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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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DERL3
derlin 3
Chromosome 22 · 22q11.23
NCBI Gene: 91319Ensembl: ENSG00000099958.16HGNC: HGNC:14236UniProt: Q96Q80
29PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
signal recognition particle bindingprotein bindingprotein-containing complex bindingprotein N-linked glycosylationcystic fibrosisatrial fibrillationhypertrophic cardiomyopathyhead injury
✦AI Summary

DERL3 (derlin 3) is a functional component of endoplasmic reticulum-associated degradation (ERAD) that selectively mediates retrotranslocation of misfolded lumenal glycoproteins into the cytosol for proteasomal degradation 1. The protein may form a channel facilitating glycoprotein extraction and mediate interactions between VCP and misfolded substrates. DERL3 also participates in ER stress-induced pre-emptive quality control, rerouting newly synthesized proteins to cytosolic degradation pathways. Clinically, DERL3 shows context-dependent roles in disease. In colorectal cancer, DERL3 acts as a tumor suppressor—low expression correlates with increased metastasis and poor prognosis, with DERL3 suppressing proliferation by negatively regulating MYCN 2. Conversely, in breast cancer and lung adenocarcinoma, elevated DERL3 associates with malignant phenotypes and shorter survival 34. In nasopharyngeal carcinoma, EBV-mediated DNA methylation silences DERL3, promoting tumor progression 5. DERL3 also contributes to rheumatoid arthritis pathogenesis through TNF-α-induced NF-κB activation in fibroblast-like synoviocytes 6. Recent evidence indicates altered DERL3 distribution in type 2 diabetes pancreatic β cells 7 and that post-translational succinylation regulates DERL3 stability during inflammatory responses 8. These findings suggest DERL3 represents a potential therapeutic target across multiple inflammatory and malignant diseases.

Sources cited
1
DERL3 is an ER stress-related protein essential for degradation of misfolded lumenal glycoproteins and correlates with immune infiltration and poor immunotherapy response in lung adenocarcinoma
PMID: 36275646
2
DERL3 is downregulated in colorectal cancer tissues and acts as a tumor suppressor by negatively regulating MYCN to suppress proliferation, migration, and invasion
PMID: 32538588
3
DERL3 overexpression is associated with malignant phenotype in breast cancer, with high expression correlating with lymph node metastasis and shorter overall survival
PMID: 28713959
4
DERL3 is upregulated in lung adenocarcinoma tissues with lymph node metastasis and promotes proliferation, invasion, and epithelial-mesenchymal transition
PMID: 32838486
5
EBV latent membrane protein 1 induces DNA methylation-mediated DERL3 repression in nasopharyngeal carcinoma, promoting tumor progression
PMID: 36372158
6
DERL3 is upregulated in rheumatoid arthritis synovial fibroblasts via TNF-α stimulation and promotes inflammation through NF-κB pathway activation
PMID: 32866644
7
DERL3 is among core ERAD components with altered distributions in type 2 diabetes pancreatic β cells during ER stress response
PMID: 40512624
8
KAT3B-mediated succinylation of DERL3 stabilizes the protein and activates TLR4/MyD88 pathway to suppress osteogenic differentiation in periodontitis
PMID: 39716643
Disease Associationsⓘ20
cystic fibrosisOpen Targets
0.46Moderate
atrial fibrillationOpen Targets
0.12Weak
hypertrophic cardiomyopathyOpen Targets
0.12Weak
head injuryOpen Targets
0.09Suggestive
lung adenocarcinomaOpen Targets
0.09Suggestive
heart failureOpen Targets
0.08Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
systolic heart failureOpen Targets
0.06Suggestive
cancerOpen Targets
0.05Suggestive
colorectal cancerOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.04Suggestive
dilated cardiomyopathyOpen Targets
0.04Suggestive
colorectal carcinomaOpen Targets
0.03Suggestive
Barrett's esophagusOpen Targets
0.03Suggestive
cardiomyopathyOpen Targets
0.03Suggestive
gastric cancerOpen Targets
0.02Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
Miyoshi myopathyOpen Targets
0.02Suggestive
rheumatoid arthritisOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SEL1L3Protein interaction97%AMFRProtein interaction96%AUP1Protein interaction96%RNF5Protein interaction96%SEL1LProtein interaction96%UFD1Protein interaction96%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
93%
Lung
83%
Ovary
32%
Heart
19%
Brain
15%
Gene Interaction Network
Click a node to explore
DERL3SEL1L3AMFRAUP1RNF5SEL1LUFD1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q96Q80
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.41LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.99 [0.71–1.41]
RankingsWhere DERL3 stands among ~20K protein-coding genes
  • #12,135of 20,598
    Most Researched29
  • #14,605of 17,882
    Most Constrained (LOEUF)1.41
Genes detectedDERL3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
VDAC1 is a target for pharmacologically induced insulin hypersecretion in β cells.
PMID: 40512624
Cell Rep · 2025
1.00
2
KAT3B-mediated succinylation of DERL3 suppresses osteogenic differentiation by promoting M1/M2 macrophage polarization.
PMID: 39716643
Biochem Pharmacol · 2025
0.90
3
Integrated profiling of endoplasmic reticulum stress-related DERL3 in the prognostic and immune features of lung adenocarcinoma.
PMID: 36275646
Front Immunol · 2022
0.80
4
DERL3 suppresses colorectal cancer metastasis through negatively regulating MYCN level.
PMID: 32538588
Minerva Med · 2023
0.70
5
Overexpression of Derlin 3 is associated with malignant phenotype of breast cancer cells.
PMID: 28713959
Oncol Rep · 2017
0.60