Human iPSC-Derived Neural Progenitors Are an Effective Drug Discovery Model for Neurological mtDNA Disorders.

doi:10.1016/j.stem.2016.12.013

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Article

Human iPSC-Derived Neural Progenitors Are an Effective Drug Discovery Model for Neurological mtDNA Disorders.

Cell stem cell - 4 May 2017

Lorenz Carmen, Lesimple Pierre, Bukowiecki Raul, Zink Annika, Inak Gizem, Mlody Barbara, Singh Manvendra, Semtner Marcus, Mah Nancy, Auré Karine, Leong Megan, Zabiegalov Oleksandr, Lyras Ekaterini-Maria, Pfiffer Vanessa, Fauler Beatrix, Eichhorst Jenny, Wiesner Burkhard, Huebner Norbert, Priller Josef, Mielke Thorsten, Meierhofer David, Izsvák Zsuzsanna, Meier Jochen C, Bouillaud Frédéric, Adjaye James, Schuelke Markus, Wanker Erich E, Lombès Anne, Prigione Alessandro

Abstract excerpt

Mitochondrial DNA (mtDNA) mutations frequently cause neurological diseases. Modeling of these defects has been difficult because of the challenges associated with engineering mtDNA. We show here that neural progenitor cells (NPCs) derived from human induced pluripotent stem cells (iPSCs) retain the parental mtDNA profile and exhibit a metabolic switch toward oxidative phosphorylation. NPCs derived in this way...

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PMID: 28132834
DOI: 10.1016/j.stem.2016.12.013

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

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