Recombinant Human E3 ubiquitin-protein ligase RNF135 (RNF135)

Uniprot Id

Q8IUD6

Target Species

Human

Target Name

RNF135

Target Full Name

E3 ubiquitin-protein ligase RNF135

Target Function

E2-dependent E3 ubiquitin-protein ligase that functions as a RIG-I/DDX58 coreceptor in the sensing of viral RNAs in cell cytoplasm and the activation of the antiviral innate immune response. Together with the UBE2D3, UBE2N and UB2V1 E2 ligases, catalyzes the 'Lys-63'-linked polyubiquitination of RIG-I/DDX58 oligomerized on viral RNAs, an essential step in the activation of the RIG-I signaling pathway. Through a ubiquitin-independent parallel mechanism, which consists in bridging RIG-I/DDX58 filaments forming on longer viral RNAs, further activates the RIG-I signaling pathway. This second mechanism that synergizes with the ubiquitin-dependent one would thereby allow an RNA length-dependent regulation of the RIG-I signaling pathway (Probable). Associated with the E2 ligase UBE2N, also constitutively synthesizes unanchored 'Lys-63'-linked polyubiquitin chains that may also activate the RIG-I signaling pathway.

Target Involvement

Macrocephaly, macrosomia, facial dysmorphism syndrome (MMFD)

Target Subcellular Location

Cytoplasm. Cytoplasm, Stress granule.

Target Tissue Specificity

Expressed in skeletal muscle, spleen, kidney, placenta, prostate, stomach, thyroid and tongue. Also weakly expressed in heart, thymus, liver and lung.

Target Synonyms

E3 ubiquitin-protein ligase RNF135; L13; MGC13061; MMFD; REUL; RIG-I E3 ubiquitin ligase; RING finger protein 135; Riplet; RN135_HUMAN; Rnf135

Target Background

The protein encoded by this gene contains a RING finger domain, a motif present in a variety of functionally distinct proteins and known to be involved in protein-protein and protein-DNA interactions. This gene is located in a chromosomal region known to be frequently deleted in patients with neurofibromatosis. Alternatively spliced transcript variants encoding distinct isoforms have been reported.