WordNet

the 2nd letter of the Roman alphabet (同)b
the blood group whose red cells carry the B antigen (同)type_B, group B

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/30 23:31:08」(JST)

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Properdin or Factor P is the only known positive regulator of complement activation that stabilizes the alternative pathway convertases. It is found in the blood serum of more complex animals.

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.^[1]

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.^[2] 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.)

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.^[3]

External links

Properdin at the US National Library of Medicine Medical Subject Headings (MeSH)

^ Smith C, Pangburn M, Vogel C-W, and Müller-Eberhard H (1984). "Molecular Architecture of Human Properdin, a Positive Regulator of the Alternative Pathway of Complement". J of Biol Chem 259: R4582–4588.
^ Hourcade D (2006). "The Role of Properdin in the Assembly of the Alternative Pathway C3 Convertases of Complement". J of Biol Chem 281: R2128–2132. doi:10.1074/jbc.m508928200.
^ Dr. Lars Otto Uttenthal Properdin September 01 2005

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UpToDate Contents

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1. 補体系の遺伝性疾患 inherited disorders of the complement system
2. 補体経路 complement pathways
3. 髄膜炎菌感染の疫学 epidemiology of neisseria meningitidis infection
4. Acquired deficiencies of the complement system
5. 補体系の概要および臨床的評価 overview and clinical assessment of the complement system

English Journal

Soluble Collectin-12 (CL-12) Is a Pattern Recognition Molecule Initiating Complement Activation via the Alternative Pathway.

Ma YJ1, Hein E1, Munthe-Fog L1, Skjoedt MO1, Bayarri-Olmos R1, Romani L2, Garred P3.
Journal of immunology (Baltimore, Md. : 1950).J Immunol.2015 Oct 1;195(7):3365-73. doi: 10.4049/jimmunol.1500493. Epub 2015 Aug 19.
Soluble defense collagens including the collectins play important roles in innate immunity. Recently, a new member of the collectin family named collectin-12 (CL-12 or CL-P1) has been identified. CL-12 is highly expressed in umbilical cord vascular endothelial cells as a transmembrane receptor and m
PMID 26290605

Inhibition of aberrant complement activation by a dimer of acetylsalicylic acid.

Lee M1, Wathier M2, Love JA2, McGeer E1, McGeer PL3.
Neurobiology of aging.Neurobiol Aging.2015 Oct;36(10):2748-56. doi: 10.1016/j.neurobiolaging.2015.06.018. Epub 2015 Jun 17.
We here report synthesis for the first time of the acetyl salicylic acid dimer 5,5'-methylenebis(2-acetoxybenzoic acid) (DAS). DAS inhibits aberrant complement activation by selectively blocking factor D of the alternative complement pathway and C9 of the membrane attack complex. We have previously
PMID 26248865

Neutrophil extracellular traps can activate alternative complement pathways.

Wang H1,2,3,4,5, Wang C1,2,3,4,5, Zhao MH1,2,3,4,5, Chen M1,2,3,4,5.
Clinical and experimental immunology.Clin Exp Immunol.2015 Sep;181(3):518-27. doi: 10.1111/cei.12654. Epub 2015 Jun 22.
The interaction between neutrophils and activation of alternative complement pathway plays a pivotal role in the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). ANCAs activate primed neutrophils to release neutrophil extracellular traps (NETs), which have rec
PMID 25963026

Japanese Journal

Nephritic factor 研究の経緯からみた膜性増殖性糸球体腎炎の病態

大井洋之
The Japanese journal of nephrology 54(7), 1006-1015, 2012-10-25
NAID 10031122285

プロパージン,H因子,I因子 (広範囲血液・尿化学検査免疫学的検査(第7版・3)その数値をどう読むか) -- (免疫学的検査補体および関連物質)

大澤勲,富野康日己
日本臨床 68(-), 77-81, 2010-06
NAID 40017181710

Expression Responses of the Complement Components in Zebrafish Organs after Stimulation with Poly I:C, Mimicry of Viral Infection

Vo Kha Tam,Tsujikura Masakazu,Somamoto Tomonori,Nakano Miki
Journal of the Faculty of Agriculture, Kyushu University 54(2), 389-395, 2009-10-29
… Upon stimulation, the hepatopancreas showed decreased expression of CD11/CD18, B/C2-A3, MASP3, and properdin, while marked up-regulation of several complement component genes was observed in the gill, suggesting an important but unknown role of the gill in host defense against viral infection. …
NAID 120001766564

Related Pictures

Properdin Antibody (C-4) | Santa Cruz Biotech Complement system Complement c3b Frontiers | Properdin and Factor H: Opposing Players on the Alternative Complement IM1 09 10: Innate and Adaptive Immune Responses at Touro University (NV) - StudyBlue [Frontiers in Bioscience 12, 4696-4708, May 1, 2007] PPT - ‍ Complement system PowerPoint Presentation - ID:5754596 Properdin; Complement Factor P

★リンクテーブル★

リンク元	「プロパージン」「B」
拡張検索	「properdin deficiency」

「プロパージン」

complement factor properdin
Identifiers
Symbol	CFP
Alt. symbols	PFC
Entrez	5199
HUGO	8864
OMIM	300383
RefSeq	NM_002621
UniProt	P27918
Other data
Locus	Chr. X p11.4

　　[★]

英: properdin
同: プロパジン、プロペルジン; factor P
関: 補体、complement-regulatory protein

[show details]

プロパージン : 64 件
プロパジン : 約 4,060 件
プロペルジン : 約 64 件

プロパージン 補体 : 45 件
プロパジン 補体 : 10 件
プロペルジン 補体 : 41 件

プロパージン properdin : 33 件
プロパジン properdin : 12 件
プロペルジン properdin : 28 件

補体のalternative pathwayに関与
外来異物表面上でC3 convertase enzyme(C3bBb複合体)を安定化する作用がある。(IMM.71)

「B」

　　[★]

気管分岐部下緑
補体のalternative pathwayに関わる蛋白質

Mg2+存在下でC3, B, Dが反応してC3bBbとなり、これがC3転換酵素(C3bBb)あるいはC5転換酵素(C3bBb3b)を形成する。これらはP(properdin)と結合して活性化し、それぞれC3、C5を活性化する

「properdin deficiency」

　　[★] プロパージン欠損症

[1] Smith C, Pangburn M, Vogel C-W, and Müller-Eberhard H (1984). "Molecular Architecture of Human Properdin, a Positive Regulator of the Alternative Pathway of Complement". J of Biol Chem 259: R4582–4588.

[2] Hourcade D (2006). "The Role of Properdin in the Assembly of the Alternative Pathway C3 Convertases of Complement". J of Biol Chem 281: R2128–2132. doi:10.1074/jbc.m508928200.

[3] Dr. Lars Otto Uttenthal Properdin September 01 2005

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properdin