VPS16 encodes a core subunit of the CORVET and HOPS endosomal tethering complexes that plays a critical role in vesicle-mediated protein trafficking to lysosomes 1. The protein facilitates autophagosome-lysosome fusion and endosome maturation by regulating SNARE-mediated membrane fusion events 1. VPS16 interacts with vacuolar-H+-ATPase subunit G to control phagosomal acidification, and its disruption can block fusion between lysosomes and phagosomes 1. Pathogenic variants in VPS16 cause DYT-VPS16, a rare monogenic form of dystonia characterized by early-onset (median 12-16.5 years) isolated dystonia that typically begins in cervical, upper limb, or laryngeal regions and progresses to generalization in approximately 50% of cases 23. The phenotypic spectrum has expanded beyond isolated dystonia to include additional features such as myoclonus, choreoathetosis, tremor, and sleep disturbances 42. Deep brain stimulation targeting the globus pallidus internus shows therapeutic efficacy, with approximately 73% of patients demonstrating significant motor improvement, though response varies with disease characteristics and timing of intervention 52. This represents a promising treatment option for this autophagy-related dystonia subtype 6.