ZNF407 is a zinc finger transcription factor that plays critical roles in neurodevelopment and metabolic regulation. As a DNA-binding transcription factor, ZNF407 regulates gene expression through direct DNA binding, with structural analyses indicating that its zinc finger domains are essential for target DNA sequence recognition 1. The protein functions as a transcriptional cofactor, particularly interacting with the PPARγ/RXRα complex in adipocytes to modulate metabolic gene expression, including GLUT4 regulation 2. ZNF407 mutations are associated with significant neurodevelopmental disorders, including intellectual disability, autism spectrum disorders, microcephaly, and hypotonia 31. Pathogenic variants include chr18 translocations that reduce ZNF407 expression and missense mutations (Y460C, P1195A, S1685W) that disrupt zinc finger integrity and DNA-binding function 31. The gene is located within the 18q22.3-18q23 critical region associated with multiple congenital anomalies, including congenital aural atresia 4. Clinical significance extends beyond neurodevelopment, as ZNF407 shows therapeutic potential in metabolic disorders through its role in glucose homeostasis regulation 2. The protein's dysfunction appears to interfere with various gene expression programs during fetal brain development, establishing it as an essential transcription factor for normal cognitive development.