decay-accelerating factor

崩壊促進因子 DAF CD55

WordNet

1. undergo decay or decomposition; "The body started to decay and needed to be cremated"
2. fall into decay or ruin; "The unoccupied house started to decay" (同)crumble, dilapidate
3. a gradual decrease; as of stored charge or current (同)decline
4. the organic phenomenon of rotting (同)decomposition
5. the spontaneous disintegration of a radioactive substance along with the emission of ionizing radiation (同)radioactive decay, disintegration
6. an inferior state resulting from the process of decaying; "the corpse was in an advanced state of decay"; "the house had fallen into a serious state of decay and disrepair"
7. the process of gradually becoming inferior
8. be a contributing factor; "make things factor into a companys profitability"
9. any of the numbers (or symbols) that form a product when multiplied together
10. an independent variable in statistics
11. anything that contributes causally to a result; "a number of factors determined the outcome"
12. consider as relevant when making a decision; "You must factor in the recent developments" (同)factor in, factor out
13. resolve into factors; "a quantum computer can factor the number 15" (同)factor in, factor out
14. an event known to have happened or something known to have existed; "your fears have no basis in fact"; "how much of the story is fact and how much fiction is hard to tell"
15. a concept whose truth can be proved; "scientific hypotheses are not facts"
16. a piece of information about circumstances that exist or events that have occurred; "first you must collect all the facts of the case"
17. a statement or assertion of verified information about something that is the case or has happened; "he supported his argument with an impressive array of facts"
18. damaged by decay; hence unsound and useless; "rotten floor boards"; "rotted beams"; "a decayed foundation" (同)rotten, rotted

PrepTutorEJDIC

1. 『腐る』,朽ちる / 〈繁栄・健康などが〉『衰える』,衰退する;〈活力などが〉低下する / 〈他〉 / …'を'『腐らせる』 / 『腐敗』;腐朽 / 『衰微』,衰え / (放射性物質の)自然崩壊
2. (…の)『要因』,(…を生み出す)要素《+『in』+『名』(do『ing』)》 / 囲数,約数 / 代理人,《おもに英》仲買人 / =factorize
3. 〈C〉『事実』,実際にある(あった)事 / 〈U〉真相,真実(truth) / 《the~》(法律用語で)犯行

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/05/29 22:15:11」(JST)

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[Wiki en表示]

Decay-accelerating factor
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1H03, 1H04, 1H2P, 1H2Q, 1M11, 1NWV, 1OJV, 1OJW, 1OJY, 1OK1, 1OK2, 1OK3, 1OK9, 1UOT, 1UPN, 2C8I, 2QZD, 2QZF, 2QZH, 3IYP, 3J24
Identifiers
Aliases	CD55, CR, CROM, DAF, TC
External IDs	OMIM: 125240 MGI: 104849 HomoloGene: 479 GeneCards: 1604
Gene ontology Molecular function • virus receptor activity • protein binding • lipid binding Cellular component • cytosol • membrane • Golgi membrane • plasma membrane • integral component of plasma membrane • transport vesicle • extracellular region • cell surface • membrane raft • anchored component of membrane • extracellular exosome • endoplasmic reticulum-Golgi intermediate compartment membrane Biological process • cellular protein metabolic process • regulation of lipopolysaccharide-mediated signaling pathway • immune system process • post-translational protein modification • positive regulation of cytosolic calcium ion concentration • positive regulation of CD4-positive, alpha-beta T cell proliferation • protein N-linked glycosylation via asparagine • respiratory burst • ER to Golgi vesicle-mediated transport • positive regulation of CD4-positive, alpha-beta T cell activation • CD4-positive, alpha-beta T cell cytokine production • complement activation, classical pathway • viral entry into host cell • membrane organization • innate immune response • viral process • regulation of complement activation • negative regulation of complement activation Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	1604	13137
Ensembl	ENSG00000196352	ENSMUSG00000026401
UniProt	P08174	Q61476
RefSeq (mRNA)	NM_000574 NM_001114543 NM_001114544 NM_001114752 NM_001300902 NM_001300903 NM_001300904	NM_007827 NM_001317361
RefSeq (protein)	NP_000565.1 NP_001108224.1 NP_001287832.1 NP_001287833.1	NP_001304290.1 NP_031853.2
Location (UCSC)	Chr 1: 207.32 – 207.39 Mb	Chr 1: 130.39 – 130.42 Mb
PubMed search	[1]	[2]
Wikidata
View/Edit Human		View/Edit Mouse

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

DAF regulates the complement system on the cell surface. It recognizes C4b and C3b fragments that are created during C4 (classical complement pathway and lectin pathway) and C3 (alternate complement pathway) activation. Interaction of DAF with cell-associated C4b of the classical and lectin pathways interferes with the conversion of C2 to C2a, thereby preventing formation of the C4b2a C3 convertase, and interaction of DAF with C3b of the alternative pathway interferes with the conversion of factor B to Bb by factor D, thereby preventing formation of the C3bBb C3 convertase of the alternative pathway. Thus, by limiting the amplification convertases of the complement cascade, DAF indirectly blocks the formation of the membrane attack complex.

This glycoprotein is broadly distributed among hematopoietic and non-hematopoietic cells. It is a determinant for the Cromer blood group system.

Structure

DAF is a 70 kDa membrane protein that attaches to cell membrane via a glycophosphatidylinositol (GPI) anchor.

DAF contains four complement control protein (CCP) repeats with a single N-linked glycan positioned between CCP1 and CCP2. CCP2, CCP3, CCP4 and three consecutive lysine residues in a positively charged pocket between CCP2 and CCP3 are involved in its inhibition of the alternate complement pathway. CCP2 and CCP3 alone are involved in its inhibition of the classical pathway.

Pathology

Paroxysmal nocturnal hemoglobinuria

Because DAF is a GPI-anchored protein, its expression is reduced in persons with mutations that reduce GPI levels such as those with paroxysmal nocturnal hemoglobinuria; in that disorder, red blood cells with very low levels of DAF and CD59 undergo complement-mediated hemolysis.

Infectious diseases

DAF is used as a receptor by some coxsackieviruses and other enteroviruses. Recombinant soluble DAF-Fc has been tested in mice as an anti-enterovirus therapy for heart damage; however, the human enterovirus that was tested binds much more strongly to human DAF than to mouse or rat DAF. Echoviruses and coxsackie B viruses that use human decay-accelerating factor (DAF) as a receptor do not bind the rodent analogues of DAF. and DAF-Fc has yet to be tested in humans.

See also

• List of human clusters of differentiation
• CD59

References

Further reading

• Selinka HC, Wolde A, Sauter M, et al. (2004). "Virus-receptor interactions of coxsackie B viruses and their putative influence on cardiotropism.". Med. Microbiol. Immunol. 193 (2-3): 127–31. doi:10.1007/s00430-003-0193-y. PMID 12920584. 
• Mikesch JH, Schier K, Roetger A, et al. (2007). "The expression and action of decay-accelerating factor (CD55) in human malignancies and cancer therapy.". Cell. Oncol. 28 (5-6): 223–32. PMID 17167176. 

External links

• Decay-Accelerating Factor at the US National Library of Medicine Medical Subject Headings (MeSH)
• Cromer blood group system at BGMUT Blood Group Antigen Gene Mutation Database at NCBI, NIH

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 発作性夜間血色素尿症の病因 pathogenesis of paroxysmal nocturnal hemoglobinuria
• 2. 補体系の遺伝性疾患 inherited disorders of the complement system
• 3. 発作性夜間血色素尿症の治療および予後 treatment and prognosis of paroxysmal nocturnal hemoglobinuria
• 4. 補体系の制御因子および受容体 regulators and receptors of the complement system
• 5. 母体 - 胎児接点の免疫 immunology of the maternal fetal interface

English Journal

• The African-387 C>T TGFB1 variant is functional and associates with the ophthalmoplegic complication in juvenile myasthenia gravis.

• Nel M1, Buys JM1, Rautenbach R1, Mowla S2, Prince S3, Heckmann JM1.
• Journal of human genetics.J Hum Genet.2015 Dec 3. doi: 10.1038/jhg.2015.146. [Epub ahead of print]
• Although extraocular muscles are commonly affected by myasthenia gravis (MG) at presentation, a treatment-resistant ophthalmoplegic complication of MG (OP-MG) occurs in younger patients with African-genetic ancestry. In MG, pathogenic antibodies activate complement-mediated muscle damage and this ma
• PMID 26632886

• Enterovirus strain and type-specific differences in growth kinetics and virus-induced cell destruction in human pancreatic duct epithelial HPDE cells.

• Smura T1, Natri O2, Ylipaasto P3, Hellman M2, Al-Hello H3, Piemonti L4, Roivainen M3.
• Virus research.Virus Res.2015 Dec 2;210:188-97. doi: 10.1016/j.virusres.2015.08.003. Epub 2015 Aug 7.
• Enterovirus infections have been suspected to be involved in the development of type 1 diabetes. However, the pathogenetic mechanism of enterovirus-induced type 1 diabetes is not known. Pancreatic ductal cells are closely associated with pancreatic islets. Therefore, enterovirus infections in ductal
• PMID 26260332

• Mutational analysis of Kaposica reveals that bridging of MG2 and CUB domains of target protein is crucial for the cofactor activity of RCA proteins.

• Gautam AK1, Panse Y1, Ghosh P2, Reza MJ1, Mullick J3, Sahu A4.
• Proceedings of the National Academy of Sciences of the United States of America.Proc Natl Acad Sci U S A.2015 Oct 13;112(41):12794-9. doi: 10.1073/pnas.1506449112. Epub 2015 Sep 29.
• The complement system has evolved to annul pathogens, but its improper regulation is linked with diseases. Efficient regulation of the system is primarily provided by a family of proteins termed regulators of complement activation (RCA). The knowledge of precise structural determinants of RCA protei
• PMID 26420870

Japanese Journal

• Comparative Gene Expression Analysis in the Skeletal Muscles of Dysferlin-deficient SJL/J and A/J Mice

• Kobayashi Kinji,Izawa Takeshi,Kuwamura Mitsuru,Yamate Jyoji
• Journal of Toxicologic Pathology 24(1), 49-62, 2011
• … SJL/J mice exhibited a marked lowering of decay-accelerating factor 1/CD55 gene expression level in all studied muscles except for the heart at all ages compared with that of BALB/c mice. …
• NAID 130000675354

• 崩壊促進因子(DAF) (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)

• 根来 孝治,中野 泰子
• 日本臨床 68(-) (980), 102-104, 2010-06
• NAID 40017181716

Related Links

• Decay-accelerating factorとは - goo Wikipedia (ウィキペディア)

Complement decay-accelerating factor, also known as CD55 or DAF, is a protein that, in humans, is encoded by the CD55 gene. [1] Decay accelerating factor is a 70 kDa membrane protein that regulates the complement system on ...

• Decay accelerating factorとは - goo Wikipedia (ウィキペディア)

^Medof ME, Lublin DM, Holers VM, Ayers DJ, Getty RR, Leykam JF, Atkinson JP, Tykocinski ML (April 1987). "Cloning and characterization of cDNAs encoding the complete sequence of decay-accelerating factor of human ...

Related Pictures

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★リンクテーブル★

リンク元	「発作性夜間血色素尿症」「崩壊促進因子」「DAF」「CD55」「CD55 antigen」
関連記事	「decay」「fact」「factor」「accelerating」

「発作性夜間血色素尿症」

　　[★]

英

paroxysmal nocturnal hemoglobinuria PNH

同

発作性夜間ヘモグロビン尿症 *難病 http://www.nanbyou.or.jp/sikkan/116_2_i.htm

マルキアファーヴァ-ミケリ症候群 Marchiafava-Micheli syndrome

関

難病

• first aid step1 2006 p.187

red urine in the morning

概念

• 補体感受性の高い赤血球の存在が原因となり、血管内溶血が起こり，貧血およびヘモグロビン尿を来す慢性の後天性溶血性貧血
• 赤血球細胞膜のGPIアンカー型蛋白質のうち、補体防御膜蛋白に属する崩壊促進因子(DAF, CD55)とCD59の欠損が補体感受性亢進の原因とされる。
• 多能性幹細胞レベルでPIG-A遺伝子の異常が起こることによる後天性のクローナル疾患と考えられている。

疫学

• 100万人に1-10人

原因

• 補体活性化異常、活性化経路制御分子の欠損　→　造血幹細胞レベルの異常

• 発作性夜間血色素尿症 PNH:paroxysmal nocternal hemglobulinemia

CD55 DAF decay accelerating factor C3b,C5bの酵素阻害

CD59 HRF homologous restriction factor MAC形成阻害

=Protectin, Mac inhibitor

PIG-A GPIアンカー型蛋白質の欠損

病態

• 静脈血栓症：原因は不明。血小板膜上の補体が小胞化による補体複合体の除去を活性化する。循環中に流れ出たこのmicroparticleはホスファチジルセリンに富んでおり、血栓原性が高い。(参考1)

症状

• 貧血、黄疸、夜間の溶血発作（血中CO2濃度の上昇が原因）

• 1.貧血：息切れ、動悸
• 2.溶血：ヘモグロビン尿、ヘモジデリン尿、ときに黄疸
• 3.汎血球減少：貧血以、出血傾向、易感染性
• 4.血栓症：深部静脈血栓、腸間膜静脈血栓症による腹痛・イレウス、肝静脈血栓症(Budd-Chiari症候群)
• 5.腎不全：急激な溶血発作により起こることがある

合併症

• 再生不良性貧血
• 急性骨髄性白血病　(HIM.661によれば症例の1-2%)
• 静脈血栓
• ヘモジデローシス(長期間の輸血による臓器への鉄沈着)

検査

血算

• 汎血球減少

→　正球性正色素性貧血、慢性経過で鉄が不足し小球性低色素性貧血

• 網赤血球：増加→減少

• 好中球アルカリフォスファターゼスコア：低値(NAPスコア：低下)

血液生化学

• LDH：上昇
• ハプトグロビン：減少
• 間接ビリルビン：上昇

• 慢性の血管内溶血により鉄が欠乏　→　血清鉄↓、血清フェリチン↓

尿検査

血管内溶血によりヘム鉄は糸球体濾過されて、ヘモグロビンは尿細管で再吸収され、鉄はヘモジデリンとして尿中に排泄される。 (ポルフィリンどこいった？)

• ヘモグロビン尿

発作時に認められる

• ヘモジデリン尿

• 発作時でなくとも認められる

補体感受性亢進の検査

• ショ糖溶血試験：陽性　→　スクリーニング検査
• Ham試験：陽性　→　確定診断
• 表面抗原解析：白血球CD59、CD55(DAF,decay-accelerating factor)欠損

治療

• ほとんどの患者で生涯にわたる支持療法(suppertive care)を受ける人が多い。(HIM.661)
• 若い患者で重症のPNHであれば同種骨髄移植を提案すべき(should be offered) (HIM.661)

支持療法

• 輸血

• フィルターで白血球を除去した赤血球製剤を使用。伝統的に溶血を引き起こすwhite cell reaction(白血球に対する抗HLA抗体による抗原抗体反応、のこと？)を防止するために洗浄赤血球が用いられてきたが、これは無駄である。(HIM.661)(also see. 参考2)

• 葉酸
• 鉄剤
• 補体成分C5に対するヒト化モノクローナル抗体 エクリズマブ eclizumab
• ×糖質コルチコイド：長期にわたる使用におけるエビデンスなし

根治療法

• 骨髄移植

USMLE

• Q book p.291 19

参考

• 1. [charged] Clinical manifestations of paroxysmal nocturnal hemoglobinuria - uptodate [1]
• 2. [charged] Diagnosis and treatment of paroxysmal nocturnal hemoglobinuria - uptodate [2]

「崩壊促進因子」

　　[★]

英

decay-accelerating factor, decay accelerating factor, DAF

同

CD55抗原、CD55

関

CD59、発作性夜間血色素尿症

「DAF」

　　[★] 崩壊促進因子, decay-accelerating factor, decay accelerating factor

「CD55」

　　[★] 崩壊促進因子, DAF, decay-accelerating factor

「CD55 antigen」

　　[★]

CD55抗原

関

decay-accelerating factor

「decay」

　　[★]

• vi

• 腐食する
• 衰える、減衰する。(理)(放射性物質・素粒子・原子核が)(自然)崩壊する

• vt.

• 朽ちさせる、腐蝕する。腐敗させる

• n.

• 腐食、腐朽、腐敗。(歯の)齲蝕、むしば。腐食部、腐敗した物質
• 減衰、衰弱、衰退、老朽化。(理)(放射性物質・素粒子・原子核の)崩壊。(電流の)減少、(磁束の)減衰、(電荷などの)消失。

　 　 　 　 　 　 　 　

「fact」

　　[★]

• n.

• 事実、現状、実際、実態

関

actual、actually、in fact、in practice、indeed、practically

　 　 　 　 　

「factor」

　　[★]

• n.

• 因子、要因、要素、ファクター

関

element、elementary、factorial、parameter

　 　 　 　 　 　 　

「accelerating」

　　[★]

• adj.

• 加速度的な、促進的な

関

accelerated、acceleratingly、acceleratory、facilitatory、promotional