Cerebral cavernous malformations (CCM) are neurovascular dysplasias that result 
in mulberry-shaped lesions predominantly located in brain and spinal tissues. 
Mutations in three genes are associated with CCM. These genes encode for the 
proteins KRIT1/CCM1 (krev interaction trapped 1/cerebral cavernous malformations 
1), cerebral cavernous malformations 2, osmosensing scaffold for MEKK3 
(CCM2/malcavernin/OSM), and cerebral cavernous malformations 3/programmed cell 
death 10 (CCM3/PDCD10). There have been many significant recent advances in our 
understanding of the structure and function of these proteins, as well as in 
their roles in cellular signaling. Here, we provide an update on the current 
knowledge of the structure of the CCM proteins and their functions within 
cellular signaling, particularly in cellular adhesion complexes and signaling 
cascades. We go on to discuss subcellular localization of the CCM proteins, the 
formation and regulation of the CCM complex signaling platform, and current 
progress towards targeted therapy for CCM disease. Recent structural studies 
have begun to shed new light on CCM protein function, and we focus here on how 
these studies have helped inform the current understanding of these roles and 
how they may aid future studies into both CCM-related biology and disease 
mechanisms.