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Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I

Nouws, Jessica, Nijtmans, Leo, Houten, Sander M., van den Brand, Mariël, Huynen, Martijn, Venselaar, Hanka, Hoefs, Saskia, Gloerich, Jolein, Kronick, Jonathan, Hutchin, Timothy, Willems, Peter, Rodenburg, Richard, Wanders, Ronald, van den Heuvel, Lambert, Smeitink, Jan, Vogel, Rutger O.·2010·Cell Metabolism
DOI 10.1016/j.cmet.2010.08.002
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Abstract

Acyl-CoA dehydrogenase 9 (ACAD9) is a recently identified member of the acyl-CoA dehydrogenase family. It closely resembles very long-chain acyl-CoA dehydrogenase (VLCAD), involved in mitochondrial beta oxidation of long-chain fatty acids. Contrary to its previously proposed involvement in fatty acid oxidation, we describe a role for ACAD9 in oxidative phosphorylation. ACAD9 binds complex I assembly factors NDUFAF1 and Ecsit and is specifically required for the assembly of complex I. Furthermore, ACAD9 mutations result in complex I deficiency and not in disturbed long-chain fatty acid oxidation. This strongly contrasts with its evolutionary ancestor VLCAD, which we show is not required for complex I assembly and clearly plays a role in fatty acid oxidation. Our results demonstrate that two closely related metabolic enzymes have diverged at the root of the vertebrate lineage to function in two separate mitochondrial metabolic pathways and have clinical implications for the diagnosis of complex I deficiency.

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Metadata

Authors
Nouws, J., Nijtmans, L., Houten, S. M., van den Brand, M., Huynen, M., Venselaar, H., Hoefs, S., Gloerich, J., Kronick, J., Hutchin, T., Willems, P., Rodenburg, R., Wanders, R., van den Heuvel, L., Smeitink, J., & Vogel, R. O.
Venue
Cell Metabolism
Publisher
Elsevier BV
Published
2010-09
Volume
12
Issue
3
Pages
283-294
ISSN
1550-4131
DOI
10.1016/j.cmet.2010.08.002
Cited by
190
References
1
Language
en
Licence
Funders
Nederlandse Organisatie voor Wetenschappelijk Onderzoek

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