Patient-derived induced pluripotent stem cells (hiPSCs) and genome-edited hiPSCs are useful in biomedical studies for various diseases. Since hiPSCs maintain self-renewal and pluripotency, once hiPSCs are generated from somatic cells of a donor patient, they can be used indefinitely as valuable bioresources. For this reason, there are hiPSC banks in many countries to utilize them in basic research and drug development. In addition, by developing genome-edited hiPSC lines carrying mutations responsible for genetic diseases generated from standard or healthy-donor hiPSCs or repaired mutations from patient-derived hiPSCs, it is possible to perform relatively accurate research studies using these isogenic hiPSC lines. For disease modeling, hiPSCs are differentiated into specific cell lineages or organoids. Since some of the differentiated cell types are highly heterogeneous, visualizing particular cell types and cell status with fluorescent reporters is valuable. Disease models are developed based on recapitulating abnormal phenotypes of cell characteristics and behavior, abnormal expression and abnormal localization of genes and proteins, and other metabolites. These models contribute to elucidating the molecular mechanisms of diseases. Furthermore, high-throughput assays based on hiPSC-derived models can be used to screen molecular targets and compounds. These systematic models are widely applicable as alternative methods which evade animal experiments for intractable diseases.