| Primary Identifier | IPR046439 | Type | Domain |
| Short Name | ZF_RZ_dom |
| description | This entry represents the RZ type zinc finger domain, found in NFX1-type zinc finger-containing protein 1 (ZNFX1) and E3 ubiquitin-protein ligase RNF213 from humans and other eukaryotic proteins. It contains two conserved histidine residues and four conserved cysteine residues in a CHC3H motif. It can bind divalent transition metals, with a preference for Zn2+.ZNFX1 is an RNA-binding protein that initiates the antiviral response and is required to restrict the replication of RNA viruses [, ].RNF213 is a giant E3 ubiquitin ligase that can catalyse ubiquitination of both proteins and lipids, which is involved in various processes, such as lipid metabolism, angiogenesis and cell-autonomous immunity. This protein functions as a key immune sensor by catalysing ubiquitination of the lipid A moiety of bacterial lipopolysaccharide (LPS) via its RZ-type zinc-finger: restricts the proliferation of cytosolic bacteria, such as Salmonella, by generating the bacterial ubiquitin coat through the ubiquitination of LPS. It is a major susceptibility factor of Moyamoya disease, a cerebrovascular disorder that can result in stroke or death [, , , , ]. |
