ArticleScientific reports2021
Vulnerability to shear stress caused by altered peri-endothelial matrix is a key feature of Moyamoya disease.
Article in Scientific reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 22 citations in OpenAlex.
- Modeling Moyamoya disease using patient-derived induced pluripotent stem cells and organoids.Molecular biology reports · 2026Review
- Article
- Moyamoya disease: epidemiology, clinical features, pathogenesis, diagnosis and therapeutic interventions.Molecular biomedicine · 2025Review
- Artificial intelligence-integrated video analysis of vessel area changes and instrument motion for microsurgical skill assessment.Scientific reports · 2025Article
- DNA methylation profiles reveals STAB1-mediated endothelial cell and immune cell interactions in Moyamoya disease.Clinical and translational medicine · 2025Article
- Impact ofJournal of clinical neurology (Seoul, Korea) · 2025Article
- HAPLN3 p.T34A contributes to incomplete penetrance of moyamoya disease in Chinese carrying RNF213 p.R4810K.European journal of neurology · 2024Article
- Prognostic value of morphology and hemodynamics in moyamoya disease for long-term outcomes and disease progression.Scientific reports · 2024Article
- Identification of diagnostic markers for moyamoya disease by combining bulk RNA-sequencing analysis and machine learning.Scientific reports · 2024Article
- Understanding genomic medicine for thoracic aortic disease through the lens of induced pluripotent stem cells.Frontiers in cardiovascular medicine · 2024Review
- Autophagy and mitophagy-related extracellular mitochondrial dysfunction of cerebrospinal fluid cells in patients with hemorrhagic moyamoya disease.Scientific reports · 2023Article
- Physiological and pathophysiological mechanisms of the molecular and cellular biology of angiogenesis and inflammation in moyamoya angiopathy and related vascular diseases.Frontiers in neurology · 2023Review
- Mechanical regulation of the early stages of angiogenesis.Journal of the Royal Society, Interface · 2022Review
- Novel Multifaceted Roles for RNF213 Protein.International journal of molecular sciences · 2022Review
- Long-Lasting Unruptured Large Intracranial Aneurysm in Bilateral Moyamoya Disease: Case Report.Case reports in neurological medicine · 2022Article
- Transcriptomic Profiling of Intracranial Arteries in Adult Patients With Moyamoya Disease Reveals Novel Insights Into Its Pathogenesis.Frontiers in molecular neuroscience · 2022Article
- Clinical Management of Moyamoya Patients.Journal of clinical medicine · 2021Review
- RNF213 and GUCY1A3 in Moyamoya Disease: Key Regulators of Metabolism, Inflammation, and Vascular Stability.Frontiers in neurology · 2021Review
Corrections and comments
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Authors and funding
12 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Moyamoya disease (MMD) is characterized by progressive bilateral stenotic changes in the terminal portion of the internal carotid arteries. Although RNF213 was identified as a susceptibility gene for MMD, the exact pathogenesis remains unknown. Immunohistochemical analysis of autopsy specimens from a patient with MMD revealed marked accumulation of hyaluronan and chondroitin sulfate (CS) in the thickened intima of occlusive lesions of MMD. Hyaluronan synthase 2 was strongly expressed in endothelial progenitor cells in the thickened intima. Furthermore, MMD lesions showed minimal staining for CS and hyaluronan in the endothelium, in contrast to control endothelium showing positive staining for both. Glycosaminoglycans of endothelial cells derived from MMD and control induced pluripotent stem cells demonstrated a decreased amount of CS, especially sulfated CS, in MMD. A computational fluid dynamics model showed highest wall shear stress values in the terminal portion of the internal carotid artery, which is the predisposing region in MMD. Because the peri-endothelial extracellular matrix plays an important role in protection, cell adhesion and migration, an altered peri-endothelial matrix in MMD may contribute to endothelial vulnerability to wall shear stress. Invading endothelial progenitor cells repairing endothelial injury would produce excessive hyaluronan and CS in the intima, and cause vascular stenosis.
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