ECSIT is a 431 amino acid ubiquitously expressed protein that plays an important role as an adaptor protein in the cytosolic signal transduction cascade events triggered by Toll receptor activation. Within the Toll pathway, ECSIT regulates MEKK1 processing for activation of NFκB, a major event leading to initiation of the innate immune response. In the mitochondria, ECSIT interacts with NDUFAF1 and assists in the formation of NADH:ubiquinone oxidoreductase (complex I), an extremely complicated multiprotein complex located in the inner mitochondrial membrane that functions in the transport of electrons from NADH to ubiquinone. Knockdown of ECSIT results in severely impaired complex I assembly and disturbed mitochondrial function. There are two isoforms of ECSIT that are produced as a result of alternative splicing events
Function:
Adapter protein of the Toll-like and IL-1 receptor signaling pathway that is involved in the activation of NF-kappa-B via MAP3K1. Promotes proteolytic activation of MAP3K1. Involved in the BMP signaling pathway. Required for normal embryonic development (By similarity). Required for efficient assembly of mitochondrial NADH:ubiquinone oxidoreductase.
Subunit:
Interacts with MAP3K1, SMAD4 and TRAF6. Interacts with SMAD1 only after BMP4-treatment (By similarity). Part of the mitochondrial complex I assembly (MCIA) complex. The complex comprises at least TMEM126B, NDUFAF1, ECSIT, and ACAD9 (By similarity). Interacts with NDUFAF1.
Subcellular Location:
Cytoplasm. Nucleus. Mitochondrion.
Similarity:
Belongs to the ECSIT family.
SWISS:
Q9BQ95
Gene ID:
51295
Database links:
Entrez Gene: 51295 Human
Entrez Gene: 26940 Mouse
Entrez Gene: 300447 Rat
Omim: 608388 Human
SwissProt: Q9BQ95 Human
SwissProt: Q9QZH6 Mouse
SwissProt: Q5XIC2 Rat
Unigene: 515146 Human
Unigene: 425568 Mouse
Unigene: 8455 Rat
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