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Protein

Complement component 1 Q subcomponent-binding protein, mitochondrial

Gene

C1qbp

Organism
Mus musculus (Mouse)
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. May play a role in antibacterial defense. Involved in regulation of antiviral response by inhibiting DDX58- and IFIH1-mediated signaling pathways probably involving its association with MAVS after viral infection.4 Publications

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Adaptive immunity, Apoptosis, Complement pathway, Immunity, Innate immunity, mRNA processing, mRNA splicing, Ribosome biogenesis, Transcription, Transcription regulation

Names & Taxonomyi

Protein namesi
Recommended name:
Complement component 1 Q subcomponent-binding protein, mitochondrial
Alternative name(s):
GC1q-R protein
Glycoprotein gC1qBP
Short name:
C1qBP
Gene namesi
Name:C1qbp
Synonyms:Gc1qbp
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Unplaced

Organism-specific databases

MGIiMGI:1194505. C1qbp.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

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

Pathology & Biotechi

Disruption phenotypei

Embryonic lethal between E10.5 and E11.5. Severe dysfunction of the mitochondrial respiratory chain because of severly impaired mitochondrial protein synthesis.1 Publication

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi88K → A: Impairs RNA binding and mitochondrial translation; when associated with A-92. 1 Publication1
Mutagenesisi92K → A: Impairs RNA binding and mitochondrial translation; when associated with A-88. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 70MitochondrionBy similarityAdd BLAST70
ChainiPRO_000001859171 – 278Complement component 1 Q subcomponent-binding protein, mitochondrialAdd BLAST208

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei88N6-acetyllysineCombined sources1
Modified residuei91N6-acetyllysineCombined sources1
Modified residuei184PhosphotyrosineBy similarity1
Modified residuei197PhosphoserineCombined sources1
Modified residuei201PhosphoserineCombined sources1
Modified residuei210PhosphothreonineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiO35658.
MaxQBiO35658.
PaxDbiO35658.
PeptideAtlasiO35658.
PRIDEiO35658.
TopDownProteomicsiO35658.

PTM databases

iPTMnetiO35658.
PhosphoSitePlusiO35658.
SwissPalmiO35658.

Expressioni

Tissue specificityi

Ubiquitous.

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 U2AF1L4.4 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
U2af1l4Q8BGJ94EBI-642072,EBI-4288480

GO - Molecular functioni

Protein-protein interaction databases

DIPiDIP-32249N.
IntActiO35658. 59 interactors.
MINTiMINT-1651069.
STRINGi10090.ENSMUSP00000077612.

Structurei

3D structure databases

ProteinModelPortaliO35658.
SMRiO35658.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni73 – 90C1q bindingBy similarityAdd BLAST18
Regioni165 – 209Interaction with MAVSBy similarityAdd BLAST45

Sequence similaritiesi

Belongs to the MAM33 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4024. Eukaryota.
ENOG4111G4Z. LUCA.
HOVERGENiHBG000914.
InParanoidiO35658.

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.

O35658-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLPLLRCVPR SLGAASGLRT AIPAQPLRHL LQPAPRPCLR PFGLLSVRAG
60 70 80 90 100
SARRSGLLQP PVPCACGCGA LHTEGDKAFV EFLTDEIKEE KKIQKHKSLP
110 120 130 140 150
KMSGDWELEV NGTEAKLLRK VAGEKITVTF NINNSIPPTF DGEEEPSQGQ
160 170 180 190 200
KAEEQEPERT STPNFVVEVT KTDGKKTLVL DCHYPEDEIG HEDEAESDIF
210 220 230 240 250
SIKEVSFQAT GDSEWRDTNY TLNTDSLDWA LYDHLMDFLA DRGVDNTFAD
260 270
ELVELSTALE HQEYITFLED LKSFVKNQ
Length:278
Mass (Da):31,013
Last modified:January 1, 1998 - v1
Checksum:i7B438DD6EF88FFF0
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ001101 mRNA. Translation: CAA04530.1.
UniGeneiMm.30049.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ001101 mRNA. Translation: CAA04530.1.
UniGeneiMm.30049.

3D structure databases

ProteinModelPortaliO35658.
SMRiO35658.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

DIPiDIP-32249N.
IntActiO35658. 59 interactors.
MINTiMINT-1651069.
STRINGi10090.ENSMUSP00000077612.

PTM databases

iPTMnetiO35658.
PhosphoSitePlusiO35658.
SwissPalmiO35658.

Proteomic databases

EPDiO35658.
MaxQBiO35658.
PaxDbiO35658.
PeptideAtlasiO35658.
PRIDEiO35658.
TopDownProteomicsiO35658.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Organism-specific databases

MGIiMGI:1194505. C1qbp.

Phylogenomic databases

eggNOGiKOG4024. Eukaryota.
ENOG4111G4Z. LUCA.
HOVERGENiHBG000914.
InParanoidiO35658.

Miscellaneous databases

PROiO35658.
SOURCEiSearch...

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_MOUSE
AccessioniPrimary (citable) accession number: O35658
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 1, 1998
Last modified: November 2, 2016
This is version 113 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. 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.