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Published on: January 28, 2020
Pathological Circulating Factors in Moyamoya Disease
Yao-Ching Fang1, Ling-Fei Wei1, Chaur-Jong Hu1,2
1Taipei Neuroscience Institute, Taipei Medical University, Taipei 11031, Taiwan.
Abstract:
Moyamoya disease (MMD) is a cerebrovascular disease that presents with vascular stenosis and a hazy network of collateral formations in angiography. However, the detailed pathogenic pathway remains unknown. Studies have indicated that in addition to variations in the of genetic factor RNF213, unusual circulating angiogenetic factors observed in patients with MMD may play a critical role in producing "Moyamoya vessels". Circulating angiogenetic factors, such as growth factors, vascular progenitor cells, cytokines, inflammatory factors, and other circulating proteins, could promote intimal hyperplasia in vessels and excessive collateral formation with defect structures through endothelial hyperplasia, smooth muscle migration, and atypical neovascularization. This study summarizes the hypothesized pathophysiology of how these circulating factors affect MMD and the interactive modulation between them.
Insights
Moyamoya disease (MMD) involves narrowed brain vessels and abnormal collaterals. This study explores how circulating angiogenic factors contribute to MMD development and the formation of "Moyamoya vessels".
Area of Science:
- Neurology
- Vascular Biology
- Genetics
Background:
- Moyamoya disease (MMD) is characterized by cerebrovascular stenosis and a network of collateral vessels.
- The precise pathogenic mechanisms underlying MMD remain incompletely understood.
- Genetic factors like RNF213 variations are implicated, but other factors are also suspected.
Purpose of the Study:
- To summarize the hypothesized pathophysiology of Moyamoya disease.
- To explore the role of circulating angiogenic factors in MMD pathogenesis.
- To investigate the interactive modulation between these circulating factors.
Main Methods:
- Literature review and synthesis of existing research on MMD.
- Analysis of proposed mechanisms involving circulating angiogenic factors.
- Hypothesizing the pathway from circulating factors to "Moyamoya vessels".
Main Results:
- Circulating angiogenic factors, including growth factors, progenitor cells, and inflammatory mediators, are implicated in MMD.
- These factors may promote intimal hyperplasia, endothelial hyperplasia, smooth muscle migration, and atypical neovascularization.
- The study outlines a hypothesized pathway for how these factors contribute to the characteristic "Moyamoya vessels".
Conclusions:
- Circulating angiogenic factors are likely critical in the development of Moyamoya disease.
- Understanding these factors and their interactions may reveal new therapeutic targets for MMD.
- Further research is needed to elucidate the exact mechanisms and validate the hypothesized pathways.
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