Decreased level and defective function of circulating endothelial progenitor cells in children with moyamoya disease
Jin Hyun Kim1, Ji-Hye Jung, Ji Hoon Phi
1Clinical Research Institute, Gyeongsang National University Hospital, Jinju, Gyeongnam, Republic of Korea.
Insights
Children with moyamoya disease (MMD) have fewer circulating endothelial progenitor cells (EPCs) with impaired function. These findings suggest a potential role for EPCs in MMD pathogenesis.
Area of Science:
- Cardiovascular Research
- Regenerative Medicine
- Pediatric Neurology
Background:
- Endothelial progenitor cells (EPCs) are crucial for neovascularization and may mitigate ischemic conditions.
- Moyamoya disease (MMD) is a significant pediatric cerebrovascular disorder.
Purpose of the Study:
- To characterize circulating EPCs in pediatric patients with MMD before surgical intervention.
- To compare EPC levels and function between MMD patients and healthy controls.
Main Methods:
- Isolation and culture of peripheral blood mononuclear cells (PBMNCs) from 28 MMD children and 12 healthy controls.
- Analysis of cell phenotype (CD34+, CD133+, KDR+) and EPC cluster formation on days 0 and 7.
- Functional assays evaluating tube formation and senescence.
Main Results:
- Children with MMD exhibited significantly reduced levels of CD34+, CD133+, and KDR+ cells and fewer EPC clusters.
- EPCs from MMD patients showed diminished tube formation capacity and an increased senescent-like phenotype.
- Temporal changes in CD34+ and KDR+ cell populations differed between MMD patients and controls.
Conclusions:
- Circulating EPCs are decreased in quantity and exhibit defective function in children with MMD.
- These findings suggest a potential association between circulating EPC dysfunction and the pathogenesis of moyamoya disease.
Abstract:
Circulating endothelial progenitor cells (EPCs) play an important role in physiological and pathological neovascularization and may be involved in attenuating ischemic diseases. This study aimed to characterize circulating EPCs in moyamoya disease (MMD), one of the most common pediatric cerebrovascular diseases. Twenty-eight children with MMD prior to any surgical treatment and 12 healthy volunteers were recruited. Peripheral blood mononuclear cells (PBMNCs) were isolated and cultured in endothelial cell growth medium. Temporal change of phenotype of cells was analyzed on days 0 and 7. The formation of EPC clusters was evaluated on day 7. The CD34(+), CD133(+), and KDR(+) cells, and the number of EPC clusters was significantly reduced in children with MMD. In controls, CD34(+) cells were significantly decreased on day 7 compared with day 0, but in MMD they were only slightly decreased. The change in KDR(+) cells on day 7 compared with day 0 was the reverse of that for CD34(+) cells. Functional assay of EPC demonstrated less tube formation and increased senescent-like phenotype in children with MMD. Analysis of the circulating EPCs of MMD children reveals decreased level and defective function. This study suggests that circulating EPCs may be associated with MMD pathogenesis.
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