InterPro domain: IPR045129

This entry represents a group of RING finger-containing proteins, including RNF123/RSPRY1 from humans and RKP from Arabidopsis.

RNF123 is the catalytic subunit of the KPC complex that acts as E3 ubiquitin-protein ligase. It also functions as an inhibitor of innate antiviral signalling mediated by DDX58 and IFIH1 independently of its E3 ligase activity [ 1 ]. The role of RSPRY1 (RING finger and SPRY domain-containing protein 1) is not known, but it may have a role in bone development. Mutations in RSPRY1 cause skeletal dysplasia [ 2 ].

RKP is an E3 ubiquitin-protein ligase that promotes the ubiquitination and proteasomal degradation of KRP1 and KRP2 [ 3 ].

1. RNF123 has an E3 ligase-independent function in RIG-I-like receptor-mediated antiviral signaling. EMBO Rep 17, 1155-68
2. Identification of a Recognizable Progressive Skeletal Dysplasia Caused by RSPRY1 Mutations. Am. J. Hum. Genet. 97, 608-15
3. Degradation of the cyclin-dependent kinase inhibitor KRP1 is regulated by two different ubiquitin E3 ligases. Plant J. 53, 705-16