Endothelial Cells Lining Sporadic Cerebral Cavernous Malformation Cavernomas Undergo Endothelial-to-Mesenchymal Transition

Stroke; a Journal of Cerebral Circulation
Luca BraviMaria Grazia Lampugnani

Abstract

Cerebral cavernous malformation (CCM) is characterized by multiple lumen vascular malformations in the central nervous system that can cause neurological symptoms and brain hemorrhages. About 20% of CCM patients have an inherited form of the disease with ubiquitous loss-of-function mutation in any one of 3 genes CCM1, CCM2, and CCM3. The rest of patients develop sporadic vascular lesions histologically similar to those of the inherited form and likely mediated by a biallelic acquired mutation of CCM genes in the brain vasculature. However, the molecular phenotypic features of endothelial cells in CCM lesions in sporadic patients are still poorly described. This information is crucial for a targeted therapy. We used immunofluorescence microscopy and immunohistochemistry to analyze the expression of endothelial-to-mesenchymal transition markers in the cavernoma of sporadic CCM patients in parallel with human familial cavernoma as a reference control. We report here that endothelial cells, a cell type critically involved in CCM development, undergo endothelial-to-mesenchymal transition in the lesions of sporadic patients. This switch in endothelial phenotype has been described only in genetic CCM patients and in murine models of t...Continue Reading

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Citations

Oct 1, 2017·Circulation Research·Sara I CunhaMaria Grazia Lampugnani
Aug 31, 2017·Cold Spring Harbor Perspectives in Biology·Maria Grazia LampugnaniCostanza Giampietro
Aug 31, 2017·Cold Spring Harbor Perspectives in Biology·Anna Szymborska, Holger Gerhardt
Mar 8, 2019·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Masato KanazawaGregory J Del Zoppo
Mar 14, 2019·Physiological Reviews·Sonsoles Piera-Velazquez, Sergio A Jimenez
Apr 17, 2018·Neurosurgery·Sean P PolsterIssam A Awad
Jul 2, 2019·Journal of Neurosurgery·Issam A Awad, Sean P Polster
Sep 6, 2018·EMBO Molecular Medicine·Cécile OttenSalim Abdelilah-Seyfried
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Sep 10, 2019·Circulation Research·Claudia Jasmin RödelSalim Abdelilah-Seyfried
Feb 18, 2017·Current Opinion in Hematology·Maria Grazia LampugnaniNoemi Rudini
Jun 22, 2020·Clinical and Translational Medicine·Lorelei D ShoemakerSteven D Chang
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Jan 16, 2021·Angiogenesis·Leah J Greenspan, Brant M Weinstein
Sep 3, 2020·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Concetta ScimoneAntonina Sidoti
Feb 24, 2021·Stroke; a Journal of Cerebral Circulation·Joppe OldenburgPeetra U Magnusson
Aug 3, 2021·Journal of Cell Science·Cecilia SavoraniCostanza Giampietro

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