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: 0301 basic medicine; 0303 health sciences; 03 medical and health sciences

Brain microvasculature constitutes a highly specialized and selective vascular barrier between blood and the central nervous system, called blood brain barrier (BBB). In these vessels endothelial cells present a highly developed system of tight junctions (TJs), absence of fenestration and low pinocytotic activity. Cells of the brain parenchyma, the astrocytes, contribute to the BBB-coverage with their foot processes, which constitute about the eighty percent of the basal aspect of the vesselsThree proteins, CCM1, CCM2 and CCM3, expressed by brain endothelial cells, are emerging as key modulators of the organization and function of the BBB. Indeed, mutations occurring in any of the genes encoding these proteins, leads to Cerebral Cavernous Malformation (CCM), a pathology characterized by brain vascular malformations. The endothelium in the lesions presents very few tight junctions and gaps are observed between endothelial cells. In addition, the vascular basal lamina is disorganized and the astrocytes do n...