WDR37 encodes a WD40 repeat-containing protein that plays essential roles in multiple cellular processes and development. The protein is required for normal ER Ca2+ handling in lymphocytes and, together with PACS1, plays an essential role in stabilizing peripheral lymphocyte populations [UniProt]. WDR37 physically interacts with PACS1 and PACS2 proteins, forming a functionally conserved axis where these proteins are mutually required for proper expression and localization 1. The WDR37-PACS1-PACS2 axis appears critical for ocular development, as coloboma is a shared feature among disorders caused by mutations in these genes 2. De novo missense variants in WDR37's N-terminal region cause neurooculocardiogenitourinary syndrome (NOCGUS), a severe multisystemic disorder characterized by ocular anomalies, dysmorphic features, neurological impairment, and variable cardiac and genitourinary defects 3. Mechanistically, these missense variants may act through a dominant-negative mechanism, as evidenced by zebrafish studies showing that heterozygous missense mutations cause poor growth and lethality, while frameshift variants allow survival 3. Additionally, WDR37 has been implicated in cancer biology, potentially functioning as an E3 ubiquitin ligase with tumor-suppressive properties in pancreatic cancer 4.