Affinage

KDELR1

ER lumen protein-retaining receptor 1 · UniProt P24390

Round 2 corrected
Length
212 aa
Mass
24.5 kDa
Annotated
2026-04-28
67 papers in source corpus 21 papers cited in narrative 21 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

KDELR1 is a seven-transmembrane Golgi-resident receptor that recognizes KDEL and related C-terminal retention motifs on escaped ER-lumenal proteins and mediates their COPI-dependent retrograde retrieval from the Golgi to the ER, thereby maintaining ER proteostasis (PMID:2194670, PMID:2172835, PMID:2194671). Ligand binding depends on charged transmembrane residues and a lumenal 22KIWK25 motif, triggers receptor oligomerization, recruits ARFGAP1 to Golgi membranes, and requires PKA-dependent phosphorylation of Ser209 for retrograde transport (PMID:8392934, PMID:9642148, PMID:9405360, PMID:14517323). Beyond cargo retrieval, KDELR1 functions as a GPCR-like signaling platform that activates Gαq/11- and Gs-PKA-dependent cascades to coordinate anterograde and retrograde membrane traffic and to modulate ER stress responses via p38/JNK MAPK signaling (PMID:22580821, PMID:25117681, PMID:12821650). Loss-of-function mutations in mice cause cell-intrinsic lymphopenia driven by excessive integrated stress responses and impaired naive T-cell survival, establishing a nonredundant role for KDELR1 in lymphocyte homeostasis (PMID:26438836, PMID:26489882).

Mechanistic history

Synthesis pass · year-by-year structured walk · 13 steps
  1. 1990 High

    Identification of ERD2/KDELR1 as the ER retention receptor resolved how cells retrieve escaped lumenal proteins: the receptor's abundance sets system capacity, and species-specific ERD2 exchange switches retention-signal specificity, proving direct receptor–ligand determination.

    Evidence Yeast ERD2 gene manipulation, secretion assays, and inter-species gene exchange between S. cerevisiae and K. lactis; cloning and biochemical characterization of human homologue

    PMID:2172835 PMID:2194670 PMID:2194671

    Open questions at the time
    • Structural basis of KDEL signal recognition not determined
    • How receptor cycles between Golgi and ER was unknown
    • Redundancy among mammalian KDELR paralogs unaddressed
  2. 1993 High

    Systematic mutagenesis established that ligand binding requires charged transmembrane residues and that retrograde transport depends on a specific aspartate in TM7, separating Golgi retention from recycling functions mechanistically.

    Evidence Transmembrane point mutagenesis in COS cells with ligand-binding and localization readouts; ligand overexpression-induced receptor redistribution and specificity-altering mutations

    PMID:1310258 PMID:7929564 PMID:8392934

    Open questions at the time
    • Identity of coat machinery coupling the receptor to COPI vesicles was unclear
    • Lumenal residues involved in ligand binding not yet mapped
  3. 1998 Medium

    Mapping of the lumenal ligand-binding site to the 22KIWK25 motif defined the minimal receptor determinant contacting KDEL-containing proteins.

    Evidence Cellulose-bound overlapping peptide arrays with soluble KDEL peptide competition

    PMID:9642148

    Open questions at the time
    • In vitro peptide assay only; not confirmed by crystallography or in-cell crosslinking
    • Contribution of individual residues within KIWK not dissected
  4. 2001 High

    Live-cell FRET imaging revealed that ligand binding induces receptor oligomerization and recruits ARFGAP to Golgi membranes, establishing the mechanistic link between cargo recognition and COPI bud formation.

    Evidence CFP/YFP FRET with multifocal multiphoton microscopy measuring interactions among ERD2, ARFGAP, ARF1, β-COP, and p23 in living cells

    PMID:11703931 PMID:9405360

    Open questions at the time
    • Stoichiometry of the oligomer and precise ARFGAP interaction surface unknown
    • Whether oligomerization is required for signaling (vs. transport) not tested
  5. 2003 High

    Two parallel regulatory inputs were defined: PKA-dependent Ser209 phosphorylation is required for coatomer/ARFGAP engagement and retrograde transport, while Src kinase activity controls receptor steady-state distribution between Golgi and ER.

    Evidence Point and truncation mutagenesis, PKA inhibitor H89, permeabilized-cell transport assays; activated Src expression and SYF knockout cells with toxin transport readouts

    PMID:12975382 PMID:14517323

    Open questions at the time
    • Direct Src phosphorylation site on KDELR1 not identified
    • Relationship between Src and PKA inputs not integrated
  6. 2003 High

    Loss of KDELR1 retrieval function induces ER stress with p38/JNK MAPK activation, establishing KDELR1 as a modulator of ER stress signaling beyond simple cargo sorting.

    Evidence Dominant-negative KDELR1 mutant expression, BiP mis-sorting, p38/JNK phosphorylation assays, p38 inhibitor studies in HeLa cells

    PMID:12821650

    Open questions at the time
    • Whether MAPK activation is a direct receptor signal or secondary to ER stress was not resolved
    • Downstream transcriptional targets of p38/JNK in this context not identified
  7. 2007 High

    A systematic BiFC screen of 152 KDEL variants showed that KDELR1, KDELR2, and KDELR3 each have unique ligand-specificity profiles, explaining how the three paralogs partition retrieval duties.

    Evidence Bimolecular fluorescence complementation screen with 152 C-terminal peptide variants and Golgi colocalization

    PMID:18086916

    Open questions at the time
    • Functional consequence of differential specificity on ER proteome maintenance not tested
    • Structural basis of specificity divergence unknown
  8. 2012 High

    Discovery that KDELR1 adopts a GPCR-like fold and directly activates Gαq/11 reframed the receptor as a signaling platform, not just a cargo sorter, coupling ligand occupancy to heterotrimeric G-protein cascades that regulate Golgi transport.

    Evidence GPCR structural modeling, G-protein binding and activation assays, Golgi transport assays

    PMID:22580821

    Open questions at the time
    • High-resolution structure of KDELR1 bound to Gα subunit lacking
    • How Gαq/11 and Gs pathways are differentially engaged was unclear
  9. 2014 High

    A complete Gs–adenylyl cyclase–PKA signaling cascade downstream of KDELR1 was mapped, showing that anterograde cargo flux activates the receptor to drive compensatory retrograde traffic and to upregulate transport gene expression via CREB1.

    Evidence Pharmacological dissection of signaling components, phosphorylation and transcription factor activation assays

    PMID:25117681

    Open questions at the time
    • Identity of specific transport genes upregulated via CREB1 not fully catalogued
    • Quantitative relationship between cargo load and signaling output not measured
  10. 2015 High

    In vivo loss-of-function established that KDELR1 is nonredundant for lymphocyte homeostasis: mutant mice show cell-intrinsic lymphopenia driven by excessive integrated stress responses in naive T cells, which can be counteracted by strong TCR signals.

    Evidence ENU missense and CRISPR/Cas9 frameshift alleles in mice, bone marrow chimeras, TCR transgenic rescue, tetramer dissociation assays

    PMID:26438836 PMID:26489882

    Open questions at the time
    • Specific ER client(s) whose mis-sorting triggers ISR in T cells not identified
    • Whether KDELR2/3 are expressed in T cells and why they fail to compensate not determined
  11. 2019 Medium

    KDELR1 knockout in human cells revealed a requirement for normal cell adhesion and ER stress resilience, with transcriptional remodeling of ECM and adhesion gene programs upon receptor loss.

    Evidence HAP1 KDELR1 knockout, transcriptomics, PDI secretion assay, adhesion assays with collagen/laminin rescue

    PMID:31337861

    Open questions at the time
    • Mechanism linking KDELR1 loss to adhesion gene upregulation not resolved
    • Single cell line; generalizability across tissues not tested
  12. 2024 Medium

    Multi-omics analysis linked KDELR1 to Integrin–PLCγ–YAP1 (Hippo) signaling in chondrosarcoma, suggesting a role in drug resistance through ECM-related pathway modulation.

    Evidence Single-cell transcriptomics and mass spectrometry proteomics with KDELR1 knockdown/overexpression in chondrosarcoma cells

    PMID:39715773

    Open questions at the time
    • Direct molecular connection between KDELR1 and Integrin-PLCγ axis not biochemically validated
    • Applicability beyond chondrosarcoma not assessed
  13. 2026 Medium

    Epistasis experiments showed KDELR1 and KDELR3 exert opposing transcriptional control over AGR2, with ERp44 mediating the KDELR3 effect, defining a paralog-specific regulatory circuit independent of the unfolded protein response.

    Evidence siRNA silencing of individual KDELRs and ER-resident proteins with AGR2 transcript readout

    PMID:41706164

    Open questions at the time
    • Mechanistic basis of KDELR1's positive effect on AGR2 transcription unknown
    • Whether this circuit operates via KDELR1 signaling or simply cargo retrieval not distinguished

Open questions

Synthesis pass · forward-looking unresolved questions
  • A high-resolution structure of KDELR1 in complex with a KDEL ligand and/or G-protein, and identification of the specific ER client(s) whose mis-sorting drives T-cell ISR, remain the principal open questions for fully integrating the receptor's cargo-sorting and signaling functions.
  • No atomic-resolution structure of ligand-bound or G-protein-bound KDELR1
  • Critical ER client(s) mediating T-cell survival phenotype not identified
  • Quantitative model relating cargo load to Gs vs. Gαq signaling output absent

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0038024 cargo receptor activity 6 GO:0098772 molecular function regulator activity 4 GO:0060089 molecular transducer activity 2
Localization
GO:0005794 Golgi apparatus 4 GO:0005783 endoplasmic reticulum 2
Pathway
R-HSA-9609507 Protein localization 4 R-HSA-162582 Signal Transduction 3 R-HSA-8953897 Cellular responses to stimuli 3 R-HSA-168256 Immune System 2
Partners
ARF1ARFGAP1COPB1GNAQGNASTMED10
Complex memberships
KDELR1 homo-oligomerKDELR1–ARFGAP1–ARF1 complex

Evidence

Reading pass · 21 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1990 The yeast ERD2 gene encodes a 26 kDa integral membrane protein (the HDEL receptor) whose abundance determines the efficiency and capacity of the ER protein retention/retrieval system; reduced ERD2 expression causes secretion of HDEL-tagged proteins, while overexpression improves retention. Yeast genetics, gene expression manipulation, secretion assays Cell High 2194670
1990 A human homologue of yeast ERD2 (hERD2/KDELR1) encodes a ~26 kDa non-glycosylated integral membrane protein with properties similar to the yeast HDEL receptor, proposed to be the mammalian KDEL receptor. Sequence analysis, biochemical characterization of the expressed protein Nature High 2172835
1990 The specificity of the luminal ER protein retention system is determined by the ERD2 gene product (the receptor): exchanging the ERD2 gene between S. cerevisiae and K. lactis alters which C-terminal signals (HDEL vs. DDEL) are recognized, proving ERD2 encodes the sorting receptor. Inter-species gene exchange, retention signal specificity assays Cell High 2194671
1992 Ligand overexpression causes redistribution of hERD2 (KDELR1) from the Golgi apparatus to the ER; mutation of hERD2 alters ligand specificity of this redistribution, demonstrating direct receptor-ligand interaction and ligand-induced receptor movement as a regulatory mechanism. Overexpression of KDEL/DDEL ligands, immunolocalization, site-directed mutagenesis Cell High 1310258
1993 Ligand binding to the human KDEL receptor depends on charged residues within the transmembrane domains; retrograde transport of occupied receptor requires a critical aspartic acid in the seventh transmembrane domain; Golgi retention is independent of ligand binding and this aspartate, indicating distinct structural requirements for each function. Mutagenesis of transmembrane residues, intracellular localization assays, ligand-binding assays in COS cells The EMBO journal High 8392934
1994 Growth of yeast requires the HDEL-dependent retrieval activity of Erd2p (KDELR1 ortholog); mutations that block receptor recycling also prevent growth; Golgi retention of the receptor is independent of recycling, but retrieval of specific HDEL-containing proteins is essential for viability. Erd2p mutant analysis, receptor overexpression saturation, viability assays The Journal of cell biology High 7929564
1997 The KDEL receptor ERD2 (KDELR1 ortholog) self-oligomerizes and interacts with ARF1 GTPase-activating protein (ARF1 GAP), recruiting cytosolic ARF1 GAP to membranes; ERD2 overexpression enhances GAP membrane recruitment and produces a phenotype reflecting ARF1 inactivation, indicating ERD2 regulates ARF1-mediated vesicle transport. Co-immunoprecipitation, overexpression phenotype analysis, membrane recruitment assays The EMBO journal High 9405360
1997 Overexpression of either ERD2.1 or ERD2.2 (KDELR1 paralogs) significantly increases cellular capacity to retain both KDEL- and HNEL-containing proteins; pulse-chase and immunolocalization show long half-life and Golgi localization for both receptors, and the novel HNEL signal of RAP interacts with the same ER retention receptors as KDEL. Stable transfection, pulse-chase labeling, immunoelectron microscopy, retention assays Journal of cell science High 9010785
1998 The sequence 22KIWK25 within a lumenal loop of the human KDEL receptor (ERD2/KDELR1) is essential for binding to KDEL-containing ER lumenal proteins (CaBP1 and CaBP2); binding is of high specificity and almost completely inhibited by KDEL-containing soluble peptides. Cellulose-bound overlapping peptide arrays, binding inhibition with KDEL peptides Biochemical and biophysical research communications Medium 9642148
2001 KDEL ligand binding induces oligomerization of ERD2 (KDELR1) and recruitment of ARFGAP to the Golgi, where the ERD2 oligomer/ARFGAP complex interacts with membrane-bound ARF1; during KDEL ligand transport, ERD2 interactions with β-COP and p23 decrease and the proteins segregate, revealing how cargo-induced ERD2 oligomerization regulates sorting into COPI-coated buds. FRET between CFP/YFP fusion proteins by multifocal multiphoton microscopy in living cells Developmental cell High 11703931
2003 Impairment of KDEL receptor (KDELR1) retrieval function by expression of a ligand-recognition mutant causes mis-sorting of the ER chaperone BiP and induces intense ER stress, accompanied by activation of p38 MAP kinase and JNK1; ligand-induced activation of the KDEL receptor also induces phosphorylation of p38 MAP kinase, indicating the KDEL receptor modulates ER stress response through MAPK signaling. Mutant KDEL receptor expression, ER stress assays, MAP kinase phosphorylation assays, p38 inhibitor studies in HeLa cells The Journal of biological chemistry High 12821650
2003 PKA phosphorylation of serine 209 in the C-terminal cytoplasmic domain of the KDEL receptor (KDELR1) is required for retrograde Golgi-to-ER transport of the receptor and for intracellular retention of KDEL ligands; this domain interacts with coatomer and ARF-GAP only when Ser209 is phosphorylated (mimicked by S209D mutation); inhibition of PKA with H89 blocks receptor redistribution to the ER. Truncation and point mutagenesis, peptide-binding assays with coatomer/ARF-GAP, PKA inhibitor (H89), permeabilized cell transport assays Molecular biology of the cell High 14517323
2003 Src kinase activity controls KDEL receptor (KDELR1) localization: activated Src relocates KDEL-R from the Golgi to the ER, and loss of Src (in SYF cells) perturbs Golgi organization; retrograde transport of Pseudomonas exotoxin (which uses the KDEL-R) is accelerated by Src inhibition or ablation. Activated Src expression, SYF knockout cell line, immunofluorescence localization, toxin transport assays The Journal of biological chemistry High 12975382
2007 Three human KDEL receptors (KDELR1, KDELR2, KDELR3) each have unique specificity profiles for KDEL-like C-terminal motifs; KDELR1 interacts with a distinct subset of KDEL variants compared to KDELR2 and KDELR3, as determined by a bimolecular fluorescence complementation screen; all three receptors localize to the Golgi. Reporter construct screen of 152 KDEL variants, bimolecular fluorescence complementation (BiFC) to determine receptor-ligand specificity, Golgi localization assays The Journal of cell biology High 18086916
2012 The KDEL receptor (KDELR1) is predicted to fold like a G-protein-coupled receptor (GPCR) and directly binds and activates the heterotrimeric G-protein Gαq/11; this Gαq/11 activation regulates transport through the Golgi complex, revealing an unexpected GPCR-like signaling mode for the KDEL receptor. GPCR structural prediction, G-protein binding and activation assays, Golgi transport assays The EMBO journal High 22580821
2014 ER-to-Golgi cargo transport activates the KDEL receptor (KDELR1) at the Golgi, which triggers a signaling cascade involving Gs protein, adenylyl cyclase, phosphodiesterase isoforms, and PKA, leading to phosphorylation of transport machinery proteins; this induces retrograde traffic to the ER to balance anterograde flux; additionally, the KDEL receptor activates CREB1 and other transcription factors that upregulate transport-related genes. Pharmacological perturbations of signaling components, phosphorylation assays, transcription factor activation assays Developmental cell High 25117681
2015 A recessive missense allele of KDELR1 in mice causes cell-intrinsic lymphopenia; homozygous mutant and CRISPR/Cas9 frameshift T cells show reduced TCR surface expression, increased CD44, and impaired viral clearance; the phenotype can be partially corrected by an MHC class I-restricted TCR transgene, revealing a nonredundant role for KDELR1 in lymphocyte homeostasis. Mouse genetics (ENU missense and CRISPR/Cas9 frameshift alleles), bone marrow chimeras for cell-intrinsic analysis, TCR transgene rescue, flow cytometry Proceedings of the National Academy of Sciences of the United States of America High 26438836
2015 KDELR1 regulates integrated stress responses (ISR) to promote naive T-cell survival; in mice with nonfunctional KDELR1 (T-Red mice), naive T cells show excessive ISR and undergo apoptosis; strong TCR-mediated signals suppress ISR, and surviving naive T cells in KDELR1-deficient mice express higher CD5 and exhibit higher TCR affinity, demonstrating that KDELR1 deficiency-induced ISR can be counteracted by strong TCR signals. T-Red mouse model, TCR transgenic rescue, tetramer dissociation assay, altered peptide ligand stimulation, ISR markers International immunology High 26489882
2019 KDELR1 knockout (HAP1 cells) causes increased secretion of the ER-resident protein PDI, decreased cell viability under ER stress, transcriptional upregulation of genes involved in cell adhesion and ECM composition, and impaired cell adhesion capacity that is partially rescued by collagen/laminin coating, indicating KDELR1 is required for ER homeostasis and normal cell adhesion. KDELR1 knockout cell line, whole transcriptome analysis, in vitro adhesion assays, PDI secretion assay, ER stress viability assay Scientific reports Medium 31337861
2024 KDELR1 contributes to chondrosarcoma drug resistance and malignant behavior through the Integrin-PLCγ-YAP1 (Hippo) signaling axis; mass spectrometry proteomics and transcriptomics revealed KDELR1 modulates Hippo-YAP pathway activity in chondrosarcoma cells, affecting ECM formation and chemotherapy resistance. Single-cell transcriptomics, mass spectrometry proteomics, KDELR1 knockdown/overexpression with proliferation and drug resistance assays Cell death & disease Medium 39715773
2026 KDELR1 and KDELR3 have opposite effects on AGR2 (a mucin folding assistant) production: KDELR1 downregulation decreases AGR2 transcripts while KDELR3 silencing dramatically increases them; silencing ERp44 (but not other ER residents) phenocopies KDELR3 knockdown, suggesting AGR2 regulation by KDELR3 depends on ERp44-KDELR3 interactions; this defines a novel regulatory circuit controlling early secretory pathway composition distinct from the unfolded protein response. siRNA silencing of KDELR1, KDELR2, KDELR3, and ER residents; transcriptional readout of AGR2; phenocopy experiments with ERp44 silencing Cellular and molecular life sciences : CMLS Medium 41706164

Source papers

Stage 0 corpus · 67 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proceedings of the National Academy of Sciences of the United States of America 1479 12477932
2017 Architecture of the human interactome defines protein communities and disease networks. Nature 1085 28514442
2020 A reference map of the human binary protein interactome. Nature 849 32296183
2007 Large-scale mapping of human protein-protein interactions by mass spectrometry. Molecular systems biology 733 17353931
2021 Dual proteome-scale networks reveal cell-specific remodeling of the human interactome. Cell 705 33961781
2011 Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Briefings in bioinformatics 656 21873635
2020 Comparative host-coronavirus protein interaction networks reveal pan-viral disease mechanisms. Science (New York, N.Y.) 564 33060197
2021 Multilevel proteomics reveals host perturbations by SARS-CoV-2 and SARS-CoV. Nature 532 33845483
1990 ERD2, a yeast gene required for the receptor-mediated retrieval of luminal ER proteins from the secretory pathway. Cell 464 2194670
2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome research 438 15489334
1992 Ligand-induced redistribution of a human KDEL receptor from the Golgi complex to the endoplasmic reticulum. Cell 365 1310258
2021 A proximity-dependent biotinylation map of a human cell. Nature 339 34079125
2003 Large-scale identification and characterization of human genes that activate NF-kappaB and MAPK signaling pathways. Oncogene 331 12761501
1998 gp25L/emp24/p24 protein family members of the cis-Golgi network bind both COP I and II coatomer. The Journal of cell biology 294 9472029
1990 A human homologue of the yeast HDEL receptor. Nature 291 2172835
2007 A molecular specificity code for the three mammalian KDEL receptors. The Journal of cell biology 214 18086916
1990 The ERD2 gene determines the specificity of the luminal ER protein retention system. Cell 211 2194671
2015 ∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis. Nature 209 26618866
1996 Endoplasmic reticulum glucosidase II is composed of a catalytic subunit, conserved from yeast to mammals, and a tightly bound noncatalytic HDEL-containing subunit. The Journal of biological chemistry 197 8910335
1997 The C-terminal HDEL sequence is sufficient for retention of secretory proteins in the endoplasmic reticulum (ER) but promotes vacuolar targeting of proteins that escape the ER. The Plant journal : for cell and molecular biology 165 9076996
2020 AMPK, a Regulator of Metabolism and Autophagy, Is Activated by Lysosomal Damage via a Novel Galectin-Directed Ubiquitin Signal Transduction System. Molecular cell 152 31995728
1992 Immunological evidence that plants use both HDEL and KDEL for targeting proteins to the endoplasmic reticulum. Journal of cell science 151 1383243
2001 KDEL-cargo regulates interactions between proteins involved in COPI vesicle traffic: measurements in living cells using FRET. Developmental cell 149 11703931
1997 The KDEL receptor, ERD2, regulates intracellular traffic by recruiting a GTPase-activating protein for ARF1. The EMBO journal 149 9405360
1993 Identification and localization of ERD2 in the malaria parasite Plasmodium falciparum: separation from sites of sphingomyelin synthesis and implications for organization of the Golgi. The EMBO journal 149 8223485
2019 Mapping the proximity interaction network of the Rho-family GTPases reveals signalling pathways and regulatory mechanisms. Nature cell biology 137 31871319
1993 Reticulocalbin, a novel endoplasmic reticulum resident Ca(2+)-binding protein with multiple EF-hand motifs and a carboxyl-terminal HDEL sequence. The Journal of biological chemistry 137 8416973
1993 Mutational analysis of the human KDEL receptor: distinct structural requirements for Golgi retention, ligand binding and retrograde transport. The EMBO journal 127 8392934
2000 A transcript map of the chromosome 19q-arm glioma tumor suppressor region. Genomics 118 10708517
2023 ESCRT-dependent STING degradation inhibits steady-state and cGAMP-induced signalling. Nature communications 104 36739287
2012 The KDEL receptor couples to Gαq/11 to activate Src kinases and regulate transport through the Golgi. The EMBO journal 103 22580821
1992 Genes that allow yeast cells to grow in the absence of the HDEL receptor. The EMBO journal 101 1327759
2004 Proteomics of endoplasmic reticulum-Golgi intermediate compartment (ERGIC) membranes from brefeldin A-treated HepG2 cells identifies ERGIC-32, a new cycling protein that interacts with human Erv46. The Journal of biological chemistry 98 15308636
2014 Control systems of membrane transport at the interface between the endoplasmic reticulum and the Golgi. Developmental cell 92 25117681
2012 Charting the landscape of tandem BRCT domain-mediated protein interactions. Science signaling 92 22990118
2003 Src regulates Golgi structure and KDEL receptor-dependent retrograde transport to the endoplasmic reticulum. The Journal of biological chemistry 91 12975382
1996 The dynamic organisation of the secretory pathway. Cell structure and function 87 9118249
1992 The Saccharomyces cerevisiae SEC20 gene encodes a membrane glycoprotein which is sorted by the HDEL retrieval system. The EMBO journal 85 1537327
2003 The KDEL receptor modulates the endoplasmic reticulum stress response through mitogen-activated protein kinase signaling cascades. The Journal of biological chemistry 84 12821650
2014 Human-chromatin-related protein interactions identify a demethylase complex required for chromosome segregation. Cell reports 80 24981860
2003 The retrieval function of the KDEL receptor requires PKA phosphorylation of its C-terminus. Molecular biology of the cell 78 14517323
1996 Identification and localization of rab6, separation of rab6 from ERD2 and implications for an 'unstacked' Golgi, in Plasmodium falciparum. Molecular and biochemical parasitology 66 9010846
2001 Use of HDEL-tagged Trichoderma reesei mannosyl oligosaccharide 1,2-alpha-D-mannosidase for N-glycan engineering in Pichia pastoris. FEBS letters 48 11513877
1994 Retrieval of HDEL proteins is required for growth of yeast cells. The Journal of cell biology 38 7929564
2012 Plant ERD2-like proteins function as endoplasmic reticulum luminal protein receptors and participate in programmed cell death during innate immunity. The Plant journal : for cell and molecular biology 37 22595145
1997 ERD2 proteins mediate ER retention of the HNEL signal of LRP's receptor-associated protein (RAP). Journal of cell science 35 9010785
2014 Arabidopsis p24δ5 and p24δ9 facilitate Coat Protein I-dependent transport of the K/HDEL receptor ERD2 from the Golgi to the endoplasmic reticulum. The Plant journal : for cell and molecular biology 31 25312353
2004 Biodistribution and safety studies of hDel-1 plasmid-based gene therapy in mouse and rabbit models. The Journal of pharmacy and pharmacology 24 15005876
2018 Loss of Arabidopsis p24 function affects ERD2 trafficking and Golgi structure, and activates the unfolded protein response. Journal of cell science 22 28871045
2003 Fusion with HDEL protects cell wall invertase from early degradation when N-glycosylation is inhibited. Plant & cell physiology 21 12610220
2018 Predominant Golgi Residency of the Plant K/HDEL Receptor Is Essential for Its Function in Mediating ER Retention. The Plant cell 19 30072420
2020 A Model for ERD2 Function in Higher Plants. Frontiers in plant science 15 32269585
2015 Mutation of the ER retention receptor KDELR1 leads to cell-intrinsic lymphopenia and a failure to control chronic viral infection. Proceedings of the National Academy of Sciences of the United States of America 15 26438836
2023 The K/HDEL receptor does not recycle but instead acts as a Golgi-gatekeeper. Nature communications 13 36959220
1997 Characterization of the bip gene of Aspergillus awamori encoding a protein with an HDEL retention signal homologous to the mammalian BiP involved in polypeptide secretion. Current genetics 13 9294262
1992 A mutant Kex2 enzyme with a C-terminal HDEL sequence releases correctly folded human insulin-like growth factor-1 from a precursor accumulated in the yeast endoplasmic reticulum. European journal of biochemistry 12 1483466
2017 N-Linked Glycosylation of the p24 Family Protein p24δ5 Modulates Retrograde Golgi-to-ER Transport of K/HDEL Ligands in Arabidopsis. Molecular plant 10 28735024
2019 Transcriptomics of a KDELR1 knockout cell line reveals modulated cell adhesion properties. Scientific reports 9 31337861
2018 The B cell death function of obinutuzumab-HDEL produced in plant (Nicotiana benthamiana L.) is equivalent to obinutuzumab produced in CHO cells. PloS one 8 29324849
2015 Strong TCR-mediated signals suppress integrated stress responses induced by KDELR1 deficiency in naive T cells. International immunology 8 26489882
2024 KDELR1 regulates chondrosarcoma drug resistance and malignant behavior through Intergrin-Hippo-YAP1 axis. Cell death & disease 6 39715773
2021 Allele-specific suppression in Caenorhabditis elegans reveals details of EMS mutagenesis and a possible moonlighting interaction between the vesicular acetylcholine transporter and ERD2 receptors. Genetics 4 33914877
1998 KDEL motif interacts with a specific sequence in mammalian erd2 receptor. Biochemical and biophysical research communications 4 9642148
2025 A multi-omics analysis reveals KDELR1 promotes malignant progression and correlates with tumor microenvironment in head and neck squamous cell carcinoma. Cellular signalling 2 39875049
2019 Arabidopsis ENDOMEMBRANE PROTEIN 12 contributes to the endoplasmic reticulum stress response by regulating K/HDEL receptor trafficking. The Plant cell 1 31123049
2026 A regulatory circuit operated by KDELR1 and KDELR3 fine-tunes the composition of the early secretory pathway. Cellular and molecular life sciences : CMLS 0 41706164
2018 Correction: The B cell death function of obinutuzumab-HDEL produced in plant (Nicotiana benthamiana L.) is equivalent to obinutuzumab produced in CHO cells. PloS one 0 29641595