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Complexome profiling of mitochondrial protein complexes to study the molecular consequences of DNAJC30 mutations

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We identified the chaperone DNAJC30 as an important factor to maintain NADH:ubiquinone oxidoreductase (complex I) activity. In this complexome analysis we detected DNAJC30 in substoichiometric amounts attached to respiratory supercomplexes (I/III2/IVn and demonstrate an accumulation of complex I containing respiratory supercomplexes in the patient cell line with DNAJC30 mutations compared to the control cell line.
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2021-09-09
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