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Protein

ER lumen protein-retaining receptor 1

Gene

KDELR1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Required for the retention of luminal endoplasmic reticulum resident proteins via vesicular recycling. This receptor recognizes the C-terminal K-D-E-L motif. COPI-coated transport intermediates, either in the form of round vesicles or as tubular processes, mediate retrograde traffic of the KDEL receptor-ligand complexes. Also required for normal vesicular traffic through the Golgi.1 Publication

GO - Molecular functioni

  • KDEL sequence binding Source: ProtInc

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Receptor

Keywords - Biological processi

ER-Golgi transport, Protein transport, Transport

Enzyme and pathway databases

ReactomeiR-HSA-6807878. COPI-mediated anterograde transport.
R-HSA-6811434. COPI-dependent Golgi-to-ER retrograde traffic.
SIGNORiP24390.

Protein family/group databases

TCDBi9.B.191.1.5. the endoplasmic reticulum retention receptor (kdelr) family.

Names & Taxonomyi

Protein namesi
Recommended name:
ER lumen protein-retaining receptor 1
Alternative name(s):
KDEL endoplasmic reticulum protein retention receptor 1
Short name:
KDEL receptor 1
Putative MAPK-activating protein PM23
Gene namesi
Name:KDELR1
Synonyms:ERD2.1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:6304. KDELR1.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 22LumenalSequence analysis
Transmembranei3 – 2119HelicalSequence analysisAdd
BLAST
Topological domaini22 – 3514CytoplasmicSequence analysisAdd
BLAST
Transmembranei36 – 5318HelicalSequence analysisAdd
BLAST
Topological domaini54 – 618LumenalSequence analysis
Transmembranei62 – 8019HelicalSequence analysisAdd
BLAST
Topological domaini81 – 9616CytoplasmicSequence analysisAdd
BLAST
Transmembranei97 – 11014HelicalSequence analysisAdd
BLAST
Topological domaini111 – 1177LumenalSequence analysis
Transmembranei118 – 13720HelicalSequence analysisAdd
BLAST
Topological domaini138 – 14912CytoplasmicSequence analysisAdd
BLAST
Transmembranei150 – 16819HelicalSequence analysisAdd
BLAST
Topological domaini169 – 17810LumenalSequence analysis
Transmembranei179 – 19921HelicalSequence analysisAdd
BLAST
Topological domaini200 – 21213CytoplasmicSequence analysisAdd
BLAST

GO - Cellular componenti

  • cis-Golgi network Source: Ensembl
  • COPI-coated vesicle membrane Source: UniProtKB-SubCell
  • endoplasmic reticulum-Golgi intermediate compartment Source: UniProtKB
  • endoplasmic reticulum-Golgi intermediate compartment membrane Source: Reactome
  • endoplasmic reticulum membrane Source: Reactome
  • Golgi membrane Source: Reactome
  • integral component of membrane Source: ProtInc
  • membrane Source: ProtInc
  • transport vesicle Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasmic vesicle, Endoplasmic reticulum, Membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi209 – 2091S → A: Inhibits coatomer/ARF-GAP recruitment, receptor redistribution, and intracellular retention of KDEL ligands. 1 Publication
Mutagenesisi209 – 2091S → D: Redistribution to the ER is not affected upon PKA inactivation. 1 Publication

Organism-specific databases

PharmGKBiPA30083.

Polymorphism and mutation databases

BioMutaiKDELR1.
DMDMi119543.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 212212ER lumen protein-retaining receptor 1PRO_0000194153Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei209 – 2091Phosphoserine; by PKA1 Publication

Post-translational modificationi

Phosphorylation by PKA at Ser-209 is required for endoplasmic reticulum retention function.1 Publication

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP24390.
MaxQBiP24390.
PaxDbiP24390.
PRIDEiP24390.
TopDownProteomicsiP24390-1. [P24390-1]

PTM databases

iPTMnetiP24390.
PhosphoSiteiP24390.

Expressioni

Gene expression databases

BgeeiP24390.
CleanExiHS_KDELR1.
ExpressionAtlasiP24390. baseline and differential.
GenevisibleiP24390. HS.

Organism-specific databases

HPAiCAB017455.
HPA047440.

Interactioni

Subunit structurei

Upon ligand binding the receptor oligomerizes and interacts with components of the transport machinery such as ARFGAP1 and ARF1.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
BZW1Q7L1Q61EBI-1043076,EBI-1046727
NEK6Q9HC981EBI-1043076,EBI-740364
SERINC3Q135301EBI-1043076,EBI-1045571
WBP5Q9UHQ71EBI-1043076,EBI-1051372

Protein-protein interaction databases

BioGridi116145. 26 interactions.
DIPiDIP-48668N.
IntActiP24390. 6 interactions.
MINTiMINT-3010262.
STRINGi9606.ENSP00000329471.

Structurei

3D structure databases

ProteinModelPortaliP24390.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the ERD2 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3106. Eukaryota.
COG5196. LUCA.
GeneTreeiENSGT00390000004010.
HOGENOMiHOG000231400.
HOVERGENiHBG001398.
InParanoidiP24390.
KOiK10949.
OrthoDBiEOG761BW0.
PhylomeDBiP24390.
TreeFamiTF314792.

Family and domain databases

InterProiIPR000133. ER_ret_rcpt.
[Graphical view]
PANTHERiPTHR10585. PTHR10585. 1 hit.
PfamiPF00810. ER_lumen_recept. 1 hit.
[Graphical view]
PRINTSiPR00660. ERLUMENR.
PROSITEiPS00951. ER_LUMEN_RECEPTOR_1. 1 hit.
PS00952. ER_LUMEN_RECEPTOR_2. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P24390-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MNLFRFLGDL SHLLAIILLL LKIWKSRSCA GISGKSQVLF AVVFTARYLD
60 70 80 90 100
LFTNYISLYN TCMKVVYIAC SFTTVWLIYS KFKATYDGNH DTFRVEFLVV
110 120 130 140 150
PTAILAFLVN HDFTPLEILW TFSIYLESVA ILPQLFMVSK TGEAETITSH
160 170 180 190 200
YLFALGVYRT LYLFNWIWRY HFEGFFDLIA IVAGLVQTVL YCDFFYLYIT
210
KVLKGKKLSL PA
Length:212
Mass (Da):24,542
Last modified:March 1, 1992 - v1
Checksum:iB863C0F09CFC0551
GO
Isoform 2 (identifier: P24390-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-62: Missing.

Note: No experimental confirmation available.
Show »
Length:150
Mass (Da):17,487
Checksum:iE2A47258FC75BC7E
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 6262Missing in isoform 2. 1 PublicationVSP_055484Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X55885 mRNA. Translation: CAA39371.1.
AB097047 mRNA. Translation: BAC77400.1.
AK075185 mRNA. Translation: BAC11457.1.
AK312647 mRNA. Translation: BAG35531.1.
AC011514 Genomic DNA. No translation available.
CH471177 Genomic DNA. Translation: EAW52340.1.
CH471177 Genomic DNA. Translation: EAW52343.1.
CH471177 Genomic DNA. Translation: EAW52345.1.
BC018778 mRNA. Translation: AAH18778.1.
CCDSiCCDS12718.1. [P24390-1]
PIRiS13293.
RefSeqiNP_006792.1. NM_006801.2. [P24390-1]
UniGeneiHs.515515.

Genome annotation databases

EnsembliENST00000330720; ENSP00000329471; ENSG00000105438. [P24390-1]
ENST00000597017; ENSP00000470841; ENSG00000105438. [P24390-2]
GeneIDi10945.
KEGGihsa:10945.
UCSCiuc002pja.2. human. [P24390-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X55885 mRNA. Translation: CAA39371.1.
AB097047 mRNA. Translation: BAC77400.1.
AK075185 mRNA. Translation: BAC11457.1.
AK312647 mRNA. Translation: BAG35531.1.
AC011514 Genomic DNA. No translation available.
CH471177 Genomic DNA. Translation: EAW52340.1.
CH471177 Genomic DNA. Translation: EAW52343.1.
CH471177 Genomic DNA. Translation: EAW52345.1.
BC018778 mRNA. Translation: AAH18778.1.
CCDSiCCDS12718.1. [P24390-1]
PIRiS13293.
RefSeqiNP_006792.1. NM_006801.2. [P24390-1]
UniGeneiHs.515515.

3D structure databases

ProteinModelPortaliP24390.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi116145. 26 interactions.
DIPiDIP-48668N.
IntActiP24390. 6 interactions.
MINTiMINT-3010262.
STRINGi9606.ENSP00000329471.

Protein family/group databases

TCDBi9.B.191.1.5. the endoplasmic reticulum retention receptor (kdelr) family.

PTM databases

iPTMnetiP24390.
PhosphoSiteiP24390.

Polymorphism and mutation databases

BioMutaiKDELR1.
DMDMi119543.

Proteomic databases

EPDiP24390.
MaxQBiP24390.
PaxDbiP24390.
PRIDEiP24390.
TopDownProteomicsiP24390-1. [P24390-1]

Protocols and materials databases

DNASUi10945.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000330720; ENSP00000329471; ENSG00000105438. [P24390-1]
ENST00000597017; ENSP00000470841; ENSG00000105438. [P24390-2]
GeneIDi10945.
KEGGihsa:10945.
UCSCiuc002pja.2. human. [P24390-1]

Organism-specific databases

CTDi10945.
GeneCardsiKDELR1.
HGNCiHGNC:6304. KDELR1.
HPAiCAB017455.
HPA047440.
MIMi131235. gene.
neXtProtiNX_P24390.
PharmGKBiPA30083.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3106. Eukaryota.
COG5196. LUCA.
GeneTreeiENSGT00390000004010.
HOGENOMiHOG000231400.
HOVERGENiHBG001398.
InParanoidiP24390.
KOiK10949.
OrthoDBiEOG761BW0.
PhylomeDBiP24390.
TreeFamiTF314792.

Enzyme and pathway databases

ReactomeiR-HSA-6807878. COPI-mediated anterograde transport.
R-HSA-6811434. COPI-dependent Golgi-to-ER retrograde traffic.
SIGNORiP24390.

Miscellaneous databases

ChiTaRSiKDELR1. human.
GeneWikiiKDELR1.
GenomeRNAii10945.
PROiP24390.
SOURCEiSearch...

Gene expression databases

BgeeiP24390.
CleanExiHS_KDELR1.
ExpressionAtlasiP24390. baseline and differential.
GenevisibleiP24390. HS.

Family and domain databases

InterProiIPR000133. ER_ret_rcpt.
[Graphical view]
PANTHERiPTHR10585. PTHR10585. 1 hit.
PfamiPF00810. ER_lumen_recept. 1 hit.
[Graphical view]
PRINTSiPR00660. ERLUMENR.
PROSITEiPS00951. ER_LUMEN_RECEPTOR_1. 1 hit.
PS00952. ER_LUMEN_RECEPTOR_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "A human homologue of the yeast HDEL receptor."
    Lewis M.J., Pelham H.R.B.
    Nature 348:162-163(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Placenta.
  2. "Large-scale identification and characterization of human genes that activate NF-kappaB and MAPK signaling pathways."
    Matsuda A., Suzuki Y., Honda G., Muramatsu S., Matsuzaki O., Nagano Y., Doi T., Shimotohno K., Harada T., Nishida E., Hayashi H., Sugano S.
    Oncogene 22:3307-3318(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Lung fibroblast.
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
    Tissue: Placenta.
  4. "The DNA sequence and biology of human chromosome 19."
    Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., Lamerdin J.E., Hellsten U., Goodstein D., Couronne O., Tran-Gyamfi M., Aerts A., Altherr M., Ashworth L., Bajorek E., Black S., Branscomb E., Caenepeel S., Carrano A.V.
    , Caoile C., Chan Y.M., Christensen M., Cleland C.A., Copeland A., Dalin E., Dehal P., Denys M., Detter J.C., Escobar J., Flowers D., Fotopulos D., Garcia C., Georgescu A.M., Glavina T., Gomez M., Gonzales E., Groza M., Hammon N., Hawkins T., Haydu L., Ho I., Huang W., Israni S., Jett J., Kadner K., Kimball H., Kobayashi A., Larionov V., Leem S.-H., Lopez F., Lou Y., Lowry S., Malfatti S., Martinez D., McCready P.M., Medina C., Morgan J., Nelson K., Nolan M., Ovcharenko I., Pitluck S., Pollard M., Popkie A.P., Predki P., Quan G., Ramirez L., Rash S., Retterer J., Rodriguez A., Rogers S., Salamov A., Salazar A., She X., Smith D., Slezak T., Solovyev V., Thayer N., Tice H., Tsai M., Ustaszewska A., Vo N., Wagner M., Wheeler J., Wu K., Xie G., Yang J., Dubchak I., Furey T.S., DeJong P., Dickson M., Gordon D., Eichler E.E., Pennacchio L.A., Richardson P., Stubbs L., Rokhsar D.S., Myers R.M., Rubin E.M., Lucas S.M.
    Nature 428:529-535(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Skin.
  7. "Mutational analysis of the human KDEL receptor: distinct structural requirements for Golgi retention, ligand binding and retrograde transport."
    Townsley F.M., Wilson D.W., Pelham H.R.B.
    EMBO J. 12:2821-2829(1993) [PubMed] [Europe PMC] [Abstract]
    Cited for: MUTAGENESIS.
  8. "KDEL-cargo regulates interactions between proteins involved in COPI vesicle traffic: measurements in living cells using FRET."
    Majoul I., Straub M., Hell S.W., Duden R., Soling H.D.
    Dev. Cell 1:139-153(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBUNIT.
  9. "The retrieval function of the KDEL receptor requires PKA phosphorylation of its C-terminus."
    Cabrera M., Muniz M., Hidalgo J., Vega L., Martin M.E., Velasco A.
    Mol. Biol. Cell 14:4114-4125(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION AT SER-209, MUTAGENESIS OF SER-209, SUBCELLULAR LOCATION.
  10. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiERD21_HUMAN
AccessioniPrimary (citable) accession number: P24390
Secondary accession number(s): B2R6N4, Q54A39, Q8NBW7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 1, 1992
Last sequence update: March 1, 1992
Last modified: June 8, 2016
This is version 138 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.