ACBD6 (acyl-CoA binding domain containing 6) is a cytosolic protein that plays essential roles in lipid and protein acylation processes. The protein binds long-chain acyl-CoA molecules with preference for unsaturated C18:1-CoA over saturated species, and serves as a critical regulator of protein N-myristoylation 1. ACBD6 forms functional complexes with N-myristoyltransferase (NMT) enzymes through its ankyrin repeat domain, protecting the rare myristoyl-CoA substrate from competitive inhibition by more abundant acyl-CoAs like palmitoyl-CoA 23. This interaction ensures proper N-myristoylation of proteins essential for neurological function. Additionally, ACBD6 regulates lipid acylation by controlling acyl-CoA availability for lysophospholipid acyltransferase enzymes, affecting phosphatidylcholine formation and lipid droplet content 4. Bi-allelic loss-of-function variants in ACBD6 cause a severe neurodevelopmental disorder characterized by intellectual disability, progressive movement disorders (particularly dystonia), cerebellar ataxia, and brain malformations 1. Patient-derived cells show significantly impaired N-myristoylation of 68 co-translational and 18 post-translational target proteins 1. The disorder can also present with metabolic complications including obesity, diabetes, and organ failure 5, highlighting ACBD6's broader role in cellular metabolism beyond neurological function.