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]

Full Name

ring finger protein 125

Function

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

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

Cellular Location

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.

Involvement in disease

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.

PTM

Autoubiquitinated, leading to its subsequent proteasomal degradation.