DYSF and neuromuscular disease caused by qualitative or quantitative defects of dysferlin: However, they typically do not display severe muscle weakness and loss of ambulation seen in humans.[15, 18, 19, 20] Accurate modeling of human dysferlinopathies in animal models is further complicated by the lack of mutational hotspots, and the poor correlation of patient genotypes with disease progression and severity.[21, 22] Combined with generally low translational success of drug candidates identified in small animal models,[23] there is a clear need to develop in vitro human LGMD2B models for patient‐specific disease modeling and drug discovery.