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Overview

Uniprot IDQ92890
Protein NameUbiquitin recognition factor in ER-associated degradation protein 1
Gene NameUFD1
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
238 SGNRLDGKKKGVEPS
240 NRLDGKKKGVEPSPS
264 GIPNYEFKLGKITFI
267 NYEFKLGKITFIRNS

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 (PubMed:26471729)

Protein Sequence

10 MFSFNMFDHP 20 IPRVFQNRFS 30 TQYRCFSVSM 40 LAGPNDRSDV 50 EKGGKIIMPP 60 SALDQLSRLN 70 ITYPMLFKLT 80 NKNSDRMTHC 90 GVLEFVADEG 100 ICYLPHWMMQ 110 NLLLEEGGLV 120 QVESVNLQVA 130 TYSKFQPQSP 140 DFLDITNPKA 150 VLENALRNFA 160 CLTTGDVIAI 170 NYNEKIYELR 180 VMETKPDKAV 190 SIIECDMNVD 200 FDAPLGYKEP 210 ERQVQHEEST 220 EGEADHSGYA 230 GELGFRAFSG 240 SGNRLDGKKK 250 GVEPSPSPIK 260 PGDIKRGIPN 270 YEFKLGKITF 280 IRNSRPLVKK 290 VEEDEAGGRF 300 VAFSGEGQSL RKKGRKP

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005829 cytosol
Cellular Component GO:0005654 nucleoplasm
Cellular Component GO:0005634 nucleus
Cellular Component GO:0036501 UFD1-NPL4 complex
Cellular Component GO:0034098 VCP-NPL4-UFD1 AAA ATPase complex
Molecular Function GO:0004843 cysteine-type deubiquitinase activity
Molecular Function GO:0036435 K48-linked polyubiquitin modification-dependent protein binding
Molecular Function GO:0031593 polyubiquitin modification-dependent protein binding
Biological Process GO:0071218 cellular response to misfolded protein
Biological Process GO:0036503 ERAD pathway
Biological Process GO:0039536 negative regulation of RIG-I signaling pathway
Biological Process GO:0032480 negative regulation of type I interferon production
Biological Process GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process
Biological Process GO:0072344 rescue of stalled cytosolic ribosome
Biological Process GO:0030970 retrograde protein transport, ER to cytosol
Biological Process GO:1990116 ribosome-associated ubiquitin-dependent protein catabolic process
Biological Process GO:0001501 skeletal system development
Biological Process GO:0006511 ubiquitin-dependent protein catabolic process

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Cheng Z, Huang H, Li M, Chen Y. Proteomic analysis identifies PFKP lactylation in SW480 colon cancer cells.. iScience 27(1):108645. 2024 Jan 19. PMID: 38155775.

[3] Guo X, Ren X, Yan C, Huang H. Quantitative Proteomics Reveals the Role of Lysine Lactylation in Lenalidomide-Resistance in Multiple Myeloma Cells.. ACS Chem Biol 20(7):1728-1738. 2025 Jul 18. PMID: 40590393.

[4] Wu Q, Li Z, Gong T, Zheng X, Zhou X et al.. Porphyromonas gingivalis infection induces lysine lactylation reprogramming in human umbilical vein endothelial cells.. Front Cell Infect Microbiol 16:1706727. 2026. PMID: 41696360.