CC2D2B (coiled-coil and C2 domain containing 2B) is a ciliary protein localized to the ciliary transition zone, where it contributes to non-motile cilium assembly 1. The protein contains transglutaminase-like (TGL) peptidase domains and C2 domains, which are key structural modules for protein-protein interactions and membrane localization during ciliogenesis 1. CC2D2B functions as part of ciliary protein complexes implicated in ciliopathies, with structural homology to other transition zone components involved in Nephronophthisis and Meckel-Gruber syndrome pathways 1. Clinically, CC2D2B emerges as a genetic disease modifier: a CC2D2B variant (c.190A>G, rs17383738) shows significant association with hereditary angioedema due to C1 inhibitor deficiency, with heterozygotes being more prevalent in asymptomatic patients (55% vs 23% in symptomatic patients; P = 0.049) 2. Additionally, CC2D2B is identified as a potential target gene of miRNAs dysregulated in nasopharyngeal carcinoma, suggesting broader roles in tissue homeostasis 3. These findings indicate CC2D2B functions in ciliary biogenesis while also modulating clinical disease phenotypes in unrelated genetic disorders.