RNF125

This gene encodes a novel E3 ubiquitin ligase that contains an N-terminal RING finger domain. The encoded protein may function as a positive regulator in the T-cell receptor signaling pathway. [provided by RefSeq]

ring finger protein 125

E3 ubiquitin-protein ligase that mediates ubiquitination and subsequent proteasomal degradation of target proteins, such as DDX58/RIG-I, MAVS/IPS1, IFIH1/MDA5, JAK1 and p53/TP53 (PubMed:15843525, PubMed:17460044, PubMed:17643463, PubMed:26027934, PubMed:26471729, PubMed:25591766, PubMed:27411375).
Acts as a negative regulator of type I interferon production by mediating ubiquitination of DDX58/RIG-I at 'Lys-181', leading to DDX58/RIG-I degradation (PubMed:17460044, PubMed:26471729).
Mediates ubiquitination and subsequent degradation of p53/TP53 (PubMed:25591766).
Mediates ubiquitination and subsequent degradation of JAK1 (PubMed:26027934).
Acts as a positive regulator of T-cell activation (PubMed:15843525).

Biological Process adaptive immune responseIEA:UniProtKB-KW
Biological Process cellular response to leukemia inhibitory factorIEA:Ensembl
Biological Process negative regulation of RIG-I signaling pathwayManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process negative regulation of type I interferon productionManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process protein polyubiquitinationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process ubiquitin-dependent protein catabolic processManual Assertion Based On ExperimentIDA:UniProtKB

Golgi apparatus membrane
Shows a reticular staining pattern within the cell and is probably expressed at other intracellular membranes in addition to the Golgi membrane. Not detected at the plasma membrane.

Tenorio syndrome (TNORS):
A disease characterized by overgrowth, macrocephaly, and intellectual disability. Some patients may have mild hydrocephaly, hypoglycemia, and inflammatory diseases resembling Sjogren syndrome.

Autoubiquitinated, leading to its subsequent proteasomal degradation.