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Protein

Complement component 1 Q subcomponent-binding protein, mitochondrial

Gene

C1QBP

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

Functioni

Is believed to be a multifunctional and multicompartmental protein involved in inflammation and infection processes, ribosome biogenesis, regulation of apoptosis, transcriptional regulation and pre-mRNA splicing. At the cell surface is thought to act as an endothelial receptor for plasma proteins of the complement and kallikrein-kinin cascades. Putative receptor for C1q; specifically binds to the globular "heads" of C1q thus inhibiting C1; may perform the receptor function through a complex with C1qR/CD93. In complex with cytokeratin-1/KRT1 is a high affinity receptor for kininogen-1/HMWK. Can also bind other plasma proteins, such as coagulation factor XII leading to its autoactivation. May function to bind initially fluid kininogen-1 to the cell membrane. The secreted form may enhance both extrinsic and intrinsic coagulation pathways. It is postulated that the cell surface form requires docking with transmembrane proteins for downstream signaling which might be specific for a cell-type or response. By acting as C1q receptor is involved in chemotaxis of immature dendritic cells and neutrophils and is proposed to signal through CD209/DC-SIGN on immature dendritic cells, through integrin alpha-4/beta-1 during trophoblast invasion of the decidua, and through integrin beta-1 during endothelial cell adhesion and spreading. Signaling involved in inhibition of innate immune response is implicating the PI3K-AKT/PKB pathway. In mitochondrial translation may be involved in formation of functional 55S mitoribosomes; the function seems to involve its RNA-binding activity. May be involved in the nucleolar ribosome maturation process; the function may involve the exchange of FBL for RRP1 in the association with pre-ribosome particles. Involved in regulation of RNA splicing by inhibiting the RNA-binding capacity of SRSF1 and its phosphorylation. Is required for the nuclear translocation of splicing factor U2AF1L4. Involved in regulation of CDKN2A- and HRK-mediated apoptosis. Stabilizes mitochondrial CDKN2A isoform smARF. May be involved in regulation of FOXC1 transcriptional activity and NFY/CCAAT-binding factor complex-mediated transcription. In infection processes acts as an attachment site for microbial proteins, including Listeria monocytogenes internalin B and Staphylococcus aureus protein A. May play a role in antibacterial defense as it can bind to cell surface hyaluronan and inhibit Streptococcus pneumoniae hyaluronate lyase. Involved in replication of Rubella virus. May be involved in modulation of the immune response; ligation by HCV core protein is resulting in suppresion of interleukin-12 production in monocyte-derived dendritic cells. Involved in regulation of antiviral response by inhibiting DDX58- and IFIH1-mediated signaling pathways probably involving its association with MAVS after viral infection. Involved in HIV-1 replication, presumably by contributing to splicing of viral RNA.21 Publications

GO - Molecular functioni

  • adrenergic receptor binding Source: UniProtKB
  • complement component C1q binding Source: UniProtKB
  • hyaluronic acid binding Source: UniProtKB
  • kininogen binding Source: UniProtKB
  • mitochondrial ribosome binding Source: UniProtKB
  • mRNA binding Source: UniProtKB
  • transcription corepressor activity Source: UniProtKB
  • transcription factor binding Source: UniProtKB

GO - Biological processi

  • apoptotic process Source: UniProtKB-KW
  • blood coagulation, intrinsic pathway Source: Reactome
  • complement activation, classical pathway Source: UniProtKB-KW
  • immune response Source: ProtInc
  • innate immune response Source: UniProtKB-KW
  • mature ribosome assembly Source: UniProtKB
  • mRNA processing Source: UniProtKB-KW
  • negative regulation of defense response to virus Source: UniProtKB
  • negative regulation of interferon-gamma production Source: UniProtKB
  • negative regulation of interleukin-12 production Source: UniProtKB
  • negative regulation of MDA-5 signaling pathway Source: UniProtKB
  • negative regulation of mRNA splicing, via spliceosome Source: UniProtKB
  • negative regulation of RIG-I signaling pathway Source: UniProtKB
  • negative regulation of transcription from RNA polymerase II promoter Source: UniProtKB
  • phosphatidylinositol 3-kinase signaling Source: UniProtKB
  • positive regulation of apoptotic process Source: UniProtKB
  • positive regulation of cell adhesion Source: UniProtKB
  • positive regulation of dendritic cell chemotaxis Source: UniProtKB
  • positive regulation of mitochondrial translation Source: UniProtKB
  • positive regulation of neutrophil chemotaxis Source: UniProtKB
  • positive regulation of protein kinase B signaling Source: UniProtKB
  • positive regulation of substrate adhesion-dependent cell spreading Source: UniProtKB
  • positive regulation of trophoblast cell migration Source: UniProtKB
  • regulation of complement activation Source: UniProtKB
  • RNA splicing Source: UniProtKB-KW
  • transcription, DNA-templated Source: UniProtKB-KW
  • viral process Source: UniProtKB-KW
Complete GO annotation...

Keywords - Biological processi

Adaptive immunity, Apoptosis, Complement pathway, Host-virus interaction, Immunity, Innate immunity, mRNA processing, mRNA splicing, Ribosome biogenesis, Transcription, Transcription regulation

Enzyme and pathway databases

BioCyciZFISH:ENSG00000108561-MONOMER.
ReactomeiR-HSA-140837. Intrinsic Pathway of Fibrin Clot Formation.

Protein family/group databases

TCDBi9.B.103.1.1. the putative ca(2+) uniporter (gc1qr) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Complement component 1 Q subcomponent-binding protein, mitochondrial
Alternative name(s):
ASF/SF2-associated protein p32
Glycoprotein gC1qBP
Short name:
C1qBP
Hyaluronan-binding protein 1
Mitochondrial matrix protein p32
gC1q-R protein
p33
Gene namesi
Name:C1QBP
Synonyms:GC1QBP, HABP1, SF2P32
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

HGNCiHGNC:1243. C1QBP.

Subcellular locationi

GO - Cellular componenti

  • cell surface Source: UniProtKB
  • cytoplasm Source: UniProtKB
  • cytosol Source: UniProtKB
  • extracellular space Source: Ensembl
  • membrane Source: UniProtKB
  • mitochondrial matrix Source: UniProtKB-SubCell
  • mitochondrion Source: UniProtKB
  • nucleolus Source: UniProtKB-SubCell
  • nucleus Source: UniProtKB
  • plasma membrane Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Cytoplasm, Membrane, Mitochondrion, Nucleus, Secreted

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi107G → D: Impairs HIV RNA splicing in mouse cells. 1 Publication1

Organism-specific databases

DisGeNETi708.
OpenTargetsiENSG00000108561.
PharmGKBiPA25624.

Chemistry databases

DrugBankiDB08818. Hyaluronic acid.

Polymorphism and mutation databases

BioMutaiC1QBP.
DMDMi730772.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 73MitochondrionCombined sources2 PublicationsAdd BLAST73
ChainiPRO_000001859074 – 282Complement component 1 Q subcomponent-binding protein, mitochondrialAdd BLAST209

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei87PhosphoserineCombined sources1
Modified residuei91N6-acetyllysineCombined sources1
Modified residuei188PhosphotyrosineCombined sources1
Modified residuei201PhosphoserineCombined sources1
Modified residuei205PhosphoserineCombined sources1
Modified residuei214PhosphothreonineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ07021.
MaxQBiQ07021.
PaxDbiQ07021.
PeptideAtlasiQ07021.
PRIDEiQ07021.
TopDownProteomicsiQ07021.

2D gel databases

DOSAC-COBS-2DPAGEQ07021.
OGPiQ07021.

PTM databases

iPTMnetiQ07021.
PhosphoSitePlusiQ07021.
SwissPalmiQ07021.

Miscellaneous databases

PMAP-CutDBQ07021.

Expressioni

Tissue specificityi

Expressed on cell surface of peripheral blood cells (at protein level); Surface expression is reported for macrophages and monocyte-derived dendritic cells.2 Publications

Inductioni

Enhanced cell surface expression upon platelet and monocyte activation.2 Publications

Gene expression databases

BgeeiENSG00000108561.
CleanExiHS_C1QBP.
ExpressionAtlasiQ07021. baseline and differential.
GenevisibleiQ07021. HS.

Organism-specific databases

HPAiHPA026483.

Interactioni

Subunit structurei

Homotrimer; three monomers form a donut-shaped structure with an unusually asymmetric charge distribution on the surface. Interacts with CDK13, HRK, VTN, NFYB, ADRA1B, FOXC1, DDX21, DDX50, NCL, SRSF1, SRSF9 and CDKN2A isoform smARF. Interacts with CD93; the association may represent a cell surface C1q receptor. Interacts with KRT1; the association represents a cell surface kininogen receptor. Interacts with CD209; the interaction is indicative for a C1q:C1QBP:CD209 signaling complex. Interacts with FBL and RRP1; the respective interactions with C1QBP are competetive. Probably associates with the mitoribosome. Interacts with MAVS; the interaction occurs upon viral transfection. Interacts with PPIF. Interacts with Rubella virus capsid protein; the interaction occurs in mitochondria. Interacts with Rubella virus protease p150, Staphylococcus aureus protein A/spa, HIV-1 Tat and HCV core protein. Interacts with U2AF1L4.By similarity19 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
P279584EBI-347528,EBI-6377335From a different organism.
CDK13Q14004-26EBI-347528,EBI-6375898
CDKN2AQ8N7263EBI-347528,EBI-625922
Cdkn2aQ643644EBI-347528,EBI-1202287From a different organism.
F12P007482EBI-347528,EBI-6378830
FOXC1Q129486EBI-347528,EBI-1175253
HRKO001987EBI-347528,EBI-701322
KNG1P010424EBI-347528,EBI-6378713
MAVSQ7Z4345EBI-347528,EBI-995373
PRKCZQ055132EBI-347528,EBI-295351
PRKD1Q151399EBI-347528,EBI-1181072
VTNP040048EBI-347528,EBI-1036653
YBX1P678096EBI-347528,EBI-354065

GO - Molecular functioni

  • adrenergic receptor binding Source: UniProtKB
  • complement component C1q binding Source: UniProtKB
  • kininogen binding Source: UniProtKB
  • transcription factor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi107169. 244 interactors.
DIPiDIP-31164N.
IntActiQ07021. 78 interactors.
MINTiMINT-246803.
STRINGi9606.ENSP00000225698.

Structurei

Secondary structure

1282
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi77 – 96Combined sources20
Turni106 – 108Combined sources3
Beta strandi110 – 114Combined sources5
Beta strandi117 – 124Combined sources8
Beta strandi127 – 134Combined sources8
Beta strandi168 – 174Combined sources7
Helixi175 – 177Combined sources3
Beta strandi180 – 187Combined sources8
Beta strandi205 – 213Combined sources9
Beta strandi225 – 228Combined sources4
Helixi233 – 244Combined sources12
Turni245 – 247Combined sources3
Helixi250 – 280Combined sources31

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1P32X-ray2.25A/B/C74-282[»]
3RPXX-ray2.65A/B/C96-282[»]
ProteinModelPortaliQ07021.
SMRiQ07021.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ07021.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni76 – 93C1q bindingAdd BLAST18
Regioni168 – 213Interaction with MAVS1 PublicationAdd BLAST46

Sequence similaritiesi

Belongs to the MAM33 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4024. Eukaryota.
ENOG4111G4Z. LUCA.
GeneTreeiENSGT00390000018406.
HOGENOMiHOG000046272.
HOVERGENiHBG000914.
InParanoidiQ07021.
KOiK15414.
OMAiCGCGGLH.
OrthoDBiEOG091G0IZD.
PhylomeDBiQ07021.
TreeFamiTF315160.

Family and domain databases

Gene3Di3.10.280.10. 1 hit.
InterProiIPR003428. MAM33.
[Graphical view]
PfamiPF02330. MAM33. 1 hit.
[Graphical view]
SUPFAMiSSF54529. SSF54529. 1 hit.

Sequencei

Sequence statusi: Complete.

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

Q07021-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLPLLRCVPR VLGSSVAGLR AAAPASPFRQ LLQPAPRLCT RPFGLLSVRA
60 70 80 90 100
GSERRPGLLR PRGPCACGCG CGSLHTDGDK AFVDFLSDEI KEERKIQKHK
110 120 130 140 150
TLPKMSGGWE LELNGTEAKL VRKVAGEKIT VTFNINNSIP PTFDGEEEPS
160 170 180 190 200
QGQKVEEQEP ELTSTPNFVV EVIKNDDGKK ALVLDCHYPE DEVGQEDEAE
210 220 230 240 250
SDIFSIREVS FQSTGESEWK DTNYTLNTDS LDWALYDHLM DFLADRGVDN
260 270 280
TFADELVELS TALEHQEYIT FLEDLKSFVK SQ
Length:282
Mass (Da):31,362
Last modified:February 1, 1995 - v1
Checksum:i2F747FA73BB1314B
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L04636 mRNA. Translation: AAA16315.1.
X75913 mRNA. Translation: CAA53512.1.
AF338439 Genomic DNA. Translation: AAK26580.1.
BT019898 mRNA. Translation: AAV38701.1.
BT019899 mRNA. Translation: AAV38702.1.
DQ372108 Genomic DNA. Translation: ABC79624.1.
BC000435 mRNA. Translation: AAH00435.1.
BC013731 mRNA. Translation: AAH13731.1.
M69039 mRNA. Translation: AAA73055.1.
AF275902 mRNA. Translation: AAF78763.1.
CCDSiCCDS11071.1.
PIRiJT0762.
RefSeqiNP_001203.1. NM_001212.3.
UniGeneiHs.555866.

Genome annotation databases

EnsembliENST00000225698; ENSP00000225698; ENSG00000108561.
GeneIDi708.
KEGGihsa:708.
UCSCiuc002gby.2. human.

Cross-referencesi

Web resourcesi

SeattleSNPs

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L04636 mRNA. Translation: AAA16315.1.
X75913 mRNA. Translation: CAA53512.1.
AF338439 Genomic DNA. Translation: AAK26580.1.
BT019898 mRNA. Translation: AAV38701.1.
BT019899 mRNA. Translation: AAV38702.1.
DQ372108 Genomic DNA. Translation: ABC79624.1.
BC000435 mRNA. Translation: AAH00435.1.
BC013731 mRNA. Translation: AAH13731.1.
M69039 mRNA. Translation: AAA73055.1.
AF275902 mRNA. Translation: AAF78763.1.
CCDSiCCDS11071.1.
PIRiJT0762.
RefSeqiNP_001203.1. NM_001212.3.
UniGeneiHs.555866.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1P32X-ray2.25A/B/C74-282[»]
3RPXX-ray2.65A/B/C96-282[»]
ProteinModelPortaliQ07021.
SMRiQ07021.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107169. 244 interactors.
DIPiDIP-31164N.
IntActiQ07021. 78 interactors.
MINTiMINT-246803.
STRINGi9606.ENSP00000225698.

Chemistry databases

DrugBankiDB08818. Hyaluronic acid.

Protein family/group databases

TCDBi9.B.103.1.1. the putative ca(2+) uniporter (gc1qr) family.

PTM databases

iPTMnetiQ07021.
PhosphoSitePlusiQ07021.
SwissPalmiQ07021.

Polymorphism and mutation databases

BioMutaiC1QBP.
DMDMi730772.

2D gel databases

DOSAC-COBS-2DPAGEQ07021.
OGPiQ07021.

Proteomic databases

EPDiQ07021.
MaxQBiQ07021.
PaxDbiQ07021.
PeptideAtlasiQ07021.
PRIDEiQ07021.
TopDownProteomicsiQ07021.

Protocols and materials databases

DNASUi708.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000225698; ENSP00000225698; ENSG00000108561.
GeneIDi708.
KEGGihsa:708.
UCSCiuc002gby.2. human.

Organism-specific databases

CTDi708.
DisGeNETi708.
GeneCardsiC1QBP.
HGNCiHGNC:1243. C1QBP.
HPAiHPA026483.
MIMi601269. gene.
neXtProtiNX_Q07021.
OpenTargetsiENSG00000108561.
PharmGKBiPA25624.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4024. Eukaryota.
ENOG4111G4Z. LUCA.
GeneTreeiENSGT00390000018406.
HOGENOMiHOG000046272.
HOVERGENiHBG000914.
InParanoidiQ07021.
KOiK15414.
OMAiCGCGGLH.
OrthoDBiEOG091G0IZD.
PhylomeDBiQ07021.
TreeFamiTF315160.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000108561-MONOMER.
ReactomeiR-HSA-140837. Intrinsic Pathway of Fibrin Clot Formation.

Miscellaneous databases

ChiTaRSiC1QBP. human.
EvolutionaryTraceiQ07021.
GeneWikiiC1QBP.
GenomeRNAii708.
PMAP-CutDBQ07021.
PROiQ07021.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000108561.
CleanExiHS_C1QBP.
ExpressionAtlasiQ07021. baseline and differential.
GenevisibleiQ07021. HS.

Family and domain databases

Gene3Di3.10.280.10. 1 hit.
InterProiIPR003428. MAM33.
[Graphical view]
PfamiPF02330. MAM33. 1 hit.
[Graphical view]
SUPFAMiSSF54529. SSF54529. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiC1QBP_HUMAN
AccessioniPrimary (citable) accession number: Q07021
Secondary accession number(s): Q2HXR8, Q9NNY8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1995
Last sequence update: February 1, 1995
Last modified: November 2, 2016
This is version 171 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

Caution

The subcellular location has been matter of debate. After being reported to be exclusively localized to mitochondria, demonstrations of promiscuous associations and locations have been rather considered as artifactual due to the extremely acidic character and the use of different tagged versions of the protein (PubMed:9305894, PubMed:11493647). However, by now the location to multiple compartments linked to diverse functions is accepted. The N-termini of the surface and secreted forms are identical to the reported processed mitochonddrial form.2 Publications

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 17
    Human chromosome 17: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. 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.