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Complement component 1 Q subcomponent-binding protein, mitochondrial

 C1QBP_BOVIN             Reviewed;         278 AA.
Q3T0B6;
30-MAY-2006, integrated into UniProtKB/Swiss-Prot.
11-OCT-2005, sequence version 1.
28-MAR-2018, entry version 82.
RecName: Full=Complement component 1 Q subcomponent-binding protein, mitochondrial;
Flags: Precursor;
Name=C1QBP;
Bos taurus (Bovine).
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Mammalia; Eutheria; Laurasiatheria; Cetartiodactyla; Ruminantia;
Pecora; Bovidae; Bovinae; Bos.
NCBI_TaxID=9913;
[1]
NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
STRAIN=Crossbred X Angus; TISSUE=Ileum;
NIH - Mammalian Gene Collection (MGC) project;
Submitted (AUG-2005) to the EMBL/GenBank/DDBJ databases.
-!- FUNCTION: 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. 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 (By similarity). {ECO:0000250}.
-!- SUBUNIT: 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 and SRSF9. 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. {ECO:0000250|UniProtKB:O35658}.
-!- SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250}. Nucleus
{ECO:0000250}. Cell membrane {ECO:0000250}; Peripheral membrane
protein {ECO:0000250}; Extracellular side {ECO:0000250}. Secreted
{ECO:0000250}. Cytoplasm {ECO:0000250}. Nucleus, nucleolus
{ECO:0000250}. Note=Seems to be predominantly localized to
mitochondria. {ECO:0000250}.
-!- SIMILARITY: Belongs to the MAM33 family. {ECO:0000305}.
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EMBL; BC102464; AAI02465.1; -; mRNA.
RefSeq; NP_001029699.1; NM_001034527.2.
UniGene; Bt.16082; -.
ProteinModelPortal; Q3T0B6; -.
SMR; Q3T0B6; -.
STRING; 9913.ENSBTAP00000013734; -.
PaxDb; Q3T0B6; -.
PeptideAtlas; Q3T0B6; -.
PRIDE; Q3T0B6; -.
Ensembl; ENSBTAT00000013734; ENSBTAP00000013734; ENSBTAG00000012739.
GeneID; 518321; -.
KEGG; bta:518321; -.
CTD; 708; -.
VGNC; VGNC:26618; C1QBP.
eggNOG; KOG4024; Eukaryota.
eggNOG; ENOG4111G4Z; LUCA.
GeneTree; ENSGT00390000018406; -.
HOGENOM; HOG000046272; -.
HOVERGEN; HBG000914; -.
InParanoid; Q3T0B6; -.
KO; K15414; -.
OMA; ATHYEHS; -.
OrthoDB; EOG091G0IZD; -.
TreeFam; TF315160; -.
Reactome; R-BTA-140837; Intrinsic Pathway of Fibrin Clot Formation.
Proteomes; UP000009136; Chromosome 19.
Bgee; ENSBTAG00000012739; -.
GO; GO:0009986; C:cell surface; IEA:Ensembl.
GO; GO:0005737; C:cytoplasm; ISS:UniProtKB.
GO; GO:0005829; C:cytosol; IEA:Ensembl.
GO; GO:0005576; C:extracellular region; IEA:UniProtKB-SubCell.
GO; GO:0005759; C:mitochondrial matrix; IEA:UniProtKB-SubCell.
GO; GO:0005730; C:nucleolus; IEA:UniProtKB-SubCell.
GO; GO:0005634; C:nucleus; ISS:UniProtKB.
GO; GO:0005886; C:plasma membrane; IEA:UniProtKB-SubCell.
GO; GO:0043234; C:protein complex; IDA:AgBase.
GO; GO:0031690; F:adrenergic receptor binding; ISS:UniProtKB.
GO; GO:0001849; F:complement component C1q binding; ISS:UniProtKB.
GO; GO:0005540; F:hyaluronic acid binding; ISS:UniProtKB.
GO; GO:0030984; F:kininogen binding; ISS:UniProtKB.
GO; GO:0097177; F:mitochondrial ribosome binding; ISS:UniProtKB.
GO; GO:0003729; F:mRNA binding; ISS:UniProtKB.
GO; GO:0003714; F:transcription corepressor activity; ISS:UniProtKB.
GO; GO:0008134; F:transcription factor binding; IEA:Ensembl.
GO; GO:0006915; P:apoptotic process; IEA:UniProtKB-KW.
GO; GO:0006958; P:complement activation, classical pathway; IEA:UniProtKB-KW.
GO; GO:0045087; P:innate immune response; IEA:UniProtKB-KW.
GO; GO:0042256; P:mature ribosome assembly; ISS:UniProtKB.
GO; GO:0006397; P:mRNA processing; IEA:UniProtKB-KW.
GO; GO:0050687; P:negative regulation of defense response to virus; ISS:UniProtKB.
GO; GO:0032689; P:negative regulation of interferon-gamma production; ISS:UniProtKB.
GO; GO:0032695; P:negative regulation of interleukin-12 production; ISS:UniProtKB.
GO; GO:0039534; P:negative regulation of MDA-5 signaling pathway; ISS:UniProtKB.
GO; GO:0048025; P:negative regulation of mRNA splicing, via spliceosome; ISS:UniProtKB.
GO; GO:0039536; P:negative regulation of RIG-I signaling pathway; ISS:UniProtKB.
GO; GO:0000122; P:negative regulation of transcription by RNA polymerase II; ISS:UniProtKB.
GO; GO:0014065; P:phosphatidylinositol 3-kinase signaling; ISS:UniProtKB.
GO; GO:0043065; P:positive regulation of apoptotic process; ISS:UniProtKB.
GO; GO:0045785; P:positive regulation of cell adhesion; ISS:UniProtKB.
GO; GO:2000510; P:positive regulation of dendritic cell chemotaxis; ISS:UniProtKB.
GO; GO:0070131; P:positive regulation of mitochondrial translation; ISS:UniProtKB.
GO; GO:0090023; P:positive regulation of neutrophil chemotaxis; ISS:UniProtKB.
GO; GO:0051897; P:positive regulation of protein kinase B signaling; ISS:UniProtKB.
GO; GO:1900026; P:positive regulation of substrate adhesion-dependent cell spreading; ISS:UniProtKB.
GO; GO:1901165; P:positive regulation of trophoblast cell migration; ISS:UniProtKB.
GO; GO:0030449; P:regulation of complement activation; ISS:UniProtKB.
GO; GO:0008380; P:RNA splicing; IEA:UniProtKB-KW.
GO; GO:0006351; P:transcription, DNA-templated; IEA:UniProtKB-KW.
Gene3D; 3.10.280.10; -; 1.
InterPro; IPR003428; MAM33.
InterPro; IPR036561; MAM33_sf.
PANTHER; PTHR10826; PTHR10826; 1.
Pfam; PF02330; MAM33; 1.
SUPFAM; SSF54529; SSF54529; 1.
2: Evidence at transcript level;
Acetylation; Adaptive immunity; Apoptosis; Cell membrane;
Complement pathway; Complete proteome; Cytoplasm; Immunity;
Innate immunity; Membrane; Mitochondrion; mRNA processing;
mRNA splicing; Nucleus; Phosphoprotein; Reference proteome;
Ribosome biogenesis; Secreted; Transcription;
Transcription regulation; Transit peptide.
TRANSIT 1 70 Mitochondrion. {ECO:0000250}.
CHAIN 71 278 Complement component 1 Q subcomponent-
binding protein, mitochondrial.
/FTId=PRO_0000238676.
REGION 73 90 C1q binding. {ECO:0000250}.
REGION 166 209 Interaction with MAVS. {ECO:0000250}.
COMPBIAS 143 146 Poly-Glu.
MOD_RES 84 84 Phosphoserine.
{ECO:0000250|UniProtKB:Q07021}.
MOD_RES 88 88 N6-acetyllysine.
{ECO:0000250|UniProtKB:Q07021}.
MOD_RES 91 91 N6-acetyllysine.
{ECO:0000250|UniProtKB:O35658}.
MOD_RES 185 185 Phosphotyrosine.
{ECO:0000250|UniProtKB:Q07021}.
MOD_RES 197 197 Phosphoserine.
{ECO:0000250|UniProtKB:Q07021}.
MOD_RES 201 201 Phosphoserine.
{ECO:0000250|UniProtKB:Q07021}.
MOD_RES 210 210 Phosphothreonine.
{ECO:0000250|UniProtKB:Q07021}.
SEQUENCE 278 AA; 30606 MW; 17C9ED0C3A426B15 CRC64;
MFQLLRCVPR VLGTAVAGLR AAAPSLPRLQ PASRPCARPF GLLSVRARSV QLPGLLKPRG
PCACGCGCSG LHTEGDKAFV DFLSDEIKEE KKIQKYKSLP KMSGGWELEV NGTEAKLVRK
VAGEKITVTF NINNSIPPAF GGEEEEPSQG QKAEEQEPEL TSTPNFVVEV TKDGSSKALV
LDCHYPEDEI GQEDDQSDIF SIKEVSFQAT GESDWKDTNY TLNTDSLDWG LYDHLMDFLA
DRGVDNTFAD ELVELSTALE HQEYISFLED LKGFVKSK


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