Document Detail


Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I.
MedLine Citation:
PMID:  20816094     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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
Related Documents :
5321404 - Formation of lactyl-coenzyme a and pyruvyl-coenzyme a from lactic acid by escherichia c...
3934574 - Metabolic relationship between arachidonate activation and its transfer to lysophosphol...
3707594 - Cell-free biosynthesis of arphamenine a.
17656314 - Self-acylation properties of type ii fatty acid biosynthesis acyl carrier protein.
505984 - Chloral hydrate poisoning: its mechanism and therapy.
24118924 - Survival and beneficial properties of lactic acid bacteria from raniculture subjected t...
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.
Affiliation:
Nijmegen Centre for Mitochondrial Disorders at the Department of Pediatrics, Radboud University Nijmegen Medical Centre, The Netherlands.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
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
Animals
Cells, Cultured
Electron Transport Complex I / biosynthesis*
Fatty Acids / metabolism
Female
Fibroblasts / cytology,  physiology
Humans
Infant
Male
Mitochondria / metabolism
Models, Molecular
Molecular Sequence Data
Mutation
NADH Dehydrogenase / genetics,  metabolism
Oxidation-Reduction
Oxidative Phosphorylation*
Phylogeny
Pregnancy
Protein Structure, Tertiary
RNA Interference
Sequence Analysis
Sequence Analysis, DNA
Chemical
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 1.3.99.13/Acyl-CoA Dehydrogenase, Long-Chain; EC 1.6.5.3/Electron Transport Complex I; EC 1.6.5.3/NDUFAF1 protein, human; EC 1.6.99.3/NADH Dehydrogenase
Comments/Corrections
Comment In:
Cell Metab. 2010 Sep 8;12(3):211-2   [PMID:  20816087 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Intestinal ferritin h is required for an accurate control of iron absorption.
Next Document:  Rescue of obesity-induced infertility in female mice due to a pituitary-specific knockout of the ins...