{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T13:50:36Z","timestamp":1790603436292,"version":"4.1.0"},"reference-count":23,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:sec><jats:title>OBJECTIVE<\/jats:title><jats:p>Moyamoya disease (MMD) is a unique cerebrovascular disorder characterized by the progressive occlusion of the bilateral internal carotid arteries. Endothelial colony-forming cells (ECFCs), previously termed \u201cendothelial progenitor cells,\u201d play an important role in the pathogenesis of MMD. In this study, the authors performed morphological and functional studies of the mitochondria of ECFCs from patients with MMD to present new insights into the pathogenesis of the disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>The morphology of ECFCs from 5 MMD patients and 5 healthy controls was examined under both a transmission electron microscope and a confocal laser scanning microscope. The oxygen consumption rates (OCRs), mitochondrial membrane potentials (MMPs), intracellular Ca<jats:sup>2+<\/jats:sup> concentrations, mitochondrial enzyme activities, and reactive oxygen species (ROS) levels were measured. Functional activity of the ECFCs was evaluated using a capillary tube formation assay.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>The ECFCs from the MMD patients displayed a disrupted mitochondrial morphology, including a shorter and more circular shape. The ECFC mitochondria from the MMD patients exhibited functional abnormalities, which were assessed as a decreased OCR and an increased intracellular Ca<jats:sup>2+<\/jats:sup> concentration. Moreover, the ECFCs from MMD patients showed increased ROS levels. Interestingly, treatment with an ROS scavenger not only reversed the mitochondrial abnormalities but also restored the angiogenic activity of the ECFCs from the MMD patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>The mitochondria of ECFCs from MMD patients, as compared with those from healthy patients, exhibited morphological and functional abnormalities. This finding suggests that the mitochondrial abnormalities may have a role in the pathogenesis of MMD.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2017.5.jns17147","type":"journal-article","created":{"date-parts":[[2017,12,8]],"date-time":"2017-12-08T14:59:24Z","timestamp":1512745164000},"page":"1151-1159","source":"Crossref","is-referenced-by-count":43,"title":["Mitochondrial abnormalities related to the dysfunction of circulating endothelial colony-forming cells in moyamoya disease"],"prefix":"10.3171","volume":"129","author":[{"given":"Jung Won","family":"Choi","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung Min","family":"Son","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Biomedical Sciences;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inhee","family":"Mook-Jung","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Biomedical Sciences;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youn Joo","family":"Moon","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Yeoun","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyu-Chang","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyun-Seung","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Hoon","family":"Phi","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seung Ah","family":"Choi","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sangjoon","family":"Chong","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jayoung","family":"Byun","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Biomedical Sciences;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seung-Ki","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Seoul National University Children\u2019s Hospital;"},{"name":"Department of Neurosurgery, Seoul National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"J Neurol Neurosurg Psychiatry","doi-asserted-by":"publisher","first-page":"900","DOI":"10.1136\/jnnp.2007.130666","article-title":"Novel epidemiological features of moyamoya disease","volume":"79","author":"Baba T","year":"2008","unstructured":"Baba T, Houkin K, Kuroda S: Novel epidemiological features of moyamoya disease. 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