The CCM3-GCKIII partnership

Abstract

Specific mutations in the CCM3 gene predispose to the development of cerebral cavernous malformations, a special type of vascular lesions. This calls for an elucidation of the precise nature of the CCM3 protein and a deep understanding of its molecular regulation. In this review, we outline our current knowledge of the different CCM3 protein complexes. We focus on the GCKIII family of kinases as partners of CCM3 and discuss the functional consequences of this partnership, putting forward a putative model for the activation of these kinases.