C4BPB Gene Summary [Human]

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]

Details

Type
Protein Coding
Official Symbol
C4BPB
Official Name
complement component 4 binding protein beta [Source:HGNC Symbol;Acc:HGNC:1328]
Ensembl ID
ENSG00000123843
Bio databases IDs NCBI: 725 Ensembl: ENSG00000123843
Aliases complement component 4 binding protein beta, complement component 4 binding protein, beta chain, C4b binding protein, beta chain
Synonyms C4BP, C4bp-ps1, complement component 4 binding protein beta, complement component 4 binding protein, beta, complement component 4 binding protein β, complement component 4 binding protein, β
Species
Human, Homo sapiens
OrthologiesRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human C4BPB often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • CCP
  • alpha helix
  • protein binding
  • Sushi repeat (SCR repeat)
  • SUSHI repeat

Pathways

Biological processes and signaling networks where the C4BPB gene in human plays a role, providing insight into its function and relevance in health or disease.

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • polycystic kidney disease
regulated by
regulates

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Extracellular Space
  • Plasma Membrane
  • plasma

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the human C4BPB gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • negative regulation of complement activation, classical pathway
  • complement activation, classical pathway
  • innate immune response
  • response to symbiotic bacterium
  • positive regulation of protein catabolic process
  • blood coagulation

Cellular Component

Where in the cell the gene product is active
  • extracellular space
  • extracellular region
  • plasma membrane

Molecular Function

What the gene product does at the molecular level
  • protein binding

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