CARD9 interacts with the CARD activation domain of BCL-10. CARD9 associates with BCL-10 and forms a complex within cells. CARD9 induces apoptosis and activates NF-kB. CARD9 is an upstream activator of BCL-10 and NF-kB signaling.
Defects in CARD9 are the cause of familial candidiasis type 2 (CANDF2) [MIM:212050]. Chronic mucocutaneous candidiasis is characterized by impaired clearance of fungal infections and results in colonization and infections of the mucosa or skin, predominantly with Candida albicans. CANDF2 is an autosomal recessive chronic mucocutaneous candidiasis.