Abstract
Moyamoya arteriopathy (MA) is a rare cerebrovascular disorder characterized by ischemic/hemorrhagic strokes. The pathophysiology is unknown. A deregulation of vasculogenic/angiogenic/inflammatory pathways has been hypothesized as a possible pathophysiological mechanism. Since lipids are implicated in modulating neo-vascularization/angiogenesis and inflammation, their deregulation is potentially involved in MA. Our aim is to evaluate angiogenic/vasculogenic/inflammatory proteins and lipid profile in plasma of MA patients and control subjects (healthy donors HD or subjects with atherosclerotic cerebrovascular disease ACVD). Angiogenic and inflammatory protein levels were measured by ELISA and a complete lipidomic analysis was performed on plasma by mass spectrometry. ELISA showed a significant decrease for MMP-9 released in plasma of MA. The untargeted lipidomic analysis showed a cumulative depletion of lipid asset in plasma of MA as compared to HD. Specifically, a decrease in membrane complex glycosphingolipids peripherally circulating in MA plasma with respect to HD was observed, likely suggestive of cerebral cellular recruitment. The quantitative targeted approach demonstrated an increase in free sphingoid bases, likely associated with a deregulated angiogenesis. Our findings indicate that lipid signature could play a central role in MA and that a detailed biomarker profile may contribute to untangle the complex, and still obscure, pathogenesis of MA.
Highlights
Moyamoya arteriopathy (MA) is characterized by a progressive steno-occlusive lesion of the terminal part of the internal carotid arteries (ICAs), with a compensatory development of unstable collateral vessels at the base of the brain (Moyamoya vessels) [1,2]
The disease presented with an ischemic event in 32.5% of patients, a hemorrhagic stroke in 20% of them, and a transient ischemic attack (TIA) in 27.5% of cases
Ring Finger Protein213 (RNF213) ants have been reported in Caucasian cases, preferentially clustering in the variants have been reported in Caucasian cases, preferentially clustering in the E3 doExamples of protein targets of conventional ubiquitination by RNF213 are master regulators main [11,39]
Summary
Moyamoya arteriopathy (MA) is characterized by a progressive steno-occlusive lesion of the terminal part of the internal carotid arteries (ICAs), with a compensatory development of unstable collateral vessels at the base of the brain (Moyamoya vessels) [1,2] These vascular hallmarks are responsible for recurrent ischemic and hemorrhagic strokes, leading patients affected by MA—often young adults and children—to severe neurological deficits, progressive physical disabilities, and even death [3,4,5,6,7]. 213 (RNF213)/Mysterin coding gene in East Asian patients strengthen the role of genetic factors in MA pathogenesis [8,9,10,11,12,13,14,15]. Due to the detection of altered levels of endothelial progenitor cells (EPC), cytokines, chemokines, and growth factors in MA patient biological fluids, impaired angiogenesis and vasculogenesis have been invoked as potential disease mechanisms [2,9,19]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
