UniProtKB - Q9BT09 (CNPY3_HUMAN)
Protein canopy homolog 3
CNPY3
Functioni
Toll-like receptor (TLR)-specific co-chaperone for HSP90B1. Required for proper TLR folding, except that of TLR3, and hence controls TLR exit from the endoplasmic reticulum. Consequently, required for both innate and adaptive immune responses (By similarity).
By similarityGO - Molecular functioni
- signaling receptor binding Source: GO_Central
GO - Biological processi
- innate immune response Source: UniProtKB-KW
Keywordsi
Molecular function | Chaperone |
Biological process | Immunity, Innate immunity |
Enzyme and pathway databases
PathwayCommonsi | Q9BT09 |
Reactomei | R-HSA-1679131, Trafficking and processing of endosomal TLR |
SignaLinki | Q9BT09 |
Names & Taxonomyi
Protein namesi | Recommended name: Protein canopy homolog 3Alternative name(s): CTG repeat protein 4a Expanded repeat-domain protein CAG/CTG 5 Protein associated with TLR4 Trinucleotide repeat-containing gene 5 protein |
Gene namesi | Name:CNPY3 Synonyms:CTG4A, ERDA5, PRAT4A, TNRC5 ORF Names:HSPC084, UNQ1934/PRO4409 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:11968, CNPY3 |
MIMi | 610774, gene |
neXtProti | NX_Q9BT09 |
VEuPathDBi | HostDB:ENSG00000137161 |
Subcellular locationi
Endoplasmic reticulum
- Endoplasmic reticulum By similarity
Endoplasmic reticulum
- endoplasmic reticulum lumen Source: Reactome
Keywords - Cellular componenti
Endoplasmic reticulumPathology & Biotechi
Involvement in diseasei
Developmental and epileptic encephalopathy 60 (DEE60)1 Publication
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_080491 | 125 | G → R in DEE60; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs1554292759EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variant, EpilepsyOrganism-specific databases
DisGeNETi | 10695 |
MalaCardsi | CNPY3 |
MIMi | 617929, phenotype |
OpenTargetsi | ENSG00000137161 |
Orphaneti | 3451, Infantile spasms syndrome |
PharmGKBi | PA162382601 |
Miscellaneous databases
Pharosi | Q9BT09, Tbio |
Genetic variation databases
BioMutai | CNPY3 |
DMDMi | 74752319 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 30 | Sequence analysisAdd BLAST | 30 | |
ChainiPRO_0000313780 | 31 – 278 | Protein canopy homolog 3Add BLAST | 248 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Disulfide bondi | 49 ↔ 206 | By similarity | ||
Disulfide bondi | 52 ↔ 194 | By similarity | ||
Disulfide bondi | 104 ↔ 166 | By similarity | ||
Glycosylationi | 153 | N-linked (GlcNAc...) asparagine1 Publication | 1 |
Keywords - PTMi
Disulfide bond, GlycoproteinProteomic databases
EPDi | Q9BT09 |
jPOSTi | Q9BT09 |
MassIVEi | Q9BT09 |
MaxQBi | Q9BT09 |
PaxDbi | Q9BT09 |
PeptideAtlasi | Q9BT09 |
PRIDEi | Q9BT09 |
ProteomicsDBi | 78939 [Q9BT09-1] 78940 [Q9BT09-2] |
PTM databases
GlyConnecti | 1651, 3 N-Linked glycans (1 site) |
GlyGeni | Q9BT09, 2 sites, 3 N-linked glycans (1 site), 1 O-linked glycan (1 site) |
iPTMneti | Q9BT09 |
PhosphoSitePlusi | Q9BT09 |
Expressioni
Gene expression databases
Bgeei | ENSG00000137161, Expressed in monocyte and 237 other tissues |
ExpressionAtlasi | Q9BT09, baseline and differential |
Genevisiblei | Q9BT09, HS |
Organism-specific databases
HPAi | ENSG00000137161, Low tissue specificity |
Interactioni
Subunit structurei
Interacts with HSP90B1; this interaction is disrupted in the presence of ATP.
Interacts with TLR1, TLR2, TLR4 and TLR9 (By similarity). Strongest interaction with TLR4 (By similarity).
By similarityBinary interactionsi
Q9BT09
GO - Molecular functioni
- signaling receptor binding Source: GO_Central
Protein-protein interaction databases
BioGRIDi | 115934, 118 interactors |
IntActi | Q9BT09, 41 interactors |
MINTi | Q9BT09 |
STRINGi | 9606.ENSP00000361926 |
Miscellaneous databases
RNActi | Q9BT09, protein |
Structurei
3D structure databases
AlphaFoldDBi | Q9BT09 |
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 47 – 271 | Saposin B-typeAdd BLAST | 225 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 215 – 278 | DisorderedSequence analysisAdd BLAST | 64 |
Coiled coil
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Coiled coili | 153 – 179 | Sequence analysisAdd BLAST | 27 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 246 – 266 | Basic and acidic residuesSequence analysisAdd BLAST | 21 |
Sequence similaritiesi
Keywords - Domaini
Coiled coil, SignalPhylogenomic databases
eggNOGi | KOG4052, Eukaryota |
GeneTreei | ENSGT00390000014072 |
HOGENOMi | CLU_078068_0_0_1 |
InParanoidi | Q9BT09 |
OMAi | DKACLDE |
PhylomeDBi | Q9BT09 |
TreeFami | TF318951 |
Family and domain databases
InterProi | View protein in InterPro IPR021852, DUF3456 |
Pfami | View protein in Pfam PF11938, DUF3456, 1 hit |
s (2)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MDSMPEPASR CLLLLPLLLL LLLLLPAPEL GPSQAGAEEN DWVRLPSKCE
60 70 80 90 100
VCKYVAVELK SAFEETGKTK EVIGTGYGIL DQKASGVKYT KSDLRLIEVT
110 120 130 140 150
ETICKRLLDY SLHKERTGSN RFAKGMSETF ETLHNLVHKG VKVVMDIPYE
160 170 180 190 200
LWNETSAEVA DLKKQCDVLV EEFEEVIEDW YRNHQEEDLT EFLCANHVLK
210 220 230 240 250
GKDTSCLAEQ WSGKKGDTAA LGGKKSKKKS SRAKAAGGRS SSSKQRKELG
260 270
GLEGDPSPEE DEGIQKASPL THSPPDEL
The sequence of this isoform differs from the canonical sequence as follows:
51-54: VCKY → GTCG
55-278: Missing.
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 272 | H → P in BAC03406 (Ref. 5) Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_080491 | 125 | G → R in DEE60; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs1554292759EnsemblClinVar. | 1 | |
Natural variantiVAR_037731 | 145 | M → I. Corresponds to variant dbSNP:rs1063252Ensembl. | 1 | |
Natural variantiVAR_037732 | 231 | S → I. Corresponds to variant dbSNP:rs9471969Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_030132 | 51 – 54 | VCKY → GTCG in isoform 2. 1 Publication | 4 | |
Alternative sequenceiVSP_030133 | 55 – 278 | Missing in isoform 2. 1 PublicationAdd BLAST | 224 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AY358960 mRNA Translation: AAQ89319.1 AL035587 Genomic DNA No translation available. BC004423 mRNA Translation: AAH04423.1 BC008133 mRNA No translation available. BC008898 mRNA Translation: AAH08898.1 BC008961 mRNA Translation: AAH08961.1 BC022093 mRNA Translation: AAH22093.3 U80744 mRNA Translation: AAB91442.1 Frameshift. AF161347 mRNA Translation: AAF28907.1 Frameshift. AK090425 mRNA Translation: BAC03406.1 |
CCDSi | CCDS4875.1 [Q9BT09-1] |
RefSeqi | NP_006577.2, NM_006586.4 [Q9BT09-1] |
Genome annotation databases
Ensembli | ENST00000372836.5; ENSP00000361926.4; ENSG00000137161.18 |
GeneIDi | 10695 |
KEGGi | hsa:10695 |
MANE-Selecti | ENST00000372836.5; ENSP00000361926.4; NM_006586.5; NP_006577.2 |
UCSCi | uc003ota.5, human [Q9BT09-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AY358960 mRNA Translation: AAQ89319.1 AL035587 Genomic DNA No translation available. BC004423 mRNA Translation: AAH04423.1 BC008133 mRNA No translation available. BC008898 mRNA Translation: AAH08898.1 BC008961 mRNA Translation: AAH08961.1 BC022093 mRNA Translation: AAH22093.3 U80744 mRNA Translation: AAB91442.1 Frameshift. AF161347 mRNA Translation: AAF28907.1 Frameshift. AK090425 mRNA Translation: BAC03406.1 |
CCDSi | CCDS4875.1 [Q9BT09-1] |
RefSeqi | NP_006577.2, NM_006586.4 [Q9BT09-1] |
3D structure databases
AlphaFoldDBi | Q9BT09 |
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Protein-protein interaction databases
BioGRIDi | 115934, 118 interactors |
IntActi | Q9BT09, 41 interactors |
MINTi | Q9BT09 |
STRINGi | 9606.ENSP00000361926 |
PTM databases
GlyConnecti | 1651, 3 N-Linked glycans (1 site) |
GlyGeni | Q9BT09, 2 sites, 3 N-linked glycans (1 site), 1 O-linked glycan (1 site) |
iPTMneti | Q9BT09 |
PhosphoSitePlusi | Q9BT09 |
Genetic variation databases
BioMutai | CNPY3 |
DMDMi | 74752319 |
Proteomic databases
EPDi | Q9BT09 |
jPOSTi | Q9BT09 |
MassIVEi | Q9BT09 |
MaxQBi | Q9BT09 |
PaxDbi | Q9BT09 |
PeptideAtlasi | Q9BT09 |
PRIDEi | Q9BT09 |
ProteomicsDBi | 78939 [Q9BT09-1] 78940 [Q9BT09-2] |
Protocols and materials databases
Antibodypediai | 16108, 181 antibodies from 30 providers |
DNASUi | 10695 |
Genome annotation databases
Ensembli | ENST00000372836.5; ENSP00000361926.4; ENSG00000137161.18 |
GeneIDi | 10695 |
KEGGi | hsa:10695 |
MANE-Selecti | ENST00000372836.5; ENSP00000361926.4; NM_006586.5; NP_006577.2 |
UCSCi | uc003ota.5, human [Q9BT09-1] |
Organism-specific databases
CTDi | 10695 |
DisGeNETi | 10695 |
GeneCardsi | CNPY3 |
HGNCi | HGNC:11968, CNPY3 |
HPAi | ENSG00000137161, Low tissue specificity |
MalaCardsi | CNPY3 |
MIMi | 610774, gene 617929, phenotype |
neXtProti | NX_Q9BT09 |
OpenTargetsi | ENSG00000137161 |
Orphaneti | 3451, Infantile spasms syndrome |
PharmGKBi | PA162382601 |
VEuPathDBi | HostDB:ENSG00000137161 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG4052, Eukaryota |
GeneTreei | ENSGT00390000014072 |
HOGENOMi | CLU_078068_0_0_1 |
InParanoidi | Q9BT09 |
OMAi | DKACLDE |
PhylomeDBi | Q9BT09 |
TreeFami | TF318951 |
Enzyme and pathway databases
PathwayCommonsi | Q9BT09 |
Reactomei | R-HSA-1679131, Trafficking and processing of endosomal TLR |
SignaLinki | Q9BT09 |
Miscellaneous databases
BioGRID-ORCSi | 10695, 6 hits in 1076 CRISPR screens |
ChiTaRSi | CNPY3, human |
GenomeRNAii | 10695 |
Pharosi | Q9BT09, Tbio |
PROi | PR:Q9BT09 |
RNActi | Q9BT09, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000137161, Expressed in monocyte and 237 other tissues |
ExpressionAtlasi | Q9BT09, baseline and differential |
Genevisiblei | Q9BT09, HS |
Family and domain databases
InterProi | View protein in InterPro IPR021852, DUF3456 |
Pfami | View protein in Pfam PF11938, DUF3456, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | CNPY3_HUMAN | |
Accessioni | Q9BT09Primary (citable) accession number: Q9BT09 Secondary accession number(s): O15412 Q9P0F2 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 15, 2008 |
Last sequence update: | June 1, 2001 | |
Last modified: | May 25, 2022 | |
This is version 152 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Reference proteomeDocuments
- Human chromosome 6
Human chromosome 6: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families