ANO10 (anoctamin 10/TMEM16K) is a transmembrane calcium-activated ion channel and phospholipid scramblase with dual functional roles 1. Unlike other anoctamins, ANO10 does not exhibit typical calcium-activated chloride channel (CaCC) activity but does demonstrate chloride channel and phospholipid scrambling activities in a calcium-dependent manner 1. ANO10 participates in multiple cellular processes including endosomal sorting, spindle assembly, mitotic spindle formation, and calcium signaling 12. Mechanistically, ANO10 functions through regulation of local calcium signaling; loss-of-function mutations cause deranged intracellular calcium dynamics in affected cells 2. This dysfunction impairs ion transport, volume regulation, and apoptotic responses in epithelial cells and macrophages 2. Biallelic ANO10 mutations cause autosomal recessive spinocerebellar ataxia type 10 (SCAR10/ATX-ANO10), characterized by slowly progressive cerebellar ataxia with pyramidal signs, cerebellar atrophy, and variable cognitive decline 34. Over 80% of cases involve loss-of-function variants 3. The c.1150_1151del variant, particularly common in Romani populations, associates with earlier disease onset 3. ANO10 defects also cause secondary coenzyme Q10 deficiency, suggesting potential therapeutic benefit from CoQ10 supplementation 5. Disease mechanisms involve calcium signaling dysregulation in Purkinje cells and endolysosomal pathway dysfunction 1.