Protein: E3 ubiquitin-protein ligase RNF126 (EC 2.3.2.27) (RING finger protein 126) (Q9BV68)
Organism: Homo sapiens (Human)
Function: E3 ubiquitin-protein ligase that mediates ubiquitination oF target proteins. Depending on the associated E2 ligase, mediates 'Lys-48'- and 'Lys-63'-linked polyubiquitination of substrates (By similarity). Part of a BAG6-dependent quality control process ensuring that proteins of the secretory pathway that are mislocalized to the cytosol are degraded by the proteasome. Probably acts by providing the ubiquitin ligase activity associated with the BAG6 complex and be responsible for ubiquitination of the hydrophobic mislocalized proteins and their targeting to the proteasome. May also play a role in the endosomal recycling of IGF2R, the cation-independent mannose-6-phosphate receptor. May play a role in the endosomal sorting and degradation of several membrane receptors including EGFR, FLT3, MET and CXCR4, by mediating their ubiquitination. By ubiquitinating CDKN1A/p21 and targeting it for degradation, may also promote cell proliferation. May monoubiquitinate AICDA.
Subcellular location: Cytoplasm {ECO:0000269|PubMed:23026136}. Nucleus {ECO:0000269|PubMed:23026136}.
Sequence: MAEASPHPGRYFCHCCSVEIVPRLPDYICPRCESGFIEELPEETRSTENGSAPSTAPTDQSRPPLEHVDQHLFTLPQGYGQFAFGIFDDSFEIPTFPPGAQADDGRDPESRRERDHPSRHRYGARQPRARLTTRRATGRHEGVPTLEGIIQQLVNGIITPATIPSLGPWGVLHSNPMDYAWGANGLDAIITQLLNQFENTGPPPADKEKIQALPTVPVTEEHVGSGLECPVCKDDYALGERVRQLPCNHLFHDGCIVPWLEQHDSCPVCRKSLTGQNTATNPPGLTGVSFSSSSSSSSSSSPSNENATSNS
InterPro domains: - IPR013083: Zinc finger, RING/FYVE/PHD-type (homologous_superfamily) [215-289]
- IPR039525: E3 ubiquitin-protein ligase RNF126-like, zinc-ribbon (domain) [10-40]
- IPR001841: Zinc finger, RING-type (domain) [228-270]
Interaction partners: - Large proline-rich protein BAG6
- Golgi to ER traffic protein 4 homolog
- Ubiquitin-conjugating enzyme E2 D2
- ATPase GET3
- Ubiquitin-like protein 4A
- Ubiquitin-conjugating enzyme E2 D1
- E2 ubiquitin-conjugating enzyme
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016740 (transferase activity), GO:0140096 (catalytic activity, acting on a protein), GO:0005515 (protein binding), GO:0005102 (signaling receptor binding), GO:0019787 (ubiquitin-like protein transferase activity), GO:0070851 (growth factor receptor binding), GO:0004842 (ubiquitin-protein transferase activity), GO:0061659 (ubiquitin-like protein ligase activity), GO:0061630 (ubiquitin protein ligase activity), GO:0005154 (epidermal growth factor receptor binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0065007 (biological regulation), GO:0048518 (positive regulation of biological process), GO:0050789 (regulation of biological process), GO:0009987 (cellular process), GO:0048519 (negative regulation of biological process), GO:0023057 (negative regulation of signaling), GO:0044237 (cellular metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0050794 (regulation of cellular process), GO:0048583 (regulation of response to stimulus), GO:0023051 (regulation of signaling), GO:0071704 (organic substance metabolic process), GO:0048523 (negative regulation of cellular process), GO:0009056 (catabolic process), GO:0048585 (negative regulation of response to stimulus), GO:0044238 (primary metabolic process), GO:0048522 (positive regulation of cellular process), GO:0009968 (negative regulation of signal transduction), GO:0010648 (negative regulation of cell communication), GO:1901564 (organonitrogen compound metabolic process), GO:0010646 (regulation of cell communication), GO:0044248 (cellular catabolic process), GO:0019538 (protein metabolic process), GO:0009966 (regulation of signal transduction), GO:1901575 (organic substance catabolic process), GO:0044260 (cellular macromolecule metabolic process), GO:0043170 (macromolecule metabolic process), GO:0036211 (protein modification process), GO:1901185 (negative regulation of ERBB signaling pathway), GO:0009057 (macromolecule catabolic process), GO:0043412 (macromolecule modification), GO:1901565 (organonitrogen compound catabolic process), GO:0006508 (proteolysis), GO:0044265 (cellular macromolecule catabolic process), GO:0030163 (protein catabolic process), GO:1901184 (regulation of ERBB signaling pathway), GO:0042059 (negative regulation of epidermal growth factor receptor signaling pathway), GO:0042058 (regulation of epidermal growth factor receptor signaling pathway), GO:0070647 (protein modification by small protein conjugation or removal), GO:0043632 (modification-dependent macromolecule catabolic process), GO:0051603 (proteolysis involved in protein catabolic process), GO:0032446 (protein modification by small protein conjugation), GO:0019941 (modification-dependent protein catabolic process), GO:0016567 (protein ubiquitination), GO:0006511 (ubiquitin-dependent protein catabolic process), GO:0051865 (protein autoubiquitination), GO:0043162 (ubiquitin-dependent protein catabolic process via the multivesicular body sorting pathway)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005829 (cytosol), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0005654 (nucleoplasm), GO:0043233 (organelle lumen), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle), GO:0070013 (intracellular organelle lumen), GO:0031981 (nuclear lumen), GO:0005634 (nucleus)