| Literature DB >> 17344420 |
Rutger O Vogel1, Rolf J R J Janssen, Mariël A M van den Brand, Cindy E J Dieteren, Sjoerd Verkaart, Werner J H Koopman, Peter H G M Willems, Wendy Pluk, Lambert P W J van den Heuvel, Jan A M Smeitink, Leo G J Nijtmans.
Abstract
Ecsit is a cytosolic adaptor protein essential for inflammatory response and embryonic development via the Toll-like and BMP (bone morphogenetic protein) signal transduction pathways, respectively. Here, we demonstrate a mitochondrial function for Ecsit (an evolutionary conserved signaling intermediate in Toll pathways) in the assembly of mitochondrial complex I (NADH:ubiquinone oxidoreductase). An N-terminal targeting signal directs Ecsit to mitochondria, where it interacts with assembly chaperone NDUFAF1 in 500- to 850-kDa complexes as demonstrated by affinity purification and vice versa RNA interference (RNAi) knockdowns. In addition, Ecsit knockdown results in severely impaired complex I assembly and disturbed mitochondrial function. These findings support a function for Ecsit in the assembly or stability of mitochondrial complex I, possibly linking assembly of oxidative phosphorylation complexes to inflammatory response and embryonic development.Entities:
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Year: 2007 PMID： 17344420 PMCID： PMC1820902 DOI： 10.1101/gad.408407
Source DB: PubMed Journal: Genes Dev ISSN： 0890-9369 Impact factor: 11.361