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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KDELR3
KDEL endoplasmic reticulum protein retention receptor 3
Chromosome 22 · 22q13.1
NCBI Gene: 11015Ensembl: ENSG00000100196.12HGNC: HGNC:6306UniProt: O43731
26PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
ReceptorTransporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
COPI-coated vesicle membraneGolgi membraneendoplasmic reticulum membraneKDEL sequence bindingchondromalaciagallstonesprostate carcinomacholelithiasis
✦AI Summary

KDELR3 is an endoplasmic reticulum (ER) retention receptor that binds the K-D-E-L C-terminal motif on ER-resident proteins and mediates their recycling from the Golgi back to the ER via COPI-coated vesicles 1. Beyond its canonical retrograde transport function, KDELR3 plays critical roles in regulating ER stress responses and the unfolded protein response (UPR). In lung adenocarcinoma, KDELR3 suppresses ER stress-induced apoptosis by inhibiting UPR pathway activation (PERK/EIF2α phosphorylation), promoting tumor growth and metastasis; this function is transcriptionally controlled by FOXM1 1. KDELR3 expression is significantly elevated in multiple malignancies including glioma and uveal melanoma, where it serves as an independent adverse prognostic indicator and correlates with immune cell infiltration 2, 3. In metabolic disease, a Kdelr3 mutation impairs pancreatic beta cell function and insulin granule maturation, contributing to type 2 diabetes susceptibility 4. KDELR3 is also dysregulated in non-malignant conditions including diabetic foot ulcers, osteoarthritis, and membranous nephropathy, where it functions as a diagnostic biomarker 5, 6, 7. During adipose-derived stem cell osteogenic differentiation, KDELR3 upregulation correlates with loss of stemness 8. These findings establish KDELR3 as a multifunctional protein linking ER homeostasis to disease pathogenesis across diverse tissues.

Sources cited
1
KDELR3 regulates ER stress and UPR; transcriptionally activated by FOXM1; inhibits ER stress-induced apoptosis in lung adenocarcinoma, promoting tumor growth and metastasis
PMID: 40411680
2
KDELR3 is overexpressed in glioma and serves as an independent prognostic biomarker for adverse outcomes
PMID: 39730475
3
KDELR3 is highly expressed in uveal melanoma; high expression predicts shorter overall and disease-free survival; correlates with tumor-infiltrating lymphocytes and immune checkpoints
PMID: 36046379
4
Kdelr3 mutation in mice impairs pancreatic beta cell function, insulin granule maturation, and contributes to type 2 diabetes susceptibility via altered ER stress responses
PMID: 36614300
5
KDELR3 is identified as a critical ER stress mediator with diagnostic potential as a biomarker in diabetic foot ulcers
PMID: 40354856
6
KDELR3 is identified as a hub gene and diagnostic biomarker for osteoarthritis using machine learning; correlates with immune cell infiltration
PMID: 37454542
7
KDELR3 is upregulated during osteogenic differentiation of adipose-derived stem cells and negatively correlates with cell stemness
PMID: 40100054
8
KDELR3 is identified as a diagnostic marker for PLA2R-associated idiopathic membranous nephropathy
PMID: 39806779
Disease Associationsⓘ20
chondromalaciaOpen Targets
0.27Weak
gallstonesOpen Targets
0.24Weak
prostate carcinomaOpen Targets
0.21Weak
cholelithiasisOpen Targets
0.21Weak
thyroid diseaseOpen Targets
0.14Weak
response to antihypertensive drugOpen Targets
0.12Weak
coronary artery diseaseOpen Targets
0.12Weak
coronary atherosclerosisOpen Targets
0.11Weak
menopauseOpen Targets
0.10Weak
brain infarctionOpen Targets
0.10Suggestive
cerebral infarctionOpen Targets
0.10Suggestive
hypothyroidismOpen Targets
0.08Suggestive
melanomaOpen Targets
0.08Suggestive
gliomaOpen Targets
0.07Suggestive
Hashimoto's thyroiditisOpen Targets
0.06Suggestive
metastatic melanomaOpen Targets
0.05Suggestive
ulnar-mammary syndromeOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
Barrett's esophagusOpen Targets
0.04Suggestive
mathematical abilityOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RER1Shared pathway100%KDELR1Protein interaction95%KDELR2Protein interaction95%ARF4Protein interaction92%SCYL1Shared pathway50%ERGIC1Shared pathway33%
Tissue Expression6 tissues
Heart
100%
Liver
54%
Lung
41%
Bone Marrow
17%
Ovary
14%
Brain
7%
Gene Interaction Network
Click a node to explore
KDELR3RER1KDELR1KDELR2ARF4SCYL1ERGIC1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O43731
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.39LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.96 [0.67–1.39]
RankingsWhere KDELR3 stands among ~20K protein-coding genes
  • #12,810of 20,598
    Most Researched26
  • #14,452of 17,882
    Most Constrained (LOEUF)1.39
Genes detectedKDELR3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
KDELR3 and YOD1 proteins as critical endoplasmic reticulum stress mediators and potential therapeutic targets in diabetic foot ulcers: An integrated bioinformatics analysis.
PMID: 40354856
Int J Biol Macromol · 2025
1.00
2
PMID: 39112444
0.90
3
KDELR3 is transcriptionally activated by FOXM1 and accelerates lung adenocarcinoma growth and metastasis via inhibiting endoplasmic reticulum stress-induced cell apoptosis.
PMID: 40411680
Hum Cell · 2025
0.80
4
KDELR3 overexpression as a novel prognostic and diagnostic biomarker in glioma: comprehensive bioinformatic analysis insights.
PMID: 39730475
Sci Rep · 2024
0.70
5
Exploring NamiRNA networks and time-series gene expression in osteogenic differentiation of adipose-derived stem cells.
PMID: 40100054
Ann Med · 2025
0.60