TY - JOUR
T1 - Vascular Remodeling in Moyamoya Angiopathy
T2 - From Peripheral Blood Mononuclear Cells to Endothelial Cells
AU - Tinelli, Francesca
AU - Nava, Sara
AU - Arioli, Francesco
AU - Bedini, Gloria
AU - Scelzo, Emma
AU - Lisini, Daniela
AU - Faragò, Giuseppe
AU - Gioppo, Andrea
AU - Ciceri, Elisa F
AU - Acerbi, Francesco
AU - Ferroli, Paolo
AU - Vetrano, Ignazio G
AU - Esposito, Silvia
AU - Saletti, Veronica
AU - Pantaleoni, Chiara
AU - Zibordi, Federica
AU - Nardocci, Nardo
AU - Zedde, Maria Luisa
AU - Pezzini, Alessandro
AU - Di Lazzaro, Vincenzo
AU - Capone, Fioravante
AU - Dell'Acqua, Maria Luisa
AU - Vajkoczy, Peter
AU - Tournier-Lasserve, Elisabeth
AU - Parati, Eugenio A
AU - Bersano, Anna
AU - Gatti, Laura
PY - 2020/8/11
Y1 - 2020/8/11
N2 - The pathophysiological mechanisms of Moyamoya angiopathy (MA), which is a rare cerebrovascular condition characterized by recurrent ischemic/hemorrhagic strokes, are still largely unknown. An imbalance of vasculogenic/angiogenic mechanisms has been proposed as one possible disease aspect. Circulating endothelial progenitor cells (cEPCs) have been hypothesized to contribute to vascular remodeling of MA, but it remains unclear whether they might be considered a disease effect or have a role in disease pathogenesis. The aim of the present study was to provide a morphological, phenotypical, and functional characterization of the cEPCs from MA patients to uncover their role in the disease pathophysiology. cEPCs were identified from whole blood as CD45dimCD34+CD133+ mononuclear cells. Morphological, biochemical, and functional assays were performed to characterize cEPCs. A significant reduced level of cEPCs was found in blood samples collected from a homogeneous group of adult (mean age 46.86 ± 11.7; 86.36% females), Caucasian, non-operated MA patients with respect to healthy donors (HD; p = 0.032). Since no difference in cEPC characteristics and functionality was observed between MA patients and HD, a defective recruitment mechanism could be involved in the disease pathophysiology. Collectively, our results suggest that cEPC level more than endothelial progenitor cell (EPC) functionality seems to be a potential marker of MA. The validation of our results on a larger population and the correlation with clinical data as well as the use of more complex cellular model could help our understanding of EPC role in MA pathophysiology.
AB - The pathophysiological mechanisms of Moyamoya angiopathy (MA), which is a rare cerebrovascular condition characterized by recurrent ischemic/hemorrhagic strokes, are still largely unknown. An imbalance of vasculogenic/angiogenic mechanisms has been proposed as one possible disease aspect. Circulating endothelial progenitor cells (cEPCs) have been hypothesized to contribute to vascular remodeling of MA, but it remains unclear whether they might be considered a disease effect or have a role in disease pathogenesis. The aim of the present study was to provide a morphological, phenotypical, and functional characterization of the cEPCs from MA patients to uncover their role in the disease pathophysiology. cEPCs were identified from whole blood as CD45dimCD34+CD133+ mononuclear cells. Morphological, biochemical, and functional assays were performed to characterize cEPCs. A significant reduced level of cEPCs was found in blood samples collected from a homogeneous group of adult (mean age 46.86 ± 11.7; 86.36% females), Caucasian, non-operated MA patients with respect to healthy donors (HD; p = 0.032). Since no difference in cEPC characteristics and functionality was observed between MA patients and HD, a defective recruitment mechanism could be involved in the disease pathophysiology. Collectively, our results suggest that cEPC level more than endothelial progenitor cell (EPC) functionality seems to be a potential marker of MA. The validation of our results on a larger population and the correlation with clinical data as well as the use of more complex cellular model could help our understanding of EPC role in MA pathophysiology.
U2 - 10.3390/ijms21165763
DO - 10.3390/ijms21165763
M3 - Article
C2 - 32796702
VL - 21
JO - International Journal of Molecular Sciences
JF - International Journal of Molecular Sciences
SN - 1661-6596
IS - 16
ER -