Structural highlights
Disease
RN216_HUMAN Cerebellar ataxia-hypogonadism syndrome. The disease is caused by variants affecting the gene represented in this entry.
Function
RN216_HUMAN Isoform 1 acts as an E3 ubiquitin ligase, which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, and then transfers it to substrates promoting their degradation by the proteasome. Promotes degradation of TRAF3, TLR4 and TLR9. Contributes to the regulation of antiviral responses. Down-regulates activation of NF-kappa-B, IRF3 activation and IFNB production. Isoform 3 inhibits TNF and IL-1 mediated activation of NF-kappa-B. Promotes TNF and RIP mediated apoptosis.[1] [2]
See Also
References
- ↑ Chuang TH, Ulevitch RJ. Triad3A, an E3 ubiquitin-protein ligase regulating Toll-like receptors. Nat Immunol. 2004 May;5(5):495-502. doi: 10.1038/ni1066. Epub 2004 Apr 25. PMID:15107846 doi:http://dx.doi.org/10.1038/ni1066
- ↑ Nakhaei P, Mesplede T, Solis M, Sun Q, Zhao T, Yang L, Chuang TH, Ware CF, Lin R, Hiscott J. The E3 ubiquitin ligase Triad3A negatively regulates the RIG-I/MAVS signaling pathway by targeting TRAF3 for degradation. PLoS Pathog. 2009 Nov;5(11):e1000650. doi: 10.1371/journal.ppat.1000650. Epub , 2009 Nov 6. PMID:19893624 doi:http://dx.doi.org/10.1371/journal.ppat.1000650
