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...
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
BMC
2013-05-01
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Series: | Molecular Medicine |
Subjects: | |
Online Access: | https://doi.org/10.2119/molmed.2012.00343 |