| Tissue Specificity | Found in adult heart, skeletal muscle and pancreas, and in fetal liver and kidney. |
| Function | Essential component of the ubiquitin-dependent proteolytic pathway which degrades ubiquitin fusion proteins. The ternary complex containing UFD1, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. It may be involved in the development of some ectoderm-derived structures. Acts as a negative regulator of type I interferon production via the complex formed with VCP and NPLOC4, which binds to RIGI and recruits RNF125 to promote ubiquitination and degradation of RIGI. |
| Protein Name | Ubiquitin Recognition Factor In Er-Associated Degradation Protein 1Ubiquitin Fusion Degradation Protein 1Ub Fusion Protein 1 |
| Database Links | Reactome: R-HSA-110320Reactome: R-HSA-5689880Reactome: R-HSA-8951664Reactome: R-HSA-9755511 |
| Cellular Localisation | NucleusCytoplasmCytosol |
| Alternative Protein Names | Ubiquitin Recognition Factor In Er-Associated Degradation Protein 1 proteinUbiquitin Fusion Degradation Protein 1 proteinUb Fusion Protein 1 proteinUFD1 proteinUFD1L protein |
Information sourced from Uniprot.org
