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Protein

Glucosidase 2 subunit beta

Gene

PRKCSH

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

Functioni

Regulatory subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc2Man9GlcNAc2 oligosaccharide precursor of immature glycoproteins (PubMed:10929008). Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia (By similarity).By similarity1 Publication

Pathwayi: N-glycan metabolism

This protein is involved in the pathway N-glycan metabolism, which is part of Glycan metabolism.1 Publication
View all proteins of this organism that are known to be involved in the pathway N-glycan metabolism and in Glycan metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei49Substrate; shared with catalytic subunitBy similarity1
Metal bindingi50Calcium 1; via carbonyl oxygenBy similarity1
Metal bindingi53Calcium 1By similarity1
Binding sitei53Substrate; shared with catalytic subunitBy similarity1
Metal bindingi55Calcium 1; via carbonyl oxygenBy similarity1
Metal bindingi57Calcium 1By similarity1
Metal bindingi63Calcium 1By similarity1
Metal bindingi64Calcium 1By similarity1
Metal bindingi91Calcium 2; via carbonyl oxygenBy similarity1
Metal bindingi94Calcium 2By similarity1
Metal bindingi96Calcium 2; via carbonyl oxygenBy similarity1
Metal bindingi98Calcium 2By similarity1
Metal bindingi104Calcium 2By similarity1
Metal bindingi105Calcium 2By similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Calcium bindingi213 – 2361PROSITE-ProRule annotationAdd BLAST24

GO - Molecular functioni

  • calcium ion binding Source: InterPro
  • ion channel binding Source: UniProtKB
  • phosphoprotein binding Source: UniProtKB
  • protein kinase C binding Source: UniProtKB

GO - Biological processi

  • cellular protein metabolic process Source: Reactome
  • intracellular signal transduction Source: UniProtKB
  • N-glycan processing Source: UniProtKB
  • post-translational protein modification Source: Reactome

Keywordsi

LigandCalcium, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS05346-MONOMER
BRENDAi3.2.1.84 2681
ReactomeiR-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle
R-HSA-879415 Advanced glycosylation endproduct receptor signaling
R-HSA-8957275 Post-translational protein phosphorylation
R-HSA-901042 Calnexin/calreticulin cycle
SignaLinkiP14314
UniPathwayi
UPA00957

Names & Taxonomyi

Protein namesi
Recommended name:
Glucosidase 2 subunit beta
Alternative name(s):
80K-H protein1 Publication
Glucosidase II subunit beta
Protein kinase C substrate 60.1 kDa protein heavy chain
Short name:
PKCSH
Gene namesi
Name:PRKCSH1 PublicationImported
Synonyms:G19P1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000130175.9
HGNCiHGNC:9411 PRKCSH
MIMi177060 gene
neXtProtiNX_P14314

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Involvement in diseasei

Polycystic liver disease 1 with or without kidney cysts (PCLD1)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts. A subset of patients may develop kidney cysts that usually do not result in clinically significant renal disease.
See also OMIM:174050
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_08093918 – 528Missing in PCLD1. 1 PublicationAdd BLAST511
Natural variantiVAR_080940156 – 528Missing in PCLD1. 1 PublicationAdd BLAST373
Natural variantiVAR_080941198 – 528Missing in PCLD1. 1 PublicationAdd BLAST331
Natural variantiVAR_080942414 – 528Missing in PCLD1. 1 PublicationAdd BLAST115
Natural variantiVAR_080943423 – 528Missing in PCLD1. 1 PublicationAdd BLAST106

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi5589
MalaCardsiPRKCSH
MIMi174050 phenotype
OpenTargetsiENSG00000130175
Orphaneti2924 Isolated polycystic liver disease
PharmGKBiPA33774

Polymorphism and mutation databases

BioMutaiPRKCSH
DMDMi116242499

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 141 PublicationAdd BLAST14
ChainiPRO_000000414315 – 528Glucosidase 2 subunit betaAdd BLAST514

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei24Phosphoserine; by FAM20C1 Publication1
Disulfide bondi39 ↔ 58By similarity
Disulfide bondi56 ↔ 70By similarity
Glycosylationi72N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi77 ↔ 99By similarity
Modified residuei89Phosphoserine; by PKCSequence analysis1
Disulfide bondi97 ↔ 112By similarity
Disulfide bondi100 ↔ 116By similarity
Modified residuei166N6-succinyllysineBy similarity1
Modified residuei168Phosphoserine; by FAM20C1 Publication1
Modified residuei383Phosphoserine; by PKCSequence analysis1
Modified residuei390Phosphoserine; by PKCSequence analysis1
Modified residuei434Phosphoserine; by PKCSequence analysis1
Glycosylationi476N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

EPDiP14314
MaxQBiP14314
PaxDbiP14314
PeptideAtlasiP14314
PRIDEiP14314
ProteomicsDBi53041
53042 [P14314-2]
TopDownProteomicsiP14314-1 [P14314-1]
P14314-2 [P14314-2]

PTM databases

GlyConnecti1269
iPTMnetiP14314
PhosphoSitePlusiP14314
SwissPalmiP14314

Miscellaneous databases

PMAP-CutDBiP14314

Expressioni

Gene expression databases

BgeeiENSG00000130175 Expressed in 237 organ(s), highest expression level in spinal cord
CleanExiHS_PRKCSH
ExpressionAtlasiP14314 baseline and differential
GenevisibleiP14314 HS

Organism-specific databases

HPAiCAB004465
HPA041940
HPA043560

Interactioni

Subunit structurei

Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH) (PubMed:10929008). Binds glycosylated PTPRC (By similarity).By similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

BioGridi111575, 64 interactors
CORUMiP14314
IntActiP14314, 25 interactors
MINTiP14314
STRINGi9606.ENSP00000252455

Structurei

3D structure databases

ProteinModelPortaliP14314
SMRiP14314
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini37 – 71LDL-receptor class A 1PROSITE-ProRule annotationAdd BLAST35
Domaini72 – 113LDL-receptor class A 2PROSITE-ProRule annotationAdd BLAST42
Domaini209 – 244EF-hand 1PROSITE-ProRule annotationAdd BLAST36
Domaini245 – 290EF-hand 2PROSITE-ProRule annotationAdd BLAST46
Domaini413 – 468PRKCSHAdd BLAST56

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi525 – 528Prevents secretion from ERPROSITE-ProRule annotation1 Publication4

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi313 – 336Glu-rich (acidic)Add BLAST24

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

eggNOGiKOG2397 Eukaryota
ENOG410XPE3 LUCA
GeneTreeiENSGT00510000047770
HOGENOMiHOG000007805
HOVERGENiHBG051738
InParanoidiP14314
KOiK08288
PhylomeDBiP14314
TreeFamiTF329550

Family and domain databases

CDDicd00112 LDLa, 1 hit
Gene3Di2.70.130.10, 1 hit
InterProiView protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR026874 Glucosidase_2_bsu
IPR039794 Gtb1-like
IPR036055 LDL_receptor-like_sf
IPR002172 LDrepeatLR_classA_rpt
IPR009011 Man6P_isomerase_rcpt-bd_dom_sf
IPR036607 PRKCSH
IPR028146 PRKCSH_N
PANTHERiPTHR12630 PTHR12630, 1 hit
PTHR12630:SF1 PTHR12630:SF1, 1 hit
PfamiView protein in Pfam
PF13202 EF-hand_5, 1 hit
PF12999 PRKCSH-like, 1 hit
PF13015 PRKCSH_1, 1 hit
SUPFAMiSSF47473 SSF47473, 1 hit
SSF57424 SSF57424, 1 hit
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 1 hit
PS50222 EF_HAND_2, 1 hit
PS00014 ER_TARGET, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

This entry has 2 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P14314-1) [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
MLLPLLLLLP MCWAVEVKRP RGVSLTNHHF YDESKPFTCL DGSATIPFDQ
60 70 80 90 100
VNDDYCDCKD GSDEPGTAAC PNGSFHCTNT GYKPLYIPSN RVNDGVCDCC
110 120 130 140 150
DGTDEYNSGV ICENTCKEKG RKERESLQQM AEVTREGFRL KKILIEDWKK
160 170 180 190 200
AREEKQKKLI ELQAGKKSLE DQVEMLRTVK EEAEKPEREA KEQHQKLWEE
210 220 230 240 250
QLAAAKAQQE QELAADAFKE LDDDMDGTVS VTELQTHPEL DTDGDGALSE
260 270 280 290 300
AEAQALLSGD TQTDATSFYD RVWAAIRDKY RSEALPTDLP APSAPDLTEP
310 320 330 340 350
KEEQPPVPSS PTEEEEEEEE EEEEEAEEEE EEEDSEEAPP PLSPPQPASP
360 370 380 390 400
AEEDKMPPYD EQTQAFIDAA QEARNKFEEA ERSLKDMEES IRNLEQEISF
410 420 430 440 450
DFGPNGEFAY LYSQCYELTT NEYVYRLCPF KLVSQKPKLG GSPTSLGTWG
460 470 480 490 500
SWIGPDHDKF SAMKYEQGTG CWQGPNRSTT VRLLCGKETM VTSTTEPSRC
510 520
EYLMELMTPA ACPEPPPEAP TEDDHDEL
Length:528
Mass (Da):59,425
Last modified:October 17, 2006 - v2
Checksum:i8DAD9776037E878E
GO
Isoform 2 (identifier: P14314-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     337-346: EAPPPLSPPQ → VQGEQPK

Show »
Length:525
Mass (Da):59,178
Checksum:iABACF156C534F30E
GO

Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
K7ELL7K7ELL7_HUMAN
Glucosidase 2 subunit beta
PRKCSH
535Annotation score:
A0A0C4DGP4A0A0C4DGP4_HUMAN
Glucosidase 2 subunit beta
PRKCSH
200Annotation score:
K7EPW7K7EPW7_HUMAN
Glucosidase 2 subunit beta
PRKCSH
168Annotation score:
K7EJ70K7EJ70_HUMAN
Glucosidase 2 subunit beta
PRKCSH
144Annotation score:
K7EKX1K7EKX1_HUMAN
Glucosidase 2 subunit beta
PRKCSH
140Annotation score:
K7EL27K7EL27_HUMAN
Glucosidase 2 subunit beta
PRKCSH
132Annotation score:
K7EIP3K7EIP3_HUMAN
Glucosidase 2 subunit beta
PRKCSH
62Annotation score:

Sequence cautioni

The sequence AAH15154 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti325Missing in AAA52493 (PubMed:2793184).Curated1
Sequence conflicti325Missing in AAA98668 (PubMed:9043864).Curated1
Sequence conflicti325Missing in AAP88860 (Ref. 4) Curated1
Sequence conflicti325Missing in AAH13586 (PubMed:15057824).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_08093918 – 528Missing in PCLD1. 1 PublicationAdd BLAST511
Natural variantiVAR_02876174S → N. Corresponds to variant dbSNP:rs10406672Ensembl.1
Natural variantiVAR_080940156 – 528Missing in PCLD1. 1 PublicationAdd BLAST373
Natural variantiVAR_080941198 – 528Missing in PCLD1. 1 PublicationAdd BLAST331
Natural variantiVAR_028762291A → T2 PublicationsCorresponds to variant dbSNP:rs11557488EnsemblClinVar.1
Natural variantiVAR_048658338A → G. Corresponds to variant dbSNP:rs35847588Ensembl.1
Natural variantiVAR_080942414 – 528Missing in PCLD1. 1 PublicationAdd BLAST115
Natural variantiVAR_080943423 – 528Missing in PCLD1. 1 PublicationAdd BLAST106

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_043749337 – 346EAPPPLSPPQ → VQGEQPK in isoform 2. 2 Publications10

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03075 mRNA Translation: AAA52493.1
U50326
, U50317, U50318, U50319, U50320, U50321, U50322, U50323, U50324, U50325 Genomic DNA Translation: AAA98668.1
AF144075 mRNA Translation: AAF66686.1
BT009858 mRNA Translation: AAP88860.1
AK290433 mRNA Translation: BAF83122.1
AC008481 Genomic DNA No translation available.
AC024575 Genomic DNA No translation available.
BC013586 mRNA Translation: AAH13586.2
BC015154 mRNA Translation: AAH15154.1 Different initiation.
CCDSiCCDS32911.1 [P14314-1]
CCDS45977.1 [P14314-2]
PIRiA32469
RefSeqiNP_001001329.1, NM_001001329.2 [P14314-2]
NP_001276031.1, NM_001289102.1 [P14314-2]
NP_002734.2, NM_002743.3 [P14314-1]
XP_011526433.1, XM_011528131.1 [P14314-1]
XP_011526434.1, XM_011528132.1 [P14314-2]
XP_016882466.1, XM_017026977.1 [P14314-1]
UniGeneiHs.610830

Genome annotation databases

EnsembliENST00000587327; ENSP00000466012; ENSG00000130175 [P14314-2]
ENST00000589838; ENSP00000465461; ENSG00000130175 [P14314-1]
ENST00000591462; ENSP00000465489; ENSG00000130175 [P14314-2]
GeneIDi5589
KEGGihsa:5589
UCSCiuc010dyb.5 human [P14314-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03075 mRNA Translation: AAA52493.1
U50326
, U50317, U50318, U50319, U50320, U50321, U50322, U50323, U50324, U50325 Genomic DNA Translation: AAA98668.1
AF144075 mRNA Translation: AAF66686.1
BT009858 mRNA Translation: AAP88860.1
AK290433 mRNA Translation: BAF83122.1
AC008481 Genomic DNA No translation available.
AC024575 Genomic DNA No translation available.
BC013586 mRNA Translation: AAH13586.2
BC015154 mRNA Translation: AAH15154.1 Different initiation.
CCDSiCCDS32911.1 [P14314-1]
CCDS45977.1 [P14314-2]
PIRiA32469
RefSeqiNP_001001329.1, NM_001001329.2 [P14314-2]
NP_001276031.1, NM_001289102.1 [P14314-2]
NP_002734.2, NM_002743.3 [P14314-1]
XP_011526433.1, XM_011528131.1 [P14314-1]
XP_011526434.1, XM_011528132.1 [P14314-2]
XP_016882466.1, XM_017026977.1 [P14314-1]
UniGeneiHs.610830

3D structure databases

ProteinModelPortaliP14314
SMRiP14314
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111575, 64 interactors
CORUMiP14314
IntActiP14314, 25 interactors
MINTiP14314
STRINGi9606.ENSP00000252455

PTM databases

GlyConnecti1269
iPTMnetiP14314
PhosphoSitePlusiP14314
SwissPalmiP14314

Polymorphism and mutation databases

BioMutaiPRKCSH
DMDMi116242499

Proteomic databases

EPDiP14314
MaxQBiP14314
PaxDbiP14314
PeptideAtlasiP14314
PRIDEiP14314
ProteomicsDBi53041
53042 [P14314-2]
TopDownProteomicsiP14314-1 [P14314-1]
P14314-2 [P14314-2]

Protocols and materials databases

DNASUi5589
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000587327; ENSP00000466012; ENSG00000130175 [P14314-2]
ENST00000589838; ENSP00000465461; ENSG00000130175 [P14314-1]
ENST00000591462; ENSP00000465489; ENSG00000130175 [P14314-2]
GeneIDi5589
KEGGihsa:5589
UCSCiuc010dyb.5 human [P14314-1]

Organism-specific databases

CTDi5589
DisGeNETi5589
EuPathDBiHostDB:ENSG00000130175.9
GeneCardsiPRKCSH
HGNCiHGNC:9411 PRKCSH
HPAiCAB004465
HPA041940
HPA043560
MalaCardsiPRKCSH
MIMi174050 phenotype
177060 gene
neXtProtiNX_P14314
OpenTargetsiENSG00000130175
Orphaneti2924 Isolated polycystic liver disease
PharmGKBiPA33774
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2397 Eukaryota
ENOG410XPE3 LUCA
GeneTreeiENSGT00510000047770
HOGENOMiHOG000007805
HOVERGENiHBG051738
InParanoidiP14314
KOiK08288
PhylomeDBiP14314
TreeFamiTF329550

Enzyme and pathway databases

UniPathwayi
UPA00957

BioCyciMetaCyc:HS05346-MONOMER
BRENDAi3.2.1.84 2681
ReactomeiR-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle
R-HSA-879415 Advanced glycosylation endproduct receptor signaling
R-HSA-8957275 Post-translational protein phosphorylation
R-HSA-901042 Calnexin/calreticulin cycle
SignaLinkiP14314

Miscellaneous databases

ChiTaRSiPRKCSH human
GeneWikiiPRKCSH
GenomeRNAii5589
PMAP-CutDBiP14314
PROiPR:P14314
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000130175 Expressed in 237 organ(s), highest expression level in spinal cord
CleanExiHS_PRKCSH
ExpressionAtlasiP14314 baseline and differential
GenevisibleiP14314 HS

Family and domain databases

CDDicd00112 LDLa, 1 hit
Gene3Di2.70.130.10, 1 hit
InterProiView protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR026874 Glucosidase_2_bsu
IPR039794 Gtb1-like
IPR036055 LDL_receptor-like_sf
IPR002172 LDrepeatLR_classA_rpt
IPR009011 Man6P_isomerase_rcpt-bd_dom_sf
IPR036607 PRKCSH
IPR028146 PRKCSH_N
PANTHERiPTHR12630 PTHR12630, 1 hit
PTHR12630:SF1 PTHR12630:SF1, 1 hit
PfamiView protein in Pfam
PF13202 EF-hand_5, 1 hit
PF12999 PRKCSH-like, 1 hit
PF13015 PRKCSH_1, 1 hit
SUPFAMiSSF47473 SSF47473, 1 hit
SSF57424 SSF57424, 1 hit
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 1 hit
PS50222 EF_HAND_2, 1 hit
PS00014 ER_TARGET, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiGLU2B_HUMAN
AccessioniPrimary (citable) accession number: P14314
Secondary accession number(s): A8K318
, Q96BU9, Q96D06, Q9P0W9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: October 17, 2006
Last modified: November 7, 2018
This is version 199 of the entry and version 2 of the sequence. See 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, Direct protein sequencing, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
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