title: Energy metabolism in neuronal/glial induction and in iPSC models of brain disorders.
abstract: The metabolic switch associated with the reprogramming of somatic cells to pluripotency has received increasing attention in recent years. However, the impact of mitochondrial and metabolic modulation on stem cell differentiation into neuronal/glial cells and related brain disease modeling still remains to be fully addressed. Here, we seek to focus on this aspect by first addressing brain energy metabolism and its inter-cellular metabolic compartmentalization. We then review the findings related to the mitochondrial and metabolic reconfiguration occurring upon neuronal/glial specification from pluripotent stem cells (PSCs). Finally, we provide an update of the PSC-based models of mitochondria-related brain disorders and discuss the challenges and opportunities that may exist on the road to develop a new era of brain disease modeling and therapy.
authors.affiliation: Max Delbrueck Center for Molecular Medicine (MDC), Berlin, Germany.
authors.forename: Barbara
authors.initials: B
authors.lastname: Mlody
authors.affiliation: Max Delbrueck Center for Molecular Medicine (MDC), Berlin, Germany.
authors.forename: Carmen
authors.initials: C
authors.lastname: Lorenz
authors.affiliation: Max Delbrueck Center for Molecular Medicine (MDC), Berlin, Germany.
authors.forename: Gizem
authors.initials: G
authors.lastname: Inak
authors.affiliation: Max Delbrueck Center for Molecular Medicine (MDC), Berlin, Germany. Electronic address: alessandro.prigione@mdc-berlin.de.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Prigione
mesh_terms: D000818:Animals; D001927:Brain Diseases / Q000378:metabolism* / Q000473:pathology; D002454:Cell Differentiation / Q000502:physiology; D004734:Energy Metabolism; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000166:cytology / Q000378:metabolism*; D009457:Neuroglia / Q000166:cytology / Q000378:metabolism*; D009474:Neurons / Q000166:cytology / Q000378:metabolism*
keywords: Glia; Metabolism; Mitochondria; Mitochondrial disorders; Neural progenitors; Neurons; Reprogramming; iPSCs