Directed hepatocytedifferentiation fromhuman induced pluripotent stem cells(iPSCs) potentially provides a unique platform for modeling liver genetic diseases and performing drug-toxicity screening invitro. Wilson' s diseaseis a genetic diseasecaused by mutations in the ATP7B gene, whose product is a liver transporter protein responsible for coordinated copper export into bile and blood. Interestingly, thespectrum of ATP7Bmutations is vast and can influence clinical presentation (a variable spectrum ofhepatic and neural manifestations), though thereason is not well understood. We describe thegeneration ofiPSCs froma Chinese patient with Wilson' s diseasethat bears theR778L Chinese hotspot mutation in the ATP7B gene. These iPSCs were pluripotentand could be readily differentiated into hepatocyte- like cellsthat displayed abnormal cytoplasmic localization ofmutated ATP7Band defective copper transport. Moreover, genecorrection usinga self-inactivating lentiviral vector that expresses codon optimized- ATP7Bor treatment with the chaperone drug curcumincould reverse thefunctional defect invitro. Hence, our work describes an attractive model for studying thepathogenesis of Wilson' s diseasethat is valuable for screening compounds or gene therapyapproaches aimed to correct theabnormality. In thefuture, once relevant safety concerns (including thestability of themature liver- likephenotype) and technical issues for thetransplantation procedure are solved, hepatocyte- like cells fromsimilarly genetically corrected iPSCs could be an option for autologous transplantation in Wilson' s disease.