Protein: Ubiquitin recognition factor in ER-associated degradation protein 1 (Ubiquitin fusion degradation protein 1) (UB fusion protein 1) (Q92890)
Organism: Homo sapiens (Human)
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 (By similarity). 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.
Subcellular location: Nucleus {ECO:0000250|UniProtKB:Q9ES53}. Cytoplasm, cytosol {ECO:0000250|UniProtKB:Q9ES53}.
Sequence: MFSFNMFDHPIPRVFQNRFSTQYRCFSVSMLAGPNDRSDVEKGGKIIMPPSALDQLSRLNITYPMLFKLTNKNSDRMTHCGVLEFVADEGICYLPHWMMQNLLLEEGGLVQVESVNLQVATYSKFQPQSPDFLDITNPKAVLENALRNFACLTTGDVIAINYNEKIYELRVMETKPDKAVSIIECDMNVDFDAPLGYKEPERQVQHEESTEGEADHSGYAGELGFRAFSGSGNRLDGKKKGVEPSPSPIKPGDIKRGIPNYEFKLGKITFIRNSRPLVKKVEEDEAGGRFVAFSGEGQSLRKKGRKP
InterPro domains: - IPR055417: Ubiquitin fusion degradation protein UFD1, N-terminal subdomain 1 (domain) [19-117]
- IPR042299: Ufd1-like, Nn domain (homologous_superfamily) [6-118]
- IPR055418: Ubiquitin fusion degradation protein UFD1, N-terminal subdomain 2 (domain) [120-194]
- IPR004854: Ubiquitin fusion degradation protein Ufd1-like (family) [15-302]
Interaction partners: - UBX domain-containing protein 7
- Transitional endoplasmic reticulum ATPase
- Nuclear protein localization protein 4 homolog
- FAS-associated factor 2
- FAS-associated factor 1
- NSFL1 cofactor p47
- Deubiquitinating protein VCPIP1
- Ubiquitin-associated domain-containing protein 2
- UBX domain-containing protein 10
- Derlin-1
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0005515 (protein binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0051179 (localization), GO:0050896 (response to stimulus), GO:0009987 (cellular process), GO:0051641 (cellular localization), GO:0042221 (response to chemical), GO:0006807 (nitrogen compound metabolic process), GO:0051234 (establishment of localization), GO:0071704 (organic substance metabolic process), GO:0009056 (catabolic process), GO:0033036 (macromolecule localization), GO:0044237 (cellular metabolic process), GO:0051716 (cellular response to stimulus), GO:0006950 (response to stress), GO:0044238 (primary metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0044248 (cellular catabolic process), GO:0019538 (protein metabolic process), GO:0051649 (establishment of localization in cell), GO:0043170 (macromolecule metabolic process), GO:0010033 (response to organic substance), GO:0045184 (establishment of protein localization), GO:0046907 (intracellular transport), GO:1901698 (response to nitrogen compound), GO:0033554 (cellular response to stress), GO:0006810 (transport), GO:0070727 (cellular macromolecule localization), GO:1901575 (organic substance catabolic process), GO:0044260 (cellular macromolecule metabolic process), GO:0071705 (nitrogen compound transport), GO:1901565 (organonitrogen compound catabolic process), GO:0006508 (proteolysis), GO:0030163 (protein catabolic process), GO:0071702 (organic substance transport), GO:1903513 (endoplasmic reticulum to cytosol transport), GO:0034976 (response to endoplasmic reticulum stress), GO:0009057 (macromolecule catabolic process), GO:0006886 (intracellular protein transport), GO:0010243 (response to organonitrogen compound), GO:0044265 (cellular macromolecule catabolic process), GO:0015031 (protein transport), GO:0008104 (protein localization), GO:0032527 (protein exit from endoplasmic reticulum), GO:0030970 (retrograde protein transport, ER to cytosol), GO:0043632 (modification-dependent macromolecule catabolic process), GO:0051603 (proteolysis involved in protein catabolic process), GO:0036503 (ERAD pathway), GO:0010498 (proteasomal protein catabolic process), GO:0030433 (ubiquitin-dependent ERAD pathway), GO:0043161 (proteasome-mediated ubiquitin-dependent protein catabolic process), GO:0019941 (modification-dependent protein catabolic process), GO:0006511 (ubiquitin-dependent protein catabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0032991 (protein-containing complex), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0140534 (endoplasmic reticulum protein-containing complex), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0098796 (membrane protein complex), GO:0012505 (endomembrane system), GO:0031984 (organelle subcompartment), GO:0042175 (nuclear outer membrane-endoplasmic reticulum membrane network), GO:0031090 (organelle membrane), GO:0034098 (VCP-NPL4-UFD1 AAA ATPase complex), GO:0005783 (endoplasmic reticulum), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0098827 (endoplasmic reticulum subcompartment), GO:0043231 (intracellular membrane-bounded organelle), GO:0005789 (endoplasmic reticulum membrane)