DCHS1 encodes a calcium-dependent cell adhesion protein that plays critical roles in development and tissue organization. The protein functions as an atypical cadherin that forms heterophilic interactions with FAT4, creating high-affinity binding interfaces essential for planar cell polarity signaling 1. DCHS1 regulates cell proliferation and differentiation through multiple pathways, including modulation of Hippo signaling via YAP-TEAD activity during osteoblast differentiation 2. In cardiac development, DCHS1 is essential for proper mitral valve morphogenesis, where it interacts with the septin-actin cytoskeleton through LIX1L to direct cell-ECM alignment and valve tissue formation 3. The protein also influences brain development, with modern human-specific glycosylation changes affecting neocortex-to-striatum proportions through modulation of EPHA4-ephrin signaling 4. Disease-associated mutations in DCHS1 cause periventricular heterotopia with neuronal hyperactivity due to altered sodium channel function and increased morphological complexity 5. Clinically, DCHS1 mutations are associated with Van Maldergem syndrome, mitral valve prolapse, and genetic generalized epilepsy, highlighting its importance across multiple developmental processes 67.