The programmed cell death 10 (

PDCD10

) gene was originally identified as an apoptosis-related gene

although it is now usually known as

CCM3

as the third causative gene of cerebral cavernous malformation (CCM). CCM is a neurovascular disease that is characterized by vascular malformations and is associated with headaches

seizures

focal neurological deficits

and cerebral hemorrhage. The PDCD10/CCM3 protein has multiple subcellular localizations and interacts with several multi-protein complexes and signaling pathways. Thus

PDCD10/CCM3

governs many cellular functions

which include cell-to-cell junctions and cytoskeleton organization

cell proliferation and apoptosis

and exocytosis and angiogenesis. Given its central role in the maintenance of homeostasis of the cell

dysregulation of

PDCD10/CCM3

can result in a wide range of altered cell functions. This can lead to severe diseases

including CCM

cognitive disability

and several types of cancers. Here

we review the multifaceted roles of

PDCD10/CCM3

in physiology and pathology

with a focus on its functions beyond CCM.