Energy metabolism in neuronal/glial induction and iPSC-based modeling of brain disorders

Barbara Mlody, Carmen Lorenz, Gizem Inak, Alessandro Prigione; Seminars in Cell & Developmental Biology, Available online 11 February 2016, ISSN 1084-9521, doi: 10.1016/j.semcdb.2016.02.018.

Neurons derived from FRDAiPSCs recapitulated the characteristic features of the disease terms of GAA expansion as well as FRDA instability. Importantly, impaired mitochondrial function could be observed in derived neuronal cells, including decreased mitochondrial membrane potential and progressive mitochondrial degeneration. Hence, the iPSC system may represent a promising tool for advancing the understanding of the mechanisms of action and brain targeting of mitochondrial disorders due to both nuclear and mitochondrial mutations.

 Energy metabolism in neuronal/glial induction and iPSC-based modeling of brain disorders