Ontology highlight
ABSTRACT:
INSTRUMENT(S): Q Exactive Plus
ORGANISM(S): Homo Sapiens (ncbitaxon:9606)
SUBMITTER: Alessandro Prigione
PROVIDER: MSV000080849 | MassIVE | Fri Mar 31 22:32:00 BST 2017
SECONDARY ACCESSION(S): PXD004977
REPOSITORIES: MassIVE
Lorenz Carmen C Lesimple Pierre P Bukowiecki Raul R Zink Annika A Inak Gizem G Mlody Barbara B Singh Manvendra M Semtner Marcus M Mah Nancy N Auré Karine K Leong Megan M Zabiegalov Oleksandr O Lyras Ekaterini-Maria EM Pfiffer Vanessa V Fauler Beatrix B Eichhorst Jenny J Wiesner Burkhard B Huebner Norbert N Priller Josef J Mielke Thorsten T Meierhofer David D Izsvák Zsuzsanna Z Meier Jochen C JC Bouillaud Frédéric F Adjaye James J Schuelke Markus M Wanker Erich E EE Lombès Anne A Prigione Alessandro A
Cell stem cell 20170126 5
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 from patients carrying a deleterious homoplasmic mutation in the mitochondrial gene MT- ...[more]