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Protein

Protein canopy homolog 3

Gene

CNPY3

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

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 similarity

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionChaperone
Biological processImmunity, Innate immunity

Enzyme and pathway databases

ReactomeiR-HSA-1679131 Trafficking and processing of endosomal TLR

Names & Taxonomyi

Protein namesi
Recommended name:
Protein canopy homolog 3
Alternative 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
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

EuPathDBiHostDB:ENSG00000137161.16
HGNCiHGNC:11968 CNPY3
MIMi610774 gene
neXtProtiNX_Q9BT09

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

Epileptic encephalopathy, early infantile, 60 (EIEE60)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of epileptic encephalopathy, a heterogeneous group of severe childhood onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. EIEE60 is an autosomal recessive condition characterized by onset of seizures in the first months of life.
See also OMIM:617929
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_080491125G → R in EIEE60; unknown pathological significance. 1 Publication1

Keywords - Diseasei

Disease mutation, Epilepsy

Organism-specific databases

DisGeNETi10695
MIMi617929 phenotype
OpenTargetsiENSG00000137161
PharmGKBiPA162382601

Polymorphism and mutation databases

BioMutaiCNPY3
DMDMi74752319

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 30Sequence analysisAdd BLAST30
ChainiPRO_000031378031 – 278Protein canopy homolog 3Add BLAST248

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi49 ↔ 206By similarity
Disulfide bondi52 ↔ 194By similarity
Disulfide bondi104 ↔ 166By similarity
Glycosylationi153N-linked (GlcNAc...) asparagine1 Publication1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiQ9BT09
MaxQBiQ9BT09
PaxDbiQ9BT09
PeptideAtlasiQ9BT09
PRIDEiQ9BT09
ProteomicsDBi78939
78940 [Q9BT09-2]

PTM databases

iPTMnetiQ9BT09
PhosphoSitePlusiQ9BT09

Expressioni

Gene expression databases

BgeeiENSG00000137161
CleanExiHS_CNPY3
ExpressionAtlasiQ9BT09 baseline and differential
GenevisibleiQ9BT09 HS

Organism-specific databases

HPAiHPA016560

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 similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi115934, 32 interactors
IntActiQ9BT09, 7 interactors
MINTiQ9BT09
STRINGi9606.ENSP00000361926

Structurei

3D structure databases

ProteinModelPortaliQ9BT09
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini47 – 271Saposin B-typeAdd BLAST225

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili153 – 179Sequence analysisAdd BLAST27

Sequence similaritiesi

Belongs to the canopy family.Curated

Keywords - Domaini

Coiled coil, Signal

Phylogenomic databases

eggNOGiKOG4052 Eukaryota
ENOG4111GVI LUCA
GeneTreeiENSGT00390000014072
HOGENOMiHOG000030912
HOVERGENiHBG107736
InParanoidiQ9BT09
OMAiKGKDTSC
OrthoDBiEOG091G0QAJ
PhylomeDBiQ9BT09
TreeFamiTF318951

Family and domain databases

InterProiView protein in InterPro
IPR021852 DUF3456
PfamiView protein in Pfam
PF11938 DUF3456, 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

Isoform 1 (identifier: Q9BT09-1) [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
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
Length:278
Mass (Da):30,748
Last modified:June 1, 2001 - v1
Checksum:i07587320D316E0CE
GO
Isoform 2 (identifier: Q9BT09-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     51-54: VCKY → GTCG
     55-278: Missing.

Note: May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Show »
Length:54
Mass (Da):5,773
Checksum:i6345B76C85D1DD60
GO

Sequence cautioni

The sequence AAB91442 differs from that shown. Reason: Frameshift at position 109.Curated
The sequence AAF28907 differs from that shown. Reason: Frameshift at position 252.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti272H → P in BAC03406 (Ref. 5) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_080491125G → R in EIEE60; unknown pathological significance. 1 Publication1
Natural variantiVAR_037731145M → I. Corresponds to variant dbSNP:rs1063252Ensembl.1
Natural variantiVAR_037732231S → I. Corresponds to variant dbSNP:rs9471969Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_03013251 – 54VCKY → GTCG in isoform 2. 1 Publication4
Alternative sequenceiVSP_03013355 – 278Missing in isoform 2. 1 PublicationAdd BLAST224

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
CCDSiCCDS4875.1 [Q9BT09-1]
RefSeqiNP_006577.2, NM_006586.4 [Q9BT09-1]
UniGeneiHs.414099

Genome annotation databases

EnsembliENST00000372836; ENSP00000361926; ENSG00000137161 [Q9BT09-1]
GeneIDi10695
KEGGihsa:10695
UCSCiuc003ota.5 human [Q9BT09-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiCNPY3_HUMAN
AccessioniPrimary (citable) accession number: Q9BT09
Secondary accession number(s): O15412
, Q0P6I2, Q8NF54, Q8WTU8, Q9P0F2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 15, 2008
Last sequence update: June 1, 2001
Last modified: July 18, 2018
This is version 130 of the entry and version 1 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, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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