UFD1L

Gene Information

Official Symbol: UFD1
Official Name: ubiquitin recognition factor in ER associated degradation 1
Aliases and Previous Symbols: N/A
Entrez ID: 7353
UniProt: Q92890
Interactions: BioGRID
PubMed articles: Open PubMed
OMIM: Open OMIM

Function Summary

Entrez Summary: N/A
UniProt Summary: 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 DDX58/RIG-I and recruits RNF125 to promote ubiquitination and degradation of DDX58/RIG-I (PubMed:26471729). {ECO:0000250|UniProtKB:Q9ES53, ECO:0000269|PubMed:26471729}.

Pfam Domains GO Terms

CRISPR Data

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Screen	Score
Monensin 0.3μM R08 exp507	-2.28
CFI-400945 25μM R08 exp469	-2.06
UM131593 0.1μM R01 exp58	1.7
CVT-10216 0.1μM R06 exp278	1.8
Hydroxyurea 15μM R06 exp289	1.94

Global Fraction of Cell Lines Where Essential: 602/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	1/1
909776.0	1/1
bile duct	25/28
blood	26/28
bone	23/26
breast	30/33
central nervous system	41/56
cervix	2/4
colorectal	13/17
esophagus	8/13
fibroblast	1/1
gastric	15/16
kidney	15/21
liver	16/20
lung	61/75
lymphocyte	16/16
ovary	23/26
pancreas	21/24
peripheral nervous system	12/16
plasma cell	13/15
prostate	1/1
skin	20/24
soft tissue	6/9
thyroid	1/2
upper aerodigestive	14/22
urinary tract	20/29
uterus	4/5

Essentiality in NALM6

Essentiality Rank: 835
Expression level (log2 read counts): 6.42

Expression Distribution

UFD1-NPL4 complex
K48-linked polyubiquitin modification-dependent protein binding
VCP-NPL4-UFD1 AAA ATPase complex
negative regulation of RIG-I signaling pathway
negative regulation of viral-induced cytoplasmic pattern recognition receptor signaling pathway
ER-associated misfolded protein catabolic process
endoplasmic reticulum to cytosol transport
regulation of RIG-I signaling pathway
retrograde protein transport, ER to cytosol
error-free translesion synthesis
regulation of viral-induced cytoplasmic pattern recognition receptor signaling pathway
cellular response to misfolded protein
negative regulation of defense response to virus
protein exit from endoplasmic reticulum
response to misfolded protein
protein quality control for misfolded or incompletely synthesized proteins
translesion synthesis
negative regulation of type I interferon production
DNA synthesis involved in DNA repair
postreplication repair
regulation of defense response to virus
ATPase binding
ERAD pathway
negative regulation of response to biotic stimulus
thiol-dependent ubiquitin-specific protease activity
DNA biosynthetic process
negative regulation of immune effector process
regulation of type I interferon production
cellular response to topologically incorrect protein
response to topologically incorrect protein
negative regulation of defense response
negative regulation of multi-organism process
response to endoplasmic reticulum stress
protein-containing complex binding
negative regulation of cytokine production
protein deubiquitination
protein modification by small protein removal
proteasome-mediated ubiquitin-dependent protein catabolic process
signaling receptor binding
proteasomal protein catabolic process