ECSIT

Summary

Gene Symbol: ECSIT
Description: ECSIT signalling integrator
Alias: SITPEC, evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial, ECSIT homolog, likely ortholog of mouse signaling intermediate in Toll pathway evolutionarily conserved
Species: human
Products:     ECSIT

Top Publications

  1. Vogel R, Janssen R, van den Brand M, Dieteren C, Verkaart S, Koopman W, et al. Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly. Genes Dev. 2007;21:615-24 pubmed
    b>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...
  2. Heide H, Bleier L, Steger M, Ackermann J, Dröse S, Schwamb B, et al. Complexome profiling identifies TMEM126B as a component of the mitochondrial complex I assembly complex. Cell Metab. 2012;16:538-49 pubmed publisher
    ..TMEM126B was found to comigrate with known assembly factors of mitochondrial complex I, namely CIA30, Ecsit, and Acad9. We propose terming this complex mitochondrial complex I assembly (MCIA) complex...
  3. Xiao C, Shim J, Klüppel M, Zhang S, Dong C, Flavell R, et al. Ecsit is required for Bmp signaling and mesoderm formation during mouse embryogenesis. Genes Dev. 2003;17:2933-49 pubmed
    ..In this study, we show that a targeted null mutation of Ecsit, encoding a signaling intermediate of the Toll pathway, leads to reduced cell proliferation, altered epiblast ..
  4. Kopp E, Medzhitov R, Carothers J, Xiao C, Douglas I, Janeway C, et al. ECSIT is an evolutionarily conserved intermediate in the Toll/IL-1 signal transduction pathway. Genes Dev. 1999;13:2059-71 pubmed
    ..This adapter protein, which we have named ECSIT (evolutionarily conserved signaling intermediate in Toll pathways), is specific for the Toll/IL-1 pathways and is ..
  5. Min Y, Wi S, Shin D, Chun E, Lee K. Peroxiredoxin-6 Negatively Regulates Bactericidal Activity and NF-?B Activity by Interrupting TRAF6-ECSIT Complex. Front Cell Infect Microbiol. 2017;7:94 pubmed publisher
    A TRAF6-ECSIT complex is crucial for the generation of mitochondrial reactive oxygen species (mROS) and nuclear factor-kappa B (NF-?B) activation induced by Toll-like receptor 4 (TLR4)...
  6. Geng J, Sun X, Wang P, Zhang S, Wang X, Wu H, et al. Kinases Mst1 and Mst2 positively regulate phagocytic induction of reactive oxygen species and bactericidal activity. Nat Immunol. 2015;16:1142-52 pubmed publisher
    ..Mst1 and Mst2 activated the GTPase Rac to promote Toll-like receptor (TLR)-triggered assembly of the TRAF6-ECSIT complex that is required for the recruitment of mitochondria to phagosomes...
  7. Carneiro F, Lepelley A, Seeley J, Hayden M, Ghosh S. An Essential Role for ECSIT in Mitochondrial Complex I Assembly and Mitophagy in Macrophages. Cell Rep. 2018;22:2654-2666 pubmed publisher
    b>ECSIT is a mitochondrial complex I (CI)-associated protein that has been shown to regulate the production of mitochondrial reactive oxygen species (mROS) following engagement of Toll-like receptors (TLRs)...
  8. Wen H, Ma H, Cai Q, Lin S, Lei X, He B, et al. Recurrent ECSIT mutation encoding V140A triggers hyperinflammation and promotes hemophagocytic syndrome in extranodal NK/T cell lymphoma. Nat Med. 2018;24:154-164 pubmed publisher
    ..419T>C) in the evolutionarily conserved signaling intermediate in Toll pathway (ECSIT) gene, encoding a V140A variant of ECSIT...
  9. Wang H, Behrens E, Pignolo R, Kaplan F. ECSIT links TLR and BMP signaling in FOP connective tissue progenitor cells. Bone. 2018;109:201-209 pubmed publisher
    ..Importantly, Evolutionarily Conserved Signaling Intermediate in the Toll Pathway (ECSIT) integrates TLR injury signaling with dysregulated BMP pathway signaling in FOP CTPCs...

More Information

Publications19

  1. Terenina E, Fabre S, Bonnet A, Monniaux D, Robert Granié C, SanCristobal M, et al. Differentially expressed genes and gene networks involved in pig ovarian follicular atresia. Physiol Genomics. 2017;49:67-80 pubmed publisher
    ..Eleven genes (DKK3, GADD45A, CAMTA2, CCDC80, DAPK2, ECSIT, MSMB, NUPR1, RUNX2, SAMD4A, and ZNF628) having a fold-change higher than five between groups could likely serve ..
  2. Alston C, Compton A, Formosa L, Strecker V, Oláhová M, Haack T, et al. Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype. Am J Hum Genet. 2016;99:217-27 pubmed publisher
    ..identified TMEM126B as a component of the mitochondrial complex I assembly complex alongside proteins ACAD9, ECSIT, NDUFAF1, and TIMMDC1...
  3. Stone M, Sharma A, Mas V, Gehrau R, Mulloy D, Zhao Y, et al. Ex Vivo Perfusion With Adenosine A2A Receptor Agonist Enhances Rehabilitation of Murine Donor Lungs After Circulatory Death. Transplantation. 2015;99:2494-503 pubmed publisher
    ..regulated by EVLP + ATL1223 including the downregulation of genes involved in IL-1 signaling, such as ADCY9, ECSIT, IRAK1, MAPK12, and TOLLIP...
  4. Qu F, Xiang Z, Wang F, Zhang Y, Li J, Zhang Y, et al. Identification and function of an evolutionarily conserved signaling intermediate in Toll pathways (ECSIT) from Crassostrea hongkongensis. Dev Comp Immunol. 2015;53:244-52 pubmed publisher
    ..In this study, we identified an ECSIT homolog in the Hong Kong oyster Crassostrea hongkongensis (ChECSIT) and investigated its biological functions...
  5. Nouws J, Nijtmans L, Houten S, van den Brand M, Huynen M, Venselaar H, et al. Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I. Cell Metab. 2010;12:283-94 pubmed publisher
    ..ACAD9 binds complex I assembly factors NDUFAF1 and Ecsit and is specifically required for the assembly of complex I...
  6. Chen W, Liu L, Jiao B, Lin W, Lin X, Lin X. Hepatitis B virus X protein increases the IL-1β-induced NF-κB activation via interaction with evolutionarily conserved signaling intermediate in Toll pathways (ECSIT). Virus Res. 2015;195:236-45 pubmed publisher
    ..IL-1β)-induced NF-κB activation via interaction with a Toll-like receptor (TLR) adapter protein, ECSIT (evolutionarily conserved signaling intermediate in Toll pathways)...
  7. Wi S, Moon G, Kim J, Kim S, Shim J, Chun E, et al. TAK1-ECSIT-TRAF6 complex plays a key role in the TLR4 signal to activate NF-κB. J Biol Chem. 2014;289:35205-14 pubmed publisher
    b>ECSIT (evolutionarily conserved signaling intermediate in Toll pathways) is known as a multifunctional regulator in different signals, including Toll-like receptors (TLRs), TGF-β, and BMP...
  8. Kondo T, Watanabe M, Hatakeyama S. TRIM59 interacts with ECSIT and negatively regulates NF-?B and IRF-3/7-mediated signal pathways. Biochem Biophys Res Commun. 2012;422:501-7 pubmed publisher
    ..In this study, we showed that one of the TRIM family ubiquitin ligases, TRIM59, interacts with ECSIT as an adaptor protein required for the TLR-mediated transduction pathway...
  9. Mi Wi S, Park J, Shim J, Chun E, Lee K. Ubiquitination of ECSIT is crucial for the activation of p65/p50 NF-κBs in Toll-like receptor 4 signaling. Mol Biol Cell. 2015;26:151-60 pubmed publisher
    Recent evidence shows that evolutionarily conserved signaling intermediate in Toll pathways (ECSIT) interacts with tumor necrosis factor receptor-associated factor 6 (TRAF6), is ubiquitinated, and contributes to bactericidal activity ..
  10. Lei C, Zhang Y, Li M, Jiang L, Zhong B, Kim Y, et al. ECSIT bridges RIG-I-like receptors to VISA in signaling events of innate antiviral responses. J Innate Immun. 2015;7:153-64 pubmed publisher
    ..How this process is mediated is less understood. In this report, we demonstrate that ECSIT is an essential scaffolding protein that mediates the association of VISA and RIG-I or MDA5...