DGCR6L is a nuclear phosphoprotein encoded by one of two highly homologous copies on chromosome 22 1. It functions as a protein binding partner with tissue-specific expression, showing high levels in heart, liver, and skeletal muscle 1. The gene exists as a functional duplicate of DGCR6, with evolutionary evidence suggesting selective pressure for maintaining both paralogs over at least 12 million years 2. Mechanistically, DGCR6L interacts with p21-activated kinase 4 (PAK4) to regulate cancer cell migration through the LIMK1 signaling pathway 3. The interaction requires specific amino acid residues (L115 of DGCR6L binding to PAK4's C-terminal region) and enhances LIMK1 and cofilin phosphorylation, promoting actin cytoskeleton reorganization 3. Clinically, DGCR6L is relevant to 22q11.2 deletion syndrome (22q11DS), the most common human microdeletion syndrome 4. Dysregulation of DGCR6 and DGCR6L expression correlates with childhood psychopathologies, particularly anxiety disorders, though imprinting does not explain this association 4. The variable expression patterns suggest epigenetic mechanisms contribute to 22q11DS phenotypic variability. Additionally, DGCR6 expression is elevated in metastatic cancer cells 1, suggesting a potential role in cancer progression.