In the present study, we show that FAM83H and its associated protein CK-1ε are co-localized with keratin filaments in mouse integumentary cells such as dental ameloblasts and epidermal germinative cells in vivo, and demonstrate that FAM83H and CK-1 regulate the organization of the keratin cytoskeleton and maintain the formation of desmosomes in human ameloblastoma cell lines. Here, CSNK1A1 is linked to ameloblastoma.