DTNA encodes α-dystrobrevin, a component of the dystrophin-glycoprotein complex (DGC) that plays critical roles in muscle membrane stability and synaptic function 1. The protein binds to dystrophin/utrophin and α-syntrophin, forming the core of a large membrane-bound complex essential for muscle fiber integrity 12. Mechanistically, α-dystrobrevin helps maintain the structural organization of the DGC, with variants in its coiled-coil domain disrupting interactions with syntrophin and causing reduced immunoreactivity of multiple DGC proteins including dystrophin and sarcoglycans 1. Disease-wise, monoallelic DTNA variants cause a dominantly inherited muscular dystrophy characterized by myalgia, exercise intolerance, elevated serum creatine kinase, and variable proximal muscle weakness 1. The gene is also associated with left ventricular non-compaction cardiomyopathy, where regulatory variants show significant enrichment in early-onset cases 34. Additionally, DTNA variants have been identified in familial Ménière's disease, with knockdown studies in Drosophila demonstrating defective proprioception and auditory function 56. Clinically, DTNA-related disorders show variable penetrance and may present with autism spectrum disorders or learning disabilities in some patients 1.