency as the cortical area. Additionally, histological analysis demonstrated that angiogenesis was impaired in both EC-Tg and KO mice. These results are indicative of the essential role of RNF213 in the maintenance of CBF.
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Scientific Reports
Springer Nature
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© The Author(s) 2018
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Medical Sciences