CITED2 is a transcriptional coregulator that functions as a bridge between transcription factors and the p300/CBP coactivator complex 1. It acts as both a positive and negative regulator of gene expression: it enhances transcriptional activation by TFAP2 factors, TGF-β signaling, PPAR-α, and estrogen receptors, while simultaneously repressing HIF1A and STAT2-mediated transcription 1. CITED2 plays essential roles in cardiac and reproductive development—it regulates left-right patterning through NODAL-mediated transcription and controls adrenal cortex differentiation via WT1-mediated activation 2. In spermatogonial stem cells, CITED2 interacts with EP300 to promote proliferation and survival through HSPA6 regulation 3. Functionally, CITED2 mediates mechanical shear-induced suppression of matrix metalloproteinases in chondrocytes via TGF-β signaling 4 and exerts anti-inflammatory effects in lung fibroblasts by inhibiting the NF-κB pathway 5. Clinically, CITED2 mutations are associated with congenital heart defects; promoter variants reduce CITED2 expression and correlate with ventricular septal defects 6 and atrial septal defects 7. These findings establish CITED2 as a critical developmental regulator with implications for cardiac malformation pathogenesis.