C4BPB - complement component 4 binding protein beta Gene

Homo sapiens

Also known as C4BP

Gene ID: 725 | Gene type: protein coding

About C4BPB

Cytogenetic location: 1q32.1 Genomic coordinates (GRCh38): 1:207,088,860-207,099,993 (from NCBI)

This gene has 8 transcripts (splice variants), 76 orthologues and 39 paralogues. Biased expression in liver (RPKM 52.4) and ovary (RPKM 16.6).

Summary

This gene encodes a member of a superfamily of proteins composed predominantly of tandemly arrayed short consensus repeats of approximately 60 Amino acids. A single, unique beta-chain encoded by this gene assembles with seven identical alpha-chains into the predominant isoform of C4b-binding protein, a multimeric protein that controls activation of the complement cascade through the classical pathway. C4b-binding protein has a regulatory role in the coagulation system also, mediated through the beta-chain binding of protein S, a vitamin K-dependent protein that serves as a cofactor of activated protein C. The genes encoding both alpha and beta chains are located adjacent to each other on human chromosome 1 in the regulator of complement activation gene cluster. Alternative splicing gives rise to multiple transcript variants. [provided by RefSeq, Jul 2008]

C4BPB Products(5)

mRNA	Protein	Name
NM_000716.3	NP_000707.1	C4b-binding protein beta chain isoform 1 precursor
NM_001017364.1	NP_001017364.1	C4b-binding protein beta chain isoform 2 precursor
NM_001017365.3	NP_001017365.1	C4b-binding protein beta chain isoform 1 precursor
NM_001017366.3	NP_001017366.1	C4b-binding protein beta chain isoform 2 precursor
NM_001017367.1	NP_001017367.1	C4b-binding protein beta chain isoform 1 precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables protein binding	IPI IPI: Inferred from physical interaction	14607961	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in negative regulation of complement activation, classical pathway	IDA IDA: Inferred from direct assay	22333221	GOA
involved in positive regulation of protein catabolic process	IDA IDA: Inferred from direct assay	22333221	GOA
involved in regulation of opsonization	IDA IDA: Inferred from direct assay	22333221	GOA
involved in response to symbiotic bacterium	IDA IDA: Inferred from direct assay	22333221	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in extracellular space	IDA IDA: Inferred from direct assay	14607961	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

C4BPB Protein Structure

Sushi

0
100
200
252 a.a.

Protein Preferred Names

Protein Names

C4b-binding protein beta chain

Related Diseases

Diseases

Alias

Patellar Tendinitis

Patellar Tendonitis

Hemolytic Uremic Syndrome, Atypical 1

Atypical Hemolytic-Uremic Syndrome	Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 1
Atypical Hemolytic Uremic Syndrome	Hemolytic Uremic Syndrome, Atypical, Susceptibility To
Ahus	AHUS1
Hemolytic-Uremic Syndrome	Ahus 1
Ahus, Susceptibility To, 1	Hemolytic Uremic Syndrome, Atypical
Non-Shiga-Like Toxin-Associated Hus	Non-Stx-Hus
Nonenteropathic Hus	Atypical Hus
Shiga Toxin-Associated Hemolytic Uremic Syndrome	D+ Hus
Ehec-Hus	Hemolytic Uremic Syndrome Associated With Shiga Toxin-Producing Escherichia Coli
Hemolytic Uremic Syndrome With Diarrhea	Stec-Hus
Shiga-Like Toxin-Associated Hus	Stx-Hus
Typical Hus	Typical Hemolytic Uremic Syndrome
Atypical Hemolytic Uremic Syndrome With Anti-Factor H Antibodies	Atypical Hus With Anti-Factor H Antibodies
Ahus With Anti-Factor H Antibodies	Ahus With Neutralizing Autoantibodies Against Factor H
Hemolytic Uremic Syndrome Atypical 1	Atypical Hemolytic Uremic Syndrome With H Factor Anomaly
D Hus	Hemolytic-Uremic Syndrome Without Diarrhea
Hemolytic-Uremic Syndrome, Atypical, Type 1	Hemolytic Uremic Syndrome, Typical

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS01748	C4BPB Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	C4BPB	VGNC	VGNC:38594
Macaca mulatta	C4BPB	VGNC	VGNC:99859
Bos taurus	C4BPB	VGNC	VGNC:26640
Felis catus	C4BPB	VGNC	VGNC:107846
Rattus norvegicus	C4BPB	RGD	RGD:2236

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