CERT1 encodes a ceramide transporter that mediates non-vesicular trafficking of ceramides from the endoplasmic reticulum to the Golgi apparatus, where they are converted to sphingomyelin. The protein efficiently transfers ceramides with long-chain fatty acids but not those with very long acyl chains, and shows minimal capacity to transfer diacylglycerol. CERT1 contains a previously uncharacterized dimeric helical domain essential for autoregulation; loss of this regulatory mechanism causes uncontrolled sphingolipid production 1. Mutations disrupting CERT autoregulation are associated with ceramide transporter (CerTra) syndrome, a neurodevelopmental disorder characterized by intellectual disability 1. Disease severity correlates with the degree of autoregulation loss 1. Inherited variants including S132L and G243R impair serine-repeat motif phosphorylation and cause excessive CERT activity with abnormal subcellular distribution 2. Pharmacological CERT inhibition corrects morphological and motor abnormalities in disease models 1. Beyond developmental disease, CERT1 has emerged as a clinical biomarker. Plasma ceramide-based risk scores incorporating CERT1 predict cardiovascular mortality and adverse events in patients with type 2 diabetes and coronary artery disease 3, 4, 5. In acute ischemic stroke, elevated plasma C16:0-ceramide and CERT1 scores are independent predictors of stroke-associated pneumonia 6. These findings position CERT1 function at the intersection of developmental neurobiology and systemic metabolic disease risk.