We have, for the first time, generated patient-specific-induced pluripotent stem cell-derived cardiomyocytes (BrS-hiPSC-CMs) from a BrS patient with recurrent SCA carrying a missense variant of uncertain variance in the CACNB2 gene (c.425C > T/p.S142F) and compared their cellular physiological and pharmacological properties with healthy and isogenic control (variant corrected) hiPSC-CMs. This evidence concerns the gene CACNB2 and autosomal dominant cerebellar ataxia.