Document Detail

Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I.
MedLine Citation:
PMID:  20816094     Owner:  NLM     Status:  MEDLINE    
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.
Jessica Nouws; Leo Nijtmans; Sander M Houten; Mariël van den Brand; Martijn Huynen; Hanka Venselaar; Saskia Hoefs; Jolein Gloerich; Jonathan Kronick; Timothy Hutchin; Peter Willems; Richard Rodenburg; Ronald Wanders; Lambert van den Heuvel; Jan Smeitink; Rutger O Vogel
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cell metabolism     Volume:  12     ISSN:  1932-7420     ISO Abbreviation:  Cell Metab.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-09-06     Completed Date:  2010-11-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101233170     Medline TA:  Cell Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  283-94     Citation Subset:  IM    
Copyright Information:
2010 Elsevier Inc. All rights reserved.
Nijmegen Centre for Mitochondrial Disorders at the Department of Pediatrics, Radboud University Nijmegen Medical Centre, The Netherlands.
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MeSH Terms
Acyl-CoA Dehydrogenase, Long-Chain / chemistry,  classification,  genetics,  metabolism
Acyl-CoA Dehydrogenases / chemistry,  classification,  genetics,  metabolism*
Adaptor Proteins, Signal Transducing / genetics,  metabolism
Amino Acid Sequence
Cells, Cultured
Electron Transport Complex I / biosynthesis*
Fatty Acids / metabolism
Fibroblasts / cytology,  physiology
Mitochondria / metabolism
Models, Molecular
Molecular Sequence Data
NADH Dehydrogenase / genetics,  metabolism
Oxidative Phosphorylation*
Protein Structure, Tertiary
RNA Interference
Sequence Analysis
Sequence Analysis, DNA
Reg. No./Substance:
0/Adaptor Proteins, Signal Transducing; 0/Ecsit protein, human; 0/Fatty Acids; EC 1.3.-/Acyl-CoA Dehydrogenases; EC 1.3.99.-/ACAD9 protein, human; EC Dehydrogenase, Long-Chain; EC Transport Complex I; EC protein, human; EC Dehydrogenase
Comment In:
Cell Metab. 2010 Sep 8;12(3):211-2   [PMID:  20816087 ]

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