Properdin

Last updated
CFP
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases CFP , BFD, PFC, PFD, PROPERDIN, complement factor properdin, properdin
External IDs OMIM: 300383 MGI: 97545 HomoloGene: 1969 GeneCards: CFP
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_002621
NM_001145252

NM_008823

RefSeq (protein)

NP_001138724
NP_002612

NP_032849

Location (UCSC) Chr X: 47.62 – 47.63 Mb Chr X: 20.79 – 20.8 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Properdin is protein that in humans is encoded by the CFP (complement factor properdin) gene.

Contents

Properdin is plasma glycoprotein that activates the complement system of the innate immune system. This protein binds to bacterial cell walls and dying human cells to stabilize the C3 and C5-convertase enzyme complexes to form an attack complex that lead to the lysis of the cell.

Structure

Properdin is a gamma globulin protein composed of multiple identical protein subunits with a separate ligand-binding site. Native properdin occurs in head-to-tail dimers, trimers and tetramers in the fixed ratio 22:52:28. [5]

Function

It is known that it participates in some specific immune responses. It plays a part in tissue inflammation as well as the engulfing of pathogens by phagocytes. In addition it is known to help to neutralize some viruses.

The properdin promotes the association of C3b with Factor B and provides a focal point for the assembly of C3bBb on a surface. It binds to preformed alternative pathway C3-convertases. [6] Properdin also inhibits the Factor H – mediated cleavage of C3b by Factor I.

Alternative pathway. Properdin is the "P" in the blue circles. (Some labels are in Polish.) Droga alternatywna.png
Alternative pathway. Properdin is the "P" in the blue circles. (Some labels are in Polish.)

The alternative pathway is not dependent on antibodies. This branch of the complement system is activated by IgA immune complexes and bacterial endotoxins, polysaccharides, and cell walls, and results in producing anaphylatoxins, opsonins, chemotactic factors, and the membrane attack complex, all of which help fight pathogens.

History

Properdin was discovered in 1954 by Dr. Louis Pillemer of the Institute of Pathology (now the Department of Pathology at Case Western Reserve University).

Deficiency

Properdin deficiency is a rare X-linked disease in which properdin is deficient. Affected individuals are susceptible to fulminant meningococcal disease. [7]

Related Research Articles

Complement system Part of the immune system that enhances the ability of antibodies and phagocytic cells

The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and attack the pathogen's cell membrane. It is part of the innate immune system, which is not adaptable and does not change during an individual's lifetime. The complement system can, however, be recruited and brought into action by antibodies generated by the adaptive immune system.

Classical complement pathway Aspect of the immune system

The classical complement pathway is one of three pathways which activate the complement system, which is part of the immune system. The classical complement pathway is initiated by antigen-antibody complexes with the antibody isotypes IgG and IgM.

Alternative complement pathway

The alternative pathway is a type of cascade reaction of the complement system and is a component of the innate immune system, a natural defense against infections.

C3-convertase

C3 convertase belongs to family of serine proteases and is necessary in innate immunity as a part of the complement system which eventuate in opsonisation of particles, release of inflammatory peptides, C5 convertase formation and cell lysis.

Complement component 3 Protein-coding gene in the species Homo sapiens

Complement component 3, often simply called C3, is a protein of the immune system. It plays a central role in the complement system and contributes to innate immunity. In humans it is encoded on chromosome 19 by a gene called C3.

C5-convertase Serine protease that plays key role in innate immunity.

C5 convertase is an enzyme belonging to a family of serine proteases that play key role in the innate immunity. It participates in the complement system ending with cell death.

<span class="mw-page-title-main">Lectin pathway</span>

The lectin pathway or lectin complement pathway is a type of cascade reaction in the complement system, similar in structure to the classical complement pathway, in that, after activation, it proceeds through the action of C4 and C2 to produce activated complement proteins further down the cascade. In contrast to the classical complement pathway, the lectin pathway does not recognize an antibody bound to its target. The lectin pathway starts with mannose-binding lectin (MBL) or ficolin binding to certain sugars.

Complement component 7

Complement component 7 is a protein involved in the complement system of the innate immune system. C7 is part of the membrane attack complex (MAC) which creates a hole on pathogen surfaces, leading to cell lysis and death.

Complement factor B Protein-coding gene in the species Homo sapiens

Complement factor B is a protein that in humans is encoded by the CFB gene.

<span class="mw-page-title-main">Decay-accelerating factor</span> Mammalian protein found in Homo sapiens

Complement decay-accelerating factor, also known as CD55 or DAF, is a protein that, in humans, is encoded by the CD55 gene.

Mannan-binding lectin

Mannose-binding lectin (MBL), also called mannan-binding lectin or mannan-binding protein (MBP), is a lectin that is instrumental in innate immunity as an opsonin and via the lectin pathway.

C3b

C3b is the larger of two elements formed by the cleavage of complement component 3, and is considered an important part of the innate immune system. C3b is potent in opsonization: tagging pathogens, immune complexes (antigen-antibody), and apoptotic cells for phagocytosis. Additionally, C3b plays a role in forming a C3 convertase when bound to Factor B, or a C5 convertase when bound to C4b and C2b or when an additional C3b molecule binds to the C3bBb complex.

IRF3 Protein-coding gene in the species Homo sapiens

Interferon regulatory factor 3, also known as IRF3, is an interferon regulatory factor.

MAPK3

Mitogen-activated protein kinase 3, also known as p44MAPK and ERK1, is an enzyme that in humans is encoded by the MAPK3 gene.

<span class="mw-page-title-main">Death receptor 4</span> Protein-coding gene in the species Homo sapiens

Death receptor 4 (DR4), also known as TRAIL receptor 1 (TRAILR1) and tumor necrosis factor receptor superfamily member 10A (TNFRSF10A), is a cell surface receptor of the TNF-receptor superfamily that binds TRAIL and mediates apoptosis.

MAP3K14

Mitogen-activated protein kinase kinase kinase 14 also known as NF-kappa-B-inducing kinase (NIK) is an enzyme that in humans is encoded by the MAP3K14 gene.

NFYC Protein-coding gene in the species Homo sapiens

Nuclear transcription factor Y subunit gamma is a protein that in humans is encoded by the NFYC gene.

<span class="mw-page-title-main">TRAF4</span>

TNF receptor-associated factor 4 (TRAF4) also known as RING finger protein 83 (RNF83) is a protein that in humans is encoded by the TRAF4 gene.

CFHR5 Protein-coding gene in the species Homo sapiens

Complement factor H-related protein 5 is a protein that in humans is encoded by the CFHR5 gene.

C3a (complement)

C3a is one of the proteins formed by the cleavage of complement component 3; the other is C3b. C3a is a 77 residue anaphylatoxin that binds to the C3a receptor (C3aR), a class A G protein-coupled receptor. It plays a large role in the immune response.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000126759 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000001128 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Smith C, Pangburn M, Vogel CW, Müller-Eberhard H (1984). "Molecular Architecture of Human Properdin, a Positive Regulator of the Alternative Pathway of Complement". J Biol Chem. 259 (7): R4582–4588. doi: 10.1016/S0021-9258(17)43086-9 . PMID   6707020.
  6. Hourcade D (2006). "The Role of Properdin in the Assembly of the Alternative Pathway C3 Convertases of Complement". J Biol Chem. 281 (4): R2128–2132. doi: 10.1074/jbc.m508928200 . PMID   16301317.
  7. Dr. Lars Otto Uttenthal Properdin September 01 2005 Archived 2012-04-02 at the Wayback Machine