ID:RN135_HUMAN DESCRIPTION: RecName: Full=E3 ubiquitin-protein ligase RNF135; EC=6.3.2.-; AltName: Full=RIG-I E3 ubiquitin ligase; Short=REUL; AltName: Full=RING finger protein 135; AltName: Full=Riplet; FUNCTION: Acts as an E2-dependent E3 ubiquitin-protein ligase, involved in innate immune defense against viruses. Ubiquitinates DDX58 and is required for full activation of the DDX58 signaling resulting in interferon beta production. PATHWAY: Protein modification; protein ubiquitination. SUBUNIT: Interacts with DDX58. Interacts with PCBP2. SUBCELLULAR LOCATION: Cytoplasm. TISSUE SPECIFICITY: Expressed in skeletal muscle, spleen, kidney, placenta, prostate, stomach, thyroid and tongue. Also weakly expressed in heart, thymus, liver and lung. DISEASE: Defects in RNF135 are the cause of macrocephaly macrosomia facial dysmorphism syndrome (MMFD) [MIM:614192]. MMFD is an autosomal dominant disorder characterized by the association of macrothrombocytopathy and progressive sensorineural hearing loss without renal dysfunction. SIMILARITY: Contains 1 B30.2/SPRY domain. SIMILARITY: Contains 1 RING-type zinc finger. WEB RESOURCE: Name=Leiden Open Variation Database; Note=Ring finger protein 135 (RNF135); URL="http://chromium.liacs.nl/LOVD2/home.php?select_db=RNF135";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q8IUD6
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0002376 immune system process GO:0016567 protein ubiquitination GO:0032480 negative regulation of type I interferon production GO:0032728 positive regulation of interferon-beta production GO:0045087 innate immune response GO:0045088 regulation of innate immune response
BC126420 - Homo sapiens ring finger protein 135, mRNA (cDNA clone MGC:161698 IMAGE:8992136), complete cds. BC126422 - Homo sapiens ring finger protein 135, mRNA (cDNA clone MGC:161700 IMAGE:8992138), complete cds. BC144143 - Homo sapiens cDNA clone IMAGE:9052663. AJ496729 - Homo sapiens mRNA for hypothetical protein (MGC13061 gene). BC005084 - Homo sapiens ring finger protein 135, mRNA (cDNA clone IMAGE:3637572), complete cds. AK226030 - Homo sapiens mRNA for ring finger protein 135 isoform 2 variant, clone: FCC104E08. BC082262 - Homo sapiens ring finger protein 135, mRNA (cDNA clone IMAGE:6421129). AK312979 - Homo sapiens cDNA, FLJ93440. AK122646 - Homo sapiens cDNA FLJ16075 fis, clone MAMGL1000173, highly similar to Homo sapiens ring finger protein 135 (RNF135), transcript variant 2, mRNA. AK122709 - Homo sapiens cDNA FLJ16196 fis, clone COLON2006417, highly similar to Homo sapiens ring finger protein 135 (RNF135), transcript variant 1, mRNA. KJ894961 - Synthetic construct Homo sapiens clone ccsbBroadEn_04355 RNF135 gene, encodes complete protein. AB470605 - Homo sapiens Riplet mRNA for Riplet, complete cds. AY598332 - Homo sapiens L13 mRNA, complete cds. JD256038 - Sequence 237062 from Patent EP1572962. JD139334 - Sequence 120358 from Patent EP1572962. JD064486 - Sequence 45510 from Patent EP1572962. JD176212 - Sequence 157236 from Patent EP1572962. JD333155 - Sequence 314179 from Patent EP1572962. JD482210 - Sequence 463234 from Patent EP1572962. JD101294 - Sequence 82318 from Patent EP1572962. JD344742 - Sequence 325766 from Patent EP1572962. JD293506 - Sequence 274530 from Patent EP1572962. JD358111 - Sequence 339135 from Patent EP1572962. JD514013 - Sequence 495037 from Patent EP1572962. JD514014 - Sequence 495038 from Patent EP1572962. JD514015 - Sequence 495039 from Patent EP1572962. JD069840 - Sequence 50864 from Patent EP1572962. JD415158 - Sequence 396182 from Patent EP1572962. JD415159 - Sequence 396183 from Patent EP1572962. JD415164 - Sequence 396188 from Patent EP1572962. JD374331 - Sequence 355355 from Patent EP1572962. JD399477 - Sequence 380501 from Patent EP1572962. JD336266 - Sequence 317290 from Patent EP1572962. JD050636 - Sequence 31660 from Patent EP1572962. JD284663 - Sequence 265687 from Patent EP1572962. JD559938 - Sequence 540962 from Patent EP1572962. JD290472 - Sequence 271496 from Patent EP1572962.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q8IUD6 (Reactome details) participates in the following event(s):
R-HSA-918224 DDX58 is K63 polyubiquitinated R-HSA-168909 viral dsRNA:IFIH1, viral dsRNA:K63polyUb-DDX58 bind MAVS R-HSA-936378 Inhibition of DDX58/IFIH1 signaling by ATG5:ATG12:MAVS R-HSA-937343 NLRC5 interacts with DDX58/IFIH1 R-HSA-168934 MAVS interacts with RIPK1 and FADD R-HSA-918227 Recruitment of TRAF3 to MAVS R-HSA-918230 Recruitment of TRAF6/TRAF2 to IPS-1 R-HSA-990528 Interaction of PCBP2 with MAVS R-HSA-933528 Interaction of MEKK1 with TRAF6 R-HSA-933537 Recruitment of TANK to TRAF6 R-HSA-933539 Recruitment of IKK complex R-HSA-918225 TBK1/IKK epsilon complex interacts with MAVS bound TRAF3 R-HSA-936381 OTUD5 deubiquitinates TRAF3 R-HSA-933526 Recruitment of caspase-8 and -10 to FADD complex R-HSA-990526 Recruitment of AIP4 and K48 ubiquitination of MAVS/IPS-1 R-HSA-933527 Recruitment of TBK1/IKK epsilon complex to TANK:TRAF6 R-HSA-918232 Recruitment of IRF3,IRF7 R-HSA-918229 Phosphorylation and release of IRF3/IRF7 R-HSA-933523 Dimerzation of procaspase-8, procaspase-10 R-HSA-933538 Recruitment of IRF7 to TRAF6 R-HSA-933525 Phosphorylation and release of IRF7 R-HSA-933530 Activation of IKK by MEKK1 R-HSA-933532 Processing of caspases R-HSA-168928 DDX58/IFIH1-mediated induction of interferon-alpha/beta R-HSA-168249 Innate Immune System R-HSA-936440 Negative regulators of DDX58/IFIH1 signaling R-HSA-168256 Immune System R-HSA-933543 NF-kB activation through FADD/RIP-1 pathway mediated by caspase-8 and -10 R-HSA-918233 TRAF3-dependent IRF activation pathway R-HSA-933541 TRAF6 mediated IRF7 activation R-HSA-933542 TRAF6 mediated NF-kB activation R-HSA-5689896 Ovarian tumor domain proteases R-HSA-5688426 Deubiquitination R-HSA-597592 Post-translational protein modification R-HSA-392499 Metabolism of proteins
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
