The name CCM refers to pathologies with familial inheritance that have been attributed to mutations in human KRIT1, CCM2, or PDCD10. Affected individuals exhibit morphological malformations of low-perfused venous endothelial beds of the neuro-vasculature that can result in dangerous cerebral bleeding [reviewed in Riant et al., 2010]. Here, CCM2 is linked to cerebral cavernous malformation.