Figure 1.

Generation and utility of human stem cell models. Human pluripotent stem cells (hPSCs) can be generated either from the inner cell mass of a blastocyst, known as human embryonic stem cells (hESCs), or by reprogramming a host’s somatic cells, most commonly fibroblasts or peripheral blood mononuclear cells (PBMCs), to make induced pluripotent stem cells (iPSCs). Both forms of hPSCs are capable of indefinite self-renewal and can differentiate to the three primary germ layers. This capacity to differentiate can be directed experimentally to form terminally differentiated cell types or more complex organoid models, making hPSCs suitable for modelling the multitude of systems affected by mitochondrial diseases.