Complement component 1 Q subcomponent-binding protein, mitochondrial

Details

Name
Complement component 1 Q subcomponent-binding protein, mitochondrial
Synonyms
  • ASF/SF2-associated protein p32
  • C1qBP
  • gC1q-R protein
  • GC1QBP
  • Glycoprotein gC1qBP
  • HABP1
  • Hyaluronan-binding protein 1
  • Mitochondrial matrix protein p32
  • p33
  • SF2P32
Gene Name
C1QBP
Organism
Humans
Amino acid sequence
>lcl|BSEQ0019649|Complement component 1 Q subcomponent-binding protein, mitochondrial
MLPLLRCVPRVLGSSVAGLRAAAPASPFRQLLQPAPRLCTRPFGLLSVRAGSERRPGLLR
PRGPCACGCGCGSLHTDGDKAFVDFLSDEIKEERKIQKHKTLPKMSGGWELELNGTEAKL
VRKVAGEKITVTFNINNSIPPTFDGEEEPSQGQKVEEQEPELTSTPNFVVEVIKNDDGKK
ALVLDCHYPEDEVGQEDEAESDIFSIREVSFQSTGESEWKDTNYTLNTDSLDWALYDHLM
DFLADRGVDNTFADELVELSTALEHQEYITFLEDLKSFVKSQ
Number of residues
282
Molecular Weight
31361.935
Theoretical pI
Not Available
GO Classification
Functions
adrenergic receptor binding / complement component C1q binding / hyaluronic acid binding / kininogen binding / mitochondrial ribosome binding / mRNA binding / transcription corepressor activity / transcription factor binding
Processes
apoptotic process / blood coagulation / blood coagulation, intrinsic pathway / complement activation, classical pathway / immune response / innate immune response / mature ribosome assembly / mRNA processing / negative regulation of defense response to virus / negative regulation of interferon-gamma production / negative regulation of interleukin-12 production / negative regulation of MDA-5 signaling pathway / negative regulation of mRNA splicing, via spliceosome / negative regulation of RIG-I signaling pathway / negative regulation of transcription from RNA polymerase II promoter / phosphatidylinositol 3-kinase signaling / positive regulation of apoptotic process / positive regulation of cell adhesion / positive regulation of dendritic cell chemotaxis / positive regulation of mitochondrial translation / positive regulation of neutrophil chemotaxis / positive regulation of protein kinase B signaling / positive regulation of substrate adhesion-dependent cell spreading / positive regulation of trophoblast cell migration / regulation of complement activation / RNA splicing / transcription, DNA-templated / viral process
Components
cell surface / cytoplasm / cytosol / extracellular space / membrane / mitochondrial matrix / mitochondrion / nucleolus / nucleus / plasma membrane
General Function
Transcription factor binding
Specific 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. 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.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion matrix
Gene sequence
>lcl|BSEQ0019650|Complement component 1 Q subcomponent-binding protein, mitochondrial (C1QBP)
ATGCTGCCTCTGCTGCGCTGCGTGCCCCGTGTGCTGGGCTCCTCCGTCGCCGGCCTCCGC
GCTGCCGCGCCCGCCTCGCCTTTCCGGCAGCTCCTGCAGCCGGCACCCCGGCTGTGCACC
CGGCCCTTCGGGCTGCTCAGCGTGCGCGCAGGTTCCGAGCGGCGGCCGGGCCTCCTGCGG
CCTCGCGGACCCTGCGCCTGTGGCTGTGGCTGCGGCTCGCTGCACACCGACGGAGACAAA
GCTTTTGTTGATTTCCTGAGTGATGAAATTAAGGAGGAAAGAAAAATTCAGAAGCATAAA
ACCCTCCCTAAGATGTCTGGAGGTTGGGAGCTGGAACTGAATGGGACAGAAGCGAAATTA
GTGCGGAAAGTTGCCGGGGAAAAAATCACGGTCACTTTCAACATTAACAACAGCATCCCA
CCAACATTTGATGGTGAGGAGGAACCCTCGCAAGGGCAGAAGGTTGAAGAACAGGAGCCT
GAACTGACATCAACTCCCAATTTCGTGGTTGAAGTTATAAAGAATGATGATGGCAAGAAG
GCCCTTGTGTTGGACTGTCATTATCCAGAGGATGAGGTTGGACAAGAAGACGAGGCTGAG
AGTGACATCTTCTCTATCAGGGAAGTTAGCTTTCAGTCCACTGGCGAGTCTGAATGGAAG
GATACTAATTATACACTCAACACAGATTCCTTGGACTGGGCCTTATATGACCACCTAATG
GATTTCCTTGCCGACCGAGGGGTGGACAACACTTTTGCAGATGAGCTGGTGGAGCTCAGC
ACAGCCCTGGAGCACCAGGAGTACATTACTTTTCTTGAAGACCTCAAGAGTTTTGTCAAG
AGCCAGTAG
Chromosome Location
17
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDQ07021
UniProtKB Entry NameC1QBP_HUMAN
HGNC IDHGNC:1243
General References
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB08818Hyaluronic acidapproved, vet_approvedunknownbinderDetails
DB09130Copperapproved, investigationalunknownDetails