RFP2

This gene encodes a member of the tripartite motif (TRIM) family. The TRIM motif includes three zinc-binding domains, a RING, a B-box type 1 and a B-box type 2, and a coiled-coil region. This gene is located on chromosome 13 within the minimal deletion region for B-cell chronic lymphocytic leukemia. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq]

Full Name

tripartite motif-containing 13

Function

Endoplasmic reticulum (ER) membrane anchored E3 ligase involved in the retrotranslocation and turnover of membrane and secretory proteins from the ER through a set of processes named ER-associated degradation (ERAD). This process acts on misfolded proteins as well as in the regulated degradation of correctly folded proteins. Enhances ionizing radiation-induced p53/TP53 stability and apoptosis via ubiquitinating MDM2 and AKT1 and decreasing AKT1 kinase activity through MDM2 and AKT1 proteasomal degradation. Regulates ER stress-induced autophagy, and may act as a tumor suppressor (PubMed:22178386).
Also plays a role in innate immune response by stimulating NF-kappa-B activity in the TLR2 signaling pathway. Ubiquitinates TRAF6 via the 'Lys-29'-linked polyubiquitination chain resulting in NF-kappa-B activation (PubMed:28087809).
Participates as well in T-cell receptor-mediated NF-kappa-B activation (PubMed:25088585).
In the presence of TNF, modulates the IKK complex by regulating IKBKG/NEMO ubiquitination leading to the repression of NF-kappa-B (PubMed:25152375).

Biological Process

Biological Process anatomical structure morphogenesisManual Assertion Based On ExperimentTAS:ProtInc
Biological Process endoplasmic reticulum mannose trimmingTAS:Reactome
Biological Process innate immune responseManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process negative regulation of viral transcriptionManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of cell deathManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of I-kappaB kinase/NF-kappaB signalingManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of macroautophagyManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of NF-kappaB transcription factor activityManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process proteasome-mediated ubiquitin-dependent protein catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process protein autoubiquitinationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process protein ubiquitinationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process response to gamma radiationManual Assertion Based On ExperimentIEP:UniProtKB
Biological Process suppression of viral release by hostManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process ubiquitin-dependent ERAD pathwayManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Endoplasmic reticulum membrane
Concentrates and colocalizes with p62/SQSTM1 and ZFYVE1 at the perinuclear endoplasmic reticulum.

Topology

Helical: 317-337

PTM

Auto-ubiquitinated; requires the RING-type zinc finger. Auto-polyubiquitination leads to proteasomal degradation.