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Category Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21

GIFtS
-

C1QBP Gene

protein-coding GIFtS: 61
GCID: GC17M005336

complement component 1, q subcomponent binding protein

(Previous symbol: HABP1)

Explore 24 diseases affiliated with
C1QBP via

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(According to ¹HGNC, ²Entrez Gene,
³UniProtKB/Swiss-Prot, ⁴UniProtKB/TrEMBL, ⁵OMIM, ⁶GeneLoc, ⁷Ensembl, ⁸DME, ⁹miRBase, and/or ¹⁰fRNAdb)
About This Section

Aliases
Complement Component 1, Q Subcomponent Binding Protein¹ ²		GC1QBP² ³
HABP1¹ ² ³ ⁵		ASF/SF2-Associated Protein P32² ³
SF2p32¹ ²		P33¹
GC1Q-R¹		C1q Globular Domain-Binding Protein²
GC1qR¹		Complement Component 1 Q Subcomponent-Binding Protein, Mitochondrial²
P32¹		Splicing Factor SF2-Associated Protein²
Glycoprotein GC1qBP² ³		C1qBP³
Hyaluronan-Binding Protein 1² ³		SF2P32³
Mitochondrial Matrix Protein P32² ³		GC1q-R Protein³

External Ids:	HGNC: 1243¹	Entrez Gene: 708²	Ensembl: ENSG00000108561⁷	OMIM: 601269⁵	UniProtKB: Q07021³

Export aliases for C1QBP gene to outside databases

Previous GC identifers: GC17M005721 GC17M005675 GC17M005280 GC17M005536 GC17M005276 GC17M005226

(According to Entrez Gene, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
About This Section

Entrez Gene summary for C1QBP:

The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum

complement system. The protein encoded by this gene is known to bind to the globular heads of C1q molecules and

inhibit C1 activation. This protein has also been identified as the p32 subunit of pre-mRNA splicing factor SF2, as

well as a hyaluronic acid-binding protein. (provided by RefSeq, Jul 2008)

UniProtKB/Swiss-Prot: C1QBP_HUMAN, Q07021

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 affinty 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

Gene Wiki entry for C1QBP

(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 69), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section

RefSeq DNA sequence:

NC_000017.10 NC_018928.1 NT_010718.16

Regulatory elements:

SABiosciences Regulatory transcription factor binding sites in the C1QBP gene promoter:
SRF E2F-4 E2F-3a E2F-5 E2F-1 E2F SRF (504 AA) E2F-2
Other transcription factors

Browse SwitchGear Promoter luciferase reporter plasmids

Search SABiosciences Chromatin IP Primers for C1QBP

Epigenetics:

QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat C1QBP

Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 17p13.3 Ensembl cytogenetic band: 17p13.2 HGNC cytogenetic band: 17p13.3

C1QBP Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
C1QBP gene location

GeneLoc information about chromosome 17 GeneLoc Exon Structure

GeneLoc location for GC17M005336: view genomic region (about GC identifiers)

Start:	5,336,097 bp from pter	End:	5,352,150 bp from pter
Size:	16,054 bases	Orientation:	minus strand

(According to ¹UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to ²PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE MOPED and PaxDb, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, and/or Uscn,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Uscn, Ontologies according to Gene Ontology Consortium 01 Mar 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, Abcam, and/or Uscn)
About This Section

UniProtKB/Swiss-Prot: C1QBP_HUMAN, Q07021 (See protein sequence)

Recommended Name: Complement component 1 Q subcomponent-binding protein, mitochondrial precursor

Size: 282 amino acids; 31362 Da

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, 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

tansfection. 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

Subcellular location: Mitochondrion matrix. Nucleus. Cell membrane; Peripheral membrane protein; Extracellular side.

Secreted. Cytoplasm. Nucleus, nucleolus. Note=Seems to be predominantly localized to mitochondria. Secreted by

activated lymphocytes

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 PDB 3D structures from and Proteopedia for C1QBP:

1P32 (3D)

3RPX (3D)

Secondary accessions: Q2HXR8 Q9NNY8

Explore the universe of human proteins at neXtProt for C1QBP: NX_Q07021

Post-translational modifications:

View modification sites using PhosphoSitePlus²

View neXtProt modification sites for NX_Q07021

C1QBP Protein expression data from MOPED and PaxDb: About this image

REFSEQ proteins: NP_001203.1

ENSEMBL proteins:

ENSP00000225698 ENSP00000460308 ENSP00000460638 ENSP00000458624

Reactome Protein details: Q07021
Human Recombinant Protein Products:

	Browse Purified and Recombinant Proteins at EMD Millipore
	R&D Systems Recombinant & Natural Proteins for C1QBP (HABP1/C1QBP)
	Browse recombinant and purified proteins available from Enzo Life Sciences
	Browse OriGene full length recombinant human proteins expressed in human HEK293 cells Browse OriGene Protein Over-expression Lysates OriGene Custom Protein Services for C1QBP
	GenScript Custom Purified and Recombinant Proteins Services for C1QBP
	Novus Biologicals C1QBP Proteins Novus Biologicals C1QBP Lysates
	Browse Sino Biological Recombinant Proteins
	ProSpec Recombinant Protein for C1QBP
	Uscn Proteins for C1QBP

Gene Ontology (GO): 5/10 cellular component terms (GO ID links to tree view) (see all 10): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0005615	extracellular space	IEA	--
GO:0005634	nucleus	IDA	15243141
GO:0005730	nucleolus	IEA	--
GO:0005737	cytoplasm	ISS	--
GO:0005739	mitochondrion	IDA	19164550

C1QBP for ontologies About GeneDecksing

C1QBP Antibody Products:

	EMD Millipore Mono- and Polyclonal Antibodies for the study of C1QBP
	R&D Systems Antibodies for C1QBP (HABP1/C1QBP)
	Cell Signaling Technology (CST) Antibodies for C1QBP
	OriGene Antibodies (see all 2): C1QBP OriGene Custom Antibody Services for C1QBP
	GenScript Custom Superior Antibodies Services for C1QBP
	Novus Biologicals C1QBP Antibodies
	Abcam antibodies for C1QBP
	Uscn Antibodies for C1QBP
	ThermoFisher Antibodies for C1QBP

Assay Products for C1QBP:

	Browse Kits and Assays available from EMD Millipore
	OriGene Custom Immunoassay Development Browse OriGene Fluorogenic Cell Assay Kits
	Browse R&D Systems for biochemical assays
	GenScript Custom Assay Services for C1QBP
	Browse Enzo Life Sciences for kits & assays
	Uscn ELISAs and CLIAs for C1QBP

(According to InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
About This Section

C1QBP for domains About GeneDecksing

1 InterPro domain/family:

IPR003428 MAM33

Graphical View of Domain Structure for InterPro Entry Q07021

ProtoNet protein and cluster: Q07021

1 Blocks protein family: IPB003428 Mitochondrial glycoprotein

UniProtKB/Swiss-Prot: C1QBP_HUMAN, Q07021

Similarity: Belongs to the MAM33 family

(According to ¹UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or ²DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013,
bound targets from SABiosciences, miRNA Gene Targets from miRTarBase shRNA from OriGene, RNAi from EMD Millipore, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, Gene Editing from DNA2.0, Clones from EMD Millipore, OriGene, SwitchGear Genomics, GenScript, Sino Biological, DNA2.0, and Vector BioLabs, Cell Lines from GenScript, LifeMap BioReagents, In Situ Hybridization Assays from Advanced Cell Diagnostics, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene.)
About This Section

Function Summary: