関: C3 complement、complement 3

WordNet

make complete or perfect; supply what is wanting or form the complement to; "I need some pepper to complement the sweet touch in the soup"
a complete number or quantity; "a full complement"
one of a series of enzymes in the blood serum that are part of the immune response
something added to complete or embellish or make perfect; "a fine wine is a perfect complement to the dinner"; "wild rice was served as an accompaniment to the main dish" (同)accompaniment
number needed to make up a whole force; "a full complement of workers" (同)full complement
a word or phrase used to complete a grammatical construction
either of two parts that mutually complete each other
the 3rd letter of the Roman alphabet (同)c
(music) the keynote of the scale of C major
a general-purpose programing language closely associated with the UNIX operating system

PrepTutorEJDIC

(あるものを完全にするため)(…を)補う物(事)《+『of』+『名』》 / 補語(文法で文の成分の一つ) / (必要な)全数,全量;(船の)定員 / …を補足する,補う
carbonの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/11/07 17:52:34」(JST)

wiki en

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.^[4]^[5]

Function

C3 plays a central role in the activation of complement system.^[6] Its activation is required for both classical and alternative complement activation pathways. People with C3 deficiency are susceptible to bacterial infection.^[7]^[8]

One form of C3-convertase, also known as C4b2a, is formed by a heterodimer of activated forms of C4 and C2. It catalyzes the proteolytic cleavage of C3 into C3a and C3b, generated during activation through the classical pathway as well as the lectin pathway. C3a is an anaphylotoxin and the precursor of some cytokines such as ASP, and C3b serves as an opsonizing agent. Factor I can cleave C3b into C3c and C3d, the latter of which plays a role in enhancing B cell responses. In the alternative complement pathway, C3 is cleaved by C3bBb, another form of C3-convertase composed of activated forms of C3 (C3b) and factor B (Bb). Once C3 is activated to C3b, it exposes a reactive thioester that allows the peptide to covalently attach to any surface that can provide a nucleophile such as a primary amine or a hydroxyl group. Activated C3 can then interact with factor B. Factor B is then activated by factor D, to form Bb. The resultant complex, C3bBb, is called the alternative pathway (AP) C3 convertase.

C3bBb is deactivated in steps. First, the proteolytic component of the convertase, Bb, is removed by complement regulatory proteins having decay-accelerating factor (DAF) activity. Next, C3b is broken down progressively to first iC3b, then C3c + C3dg, and then finally C3d. Factor I is the protease that performs these cuts but it requires the help of another protein (Factor H, CR1, MCP or C4BP) to supply cofactor activity.

Structure

Several crystallographic structures of C3 have been determined^[9] and reveal that this protein contains 13 domains.^[10]^[11]^[12]^[13]

Clinical use

Complement tests
C4 (C)	FB (A)	C3	CH50	Conditions
·	↓	↓	↓	PSG, C3 NeF AA
↓	·	↓	·	HA, C4D
·	·	·	↓	TCPD
↓	· /↓	↓	↓	SLE
↑	↑	↑	↑	inflammation

Levels of C3 in the blood may be measured to support or refute a particular medical diagnosis. For example, low C3 levels are associated with Systemic Lupus Eritematosus ^[14] and some types of kidney disease such as post-infectious glomerulonephritis, membranoproliferative glomerulonephritis, and shunt nephritis.

.

Interactions

Complement component 3 has been shown to interact with Factor H.^[15]^[16]

Pathology

Deficiencies in C3 lead to generic infections, usually fatal to the newborn.^[17]

References

^ "Diseases that are genetically associated with C3 view/edit references on wikidata".
^ "Human PubMed Reference:".
^ "Mouse PubMed Reference:".
^ de Bruijn MH, Fey GH (Feb 1985). "Human complement component C3: cDNA coding sequence and derived primary structure". Proceedings of the National Academy of Sciences of the United States of America. 82 (3): 708–12. doi:10.1073/pnas.82.3.708. PMC 397115. PMID 2579379.
^ "Entrez Gene: C3 complement component 3".
^ Sahu A, Lambris JD (Apr 2001). "Structure and biology of complement protein C3, a connecting link between innate and acquired immunity". Immunological Reviews. 180: 35–48. doi:10.1034/j.1600-065X.2001.1800103.x. PMID 11414361.
^ Lachmann P (Dec 1975). "Genetics of the complement system". Journal of Medical Genetics. 12 (4): 372–7. doi:10.1136/jmg.12.4.372. PMC 1013316. PMID 768477.
^ Matsuyama W, Nakagawa M, Takashima H, Muranaga F, Sano Y, Osame M (Dec 2001). "Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters". Internal Medicine. 40 (12): 1254–8. doi:10.2169/internalmedicine.40.1254. PMID 11813855.
^ "Advanced search results for UniProt accession P01024". Protein Data Bank in Europe (PDBe). European Bioinformatics Institute. Retrieved 2010-12-27.
^ Janssen BJ, Huizinga EG, Raaijmakers HC, Roos A, Daha MR, Nilsson-Ekdahl K, Nilsson B, Gros P (Sep 2005). "Structures of complement component C3 provide insights into the function and evolution of immunity". Nature. 437 (7058): 505–11. doi:10.1038/nature04005. PMID 16177781.
^ Janssen BJ, Christodoulidou A, McCarthy A, Lambris JD, Gros P (Nov 2006). "Structure of C3b reveals conformational changes that underlie complement activity". Nature. 444 (7116): 213–6. doi:10.1038/nature05172. PMID 17051160.
^ Wiesmann C, Katschke KJ, Yin J, Helmy KY, Steffek M, Fairbrother WJ, McCallum SA, Embuscado L, DeForge L, Hass PE, van Lookeren Campagne M (Nov 2006). "Structure of C3b in complex with CRIg gives insights into regulation of complement activation". Nature. 444 (7116): 217–20. doi:10.1038/nature05263. PMID 17051150.
^ Fredslund F, Jenner L, Husted LB, Nyborg J, Andersen GR, Sottrup-Jensen L (Aug 2006). "The structure of bovine complement component 3 reveals the basis for thioester function". Journal of Molecular Biology. 361 (1): 115–27. doi:10.1016/j.jmb.2006.06.009. PMID 16831446.
^ https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=167&contentid=complement_c3_blood
^ Soames CJ, Sim RB (Sep 1997). "Interactions between human complement components factor H, factor I and C3b". The Biochemical Journal. 326 ( Pt 2): 553–61. PMC 1218704. PMID 9291131.
^ Jokiranta TS, Westin J, Nilsson UR, Nilsson B, Hellwage J, Löfås S, Gordon DL, Ekdahl KN, Meri S (Mar 2001). "Complement C3b interactions studied with surface plasmon resonance technique". International Immunopharmacology. 1 (3): 495–506. doi:10.1016/S1567-5769(00)00042-4. PMID 11367533.
^ Abbas, Abul K.; Lichtman, Andrew H.; Pillai, Shiv (2012). "Chapter 8: Effector Mechanisms of Humoral Immunity". Basic Immunology: Functions and Disorders of the Immune System (Fourth ed.). Philadelphia, PA: Elsevier Saunders. p. 162. ISBN 978-1-4557-0707-2.

External links

GeneReviews/NCBI/NIH/UW entry on Atypical Hemolytic-Uremic Syndrome
OMIM entries on Atypical Hemolytic-Uremic Syndrome
GeneReviews/NCBI/NIH/UW entry on Dense Deposit Disease/Membranoproliferative Glomerulonephritis Type II
Complement C3 at the US National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

UpToDate Contents

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1. 補体系の概要および臨床的評価 overview and clinical assessment of the complement system
2. C3糸球体腎症：デンスデポジット病およびC3糸球体腎炎 c3 glomerulopathies dense deposit disease and c3 glomerulonephritis
3. 補体系の遺伝性疾患 inherited disorders of the complement system
4. 補体系の後天性欠損 acquired deficiencies of the complement system
5. 補体経路 complement pathways

English Journal

Eculizumab use in kidney transplantation.

Johnson CK1, Leca N.
Current opinion in organ transplantation.Curr Opin Organ Transplant.2015 Dec;20(6):643-51. doi: 10.1097/MOT.0000000000000249.
PURPOSE OF REVIEW: Eculizumab suppresses the effector functions of the complement system and represents a therapeutic breakthrough for patients with paroxysmal nocturnal hemoglobinuria or atypical hemolytic uremic syndrome (aHUS). Safety monitoring is ongoing; so far, most notable is the expected in
PMID 26536427

Remarkable Activation of the Complement System and Aberrant Neuronal Localization of the Membrane Attack Complex in the Brain Tissues of Scrapie-Infected Rodents.

Lv Y1,2, Chen C1,2, Zhang BY1, Xiao K1, Wang J1,2, Chen LN1, Sun J1, Gao C1,2, Shi Q1,2, Dong XP3,4,5.
Molecular neurobiology.Mol Neurobiol.2015 Dec;52(3):1165-79. doi: 10.1007/s12035-014-8915-2. Epub 2014 Oct 14.
As an integral part of the innate immunity, the complement system has been reported to involve in the pathogenesis of prion diseases (PrD). However, the states of expression and activity of complement proteins in experimental models of scrapie infection are still not fully understood. Herein, the st
PMID 25311207

Complement Evasion Mediated by Enhancement of Captured Factor H: Implications for Protection of Self-Surfaces from Complement.

Herbert AP1, Makou E1, Chen ZA2, Kerr H3, Richards A4, Rappsilber J2, Barlow PN5.
Journal of immunology (Baltimore, Md. : 1950).J Immunol.2015 Nov 15;195(10):4986-98. doi: 10.4049/jimmunol.1501388. Epub 2015 Oct 12.
In an attempt to evade annihilation by the vertebrate complement system, many microbes capture factor H (FH), the key soluble complement-regulating protein in human plasma. However, FH is normally an active complement suppressor exclusively on self-surfaces and this selective action of FH is pivotal
PMID 26459349

Japanese Journal

膜性腎症症例の中で超微形態にて糸球体基底膜にうねりを示す20例の臨床病理学的検討

自治医科大学紀要 = Jichi Medical University Journal 37, 11-18, 2015-03
NAID 120005610097

TMAの診断と治療

臨床血液 56(10), 2092-2099, 2015
NAID 130005103278

自己免疫疾患患者における免疫抑制療法と感染予防法の最適化

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NAID 130005100630

「補体C3」

C3
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	4ONT, 1C3D, 1GHQ, 1W2S, 2A73, 2A74, 2GOX, 2I07, 2ICE, 2ICF, 2NOJ, 2QKI, 2WII, 2WIN, 2WY7, 2WY8, 2XQW, 2XWB, 2XWJ, 3D5R, 3D5S, 3G6J, 3L3O, 3L5N, 3NMS, 3OED, 3OHX, 3OXU, 3RJ3, 3T4A, 4HW5, 4HWJ, 4I6O, 4M76, 4ZH1, 5FOB, 5FO9, 5FO7, 5FO8, 5FOA
Identifiers
Aliases	C3, AHUS5, ARMD9, ASP, C3a, C3b, CPAMD1, HEL-S-62p, complement component 3
External IDs	OMIM: 120700 MGI: 88227 HomoloGene: 68031 GeneCards: C3
Genetically Related Diseases
	age related macular degeneration
Gene ontology
Molecular function	• C5L2 anaphylatoxin chemotactic receptor binding; • protein binding; • endopeptidase inhibitor activity; • receptor binding; • serine-type endopeptidase activity;
Cellular component	• blood microparticle; • plasma membrane; • extracellular space; • extracellular region; • extracellular exosome;
Biological process	• G-protein coupled receptor signaling pathway; • positive regulation of phagocytosis; • positive regulation of protein phosphorylation; • complement activation, alternative pathway; • lipid metabolic process; • immune system process; • complement activation; • positive regulation of lipid storage; • positive regulation of type IIa hypersensitivity; • fatty acid metabolic process; • positive regulation of angiogenesis; • positive regulation of G-protein coupled receptor protein signaling pathway; • positive regulation of apoptotic cell clearance; • immune response; • complement activation, classical pathway; • regulation of immune response; • positive regulation of activation of membrane attack complex; • positive regulation of vascular endothelial growth factor production; • inflammatory response; • positive regulation of glucose transport; • regulation of complement activation; • signal transduction; • negative regulation of endopeptidase activity; • regulation of triglyceride biosynthetic process; • proteolysis; • innate immune response;
	Sources:Amigo / QuickGO
Orthologs
	718
	12266
	ENSG00000125730
	ENSMUSG00000024164
	P01024
	P01027
	NM_000064
	NM_009778
	NP_000055.2
	NP_033908.2
	Wikidata
View/Edit Human	View/Edit Mouse

　　[★]

英: complement C3、C3 complement、complement 3
関: 補体第三成分

「C3 complement」

　　[★]

補体C3、補体第三成分

関: complement 3、complement C3

「補体第三成分」

　　[★]

英: complement C3、C3 complement、complement 3
関: 補体C3

「complement C3 nephritic factor」

　　[★]

補体C3腎炎因子、補体C3腎炎性因子

「alternative pathway complement C3-C5 convertase」

　　[★]

代替経路補体C3-C5転換酵素

「complement C3b」

　　[★]

補体C3b

関: C3b complement

「alternative pathway complement C3 convertase」

　　[★]

代替経路補体C3転換酵素

「C3」

　　[★]

同: 補体第三成分 third component of complement
関: 補体、C3a, C3b

補体の一つ
C3は血清中に最も多く存在する
C3 convertaseによってC3aとC3bを生じる
C3 convertase活性を持つのは２つ

1. C4bC2a (古典的経路)

2. C3bBbP (別経路)

「C」

　　[★]

炭素 carbon
シトシン cytosine
システイン cysteine
シチジン
セルシウス度摂氏 degrees Celsius degree C
産婦人科領域で「子宮外頚子宮外頚部 exocervix」を表現するための略語

「complement」

　　[★]

n.

補って完全にする物、補完物。(免疫)補体
(必要な)全数、善良

vt.

補って完全にする。補完する。～の補足となる。

[1] "Diseases that are genetically associated with C3 view/edit references on wikidata".

[2] "Human PubMed Reference:".

[3] "Mouse PubMed Reference:".

[pmid2579379-4] Bruijn MH, Fey GH (Feb 1985). "Human complement component C3: cDNA coding sequence and derived primary structure". Proceedings of the National Academy of Sciences of the United States of America. 82 (3): 708–12. doi:10.1073/pnas.82.3.708. PMC 397115. PMID 2579379.

[entrez_718-5] "Entrez Gene: C3 complement component 3".

[pmid11414361-6] Sahu A, Lambris JD (Apr 2001). "Structure and biology of complement protein C3, a connecting link between innate and acquired immunity". Immunological Reviews. 180: 35–48. doi:10.1034/j.1600-065X.2001.1800103.x. PMID 11414361.

[pmid768477-7] Lachmann P (Dec 1975). "Genetics of the complement system". Journal of Medical Genetics. 12 (4): 372–7. doi:10.1136/jmg.12.4.372. PMC 1013316. PMID 768477.

[pmid11813855-8] Matsuyama W, Nakagawa M, Takashima H, Muranaga F, Sano Y, Osame M (Dec 2001). "Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters". Internal Medicine. 40 (12): 1254–8. doi:10.2169/internalmedicine.40.1254. PMID 11813855.

[urlwww.ebi.ac.uk-9] "Advanced search results for UniProt accession P01024". Protein Data Bank in Europe (PDBe). European Bioinformatics Institute. Retrieved 2010-12-27.

[pmid16177781-10] Janssen BJ, Huizinga EG, Raaijmakers HC, Roos A, Daha MR, Nilsson-Ekdahl K, Nilsson B, Gros P (Sep 2005). "Structures of complement component C3 provide insights into the function and evolution of immunity". Nature. 437 (7058): 505–11. doi:10.1038/nature04005. PMID 16177781.

[pmid17051160-11] Janssen BJ, Christodoulidou A, McCarthy A, Lambris JD, Gros P (Nov 2006). "Structure of C3b reveals conformational changes that underlie complement activity". Nature. 444 (7116): 213–6. doi:10.1038/nature05172. PMID 17051160.

[pmid17051150-12] Wiesmann C, Katschke KJ, Yin J, Helmy KY, Steffek M, Fairbrother WJ, McCallum SA, Embuscado L, DeForge L, Hass PE, van Lookeren Campagne M (Nov 2006). "Structure of C3b in complex with CRIg gives insights into regulation of complement activation". Nature. 444 (7116): 217–20. doi:10.1038/nature05263. PMID 17051150.

[pmid16831446-13] Fredslund F, Jenner L, Husted LB, Nyborg J, Andersen GR, Sottrup-Jensen L (Aug 2006). "The structure of bovine complement component 3 reveals the basis for thioester function". Journal of Molecular Biology. 361 (1): 115–27. doi:10.1016/j.jmb.2006.06.009. PMID 16831446.

[14] ttps://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=167&contentid=complement_c3_blood

[pmid9291131-15] Soames CJ, Sim RB (Sep 1997). "Interactions between human complement components factor H, factor I and C3b". The Biochemical Journal. 326 ( Pt 2): 553–61. PMC 1218704. PMID 9291131.

[pmid11367533-16] Jokiranta TS, Westin J, Nilsson UR, Nilsson B, Hellwage J, Löfås S, Gordon DL, Ekdahl KN, Meri S (Mar 2001). "Complement C3b interactions studied with surface plasmon resonance technique". International Immunopharmacology. 1 (3): 495–506. doi:10.1016/S1567-5769(00)00042-4. PMID 11367533.

[17] Abbas, Abul K.; Lichtman, Andrew H.; Pillai, Shiv (2012). "Chapter 8: Effector Mechanisms of Humoral Immunity". Basic Immunology: Functions and Disorders of the Immune System (Fourth ed.). Philadelphia, PA: Elsevier Saunders. p. 162. ISBN 978-1-4557-0707-2.

v t e Acute-phase proteins

Amyloid	SAP SAA

Other positive	Alpha 1-antichymotrypsin Alpha 1-antitrypsin Alpha 2-macroglobulin C-reactive protein Ceruloplasmin C3 Ferritin Fibrin Haptoglobin Hemopexin Orosomucoid

Negative	Serum albumin Transferrin

リンク元	「補体C3」「C3 complement」「補体第三成分」
拡張検索	「complement C3 nephritic factor」「alternative pathway complement C3-C5 convertase」「complement C3b」「alternative pathway complement C3 convertase」
関連記事	「C3」「C」「complement」

匿名

検索

案内

案内

complement C3