The Pathological Modifications of the Blood Brain Barrier and Cerebral Cavernous Malformations

• DOI: 10.1096/fasebj.29.1_supplement.81.1
• Publication Date: 2021-06-21T17:03:12Z
• Authors: Elisabetta Dejana; Noemi Rudini; Roberto Cuttano; Luca Bravi; Maria Lampugnani
• Keywords: Cavernous malformations

Brain microvasculature constitutes a highly specialized and selective vascular barrier between blood the central nervous system, called brain (BBB). In these vessels endothelial cells present developed system of tight junctions (TJs), absence fenestration low pinocytotic activity. Cells parenchyma, astrocytes, contribute to BBB-coverage with their foot processes, which constitute about eighty percent basal aspect vesselsThree proteins, CCM1, CCM2 CCM3, expressed by cells, are emerging as key modulators organization function BBB. Indeed, mutations occurring in any genes encoding leads Cerebral Cavernous Malformation (CCM), pathology characterized malformations. The endothelium lesions presents very few gaps observed cells. addition, lamina is disorganized astrocytes do not take contact wall. structural alterations BBB CCM associated severe clinical manifestations, such cerebral hemorrhages stroke. Additional data point possible link inflammatory cytokines proteins regulation stability. These open new therapeutic opportunities for this so far incurable disease