Replacement of the C6ORF66 Assembly Factor (NDUFAF4) Restores Complex I Activity in Patient Cells

Abstract Disorders of the oxidative phosphorylation (OXPHOS) system frequently result in a severe multisystem disease with the consequence of early childhood death. Among these disorders, isolated complex I deficiency is the most frequently diagnosed, accounting for one-third of all cases of respira...

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Bibliographic Details
Main Authors: Dana Marcus, Michal Lichtenstein, Ann Saada, Haya Lorberboum-Galski
Format: Article
Language:English
Published: BMC 2013-05-01
Series:Molecular Medicine
Subjects:
Online Access:https://doi.org/10.2119/molmed.2012.00343