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衰变加速因子

衰变加速因子
已知的结构
PDB直系同源搜索: PDBe RCSB
识别号
别名CD55;, CR, CROM, DAF, TC, CD55 molecule (Cromer blood group), CHAPLE
外部IDOMIM125240 MGI104850 HomoloGene:479 GeneCardsCD55
相关疾病
protein-losing enteropathy[1]
基因位置(人类
1号染色体
染色体1号染色体[2]
1号染色体
衰变加速因子的基因位置
衰变加速因子的基因位置
基因座1q32.2起始207,321,519 bp[2]
终止207,386,804 bp[2]
RNA表达模式


查阅更多表达数据
直系同源
物种人类小鼠
Entrez
Ensembl
UniProt
mRNA​序列

NM_010016

蛋白序列

NP_000565
​NP_001108224
​NP_001287831
​NP_001287832
​NP_001287833

NP_034146
​NP_001391888
​NP_001391889

基因位置​(UCSC)Chr 1: 207.32 – 207.39 MbChr 1: 130.37 – 130.39 Mb
PubMed​查找[4][5]
维基数据
查看/编辑人类查看/编辑小鼠

衰变加速因子(Decay-accelerating factor, DAF),亦称为CD55,在人体内是一种由CD55基因编码的膜蛋白,分子量为70kD。CD55可以通过间接抑制膜攻击复合物(MAC)的形成,使细胞不被补体系统攻击[6][7]

分子结构

CD55分子量为70kD,属于膜蛋白,通过糖基磷脂酰肌醇(GPI)基团固定在膜上[6][7]

CD55含有四个补体调控蛋白英语complement control protein重复(complement control protein repeat, CCP重复)。其中,CCP1与CCP2之间有一个N-糖基化位点,CCP2、CCP3、CCP4以及CCP2、CCP3之间的三个连续的赖氨酸残基形成的正电荷口袋区参与抑制补体系统的替代途径英语Alternative pathway。而CCP2和CCP3则可单独参与抑制经典途径英语Classical pathway[8]

作用机理

CD55的作用是通过间接阻止膜攻击复合物(MAC)的形成,防止细胞被补体系统攻击。其作用是识别补体系统产生的C3b、C4b。在补体系统经典途径英语Classical pathway凝集素途径中,CD55与C4b发生相互作用后,能抑制C2英语C2转化为C2b,进而抑制C4b2a形成C3转化酶英语C3-convertase。而在替代途径英语Alternative pathway中,与C3b发生相互作用后CD55可以干扰D因子英语Complement factor DB因子英语Complement factor B转化为Bb。上述两种机制都可以阻止膜攻击复合物的最终形成,达到抑制补体系统的作用[8][7]

临床应用

因为CD55是一种通过GPI锚定于膜上的蛋白,故能影响GPI基团的基因突变会引起红细胞膜上的CD55分子和CD59分子数量减少,会引起补体系统攻击红细胞,导致红细胞破裂。这可能会引发阵发性夜间血红素尿症[9]

克沙奇病毒肠道病毒即是以CD55作为受体[10]

参见

参考

  1. ^ 與衰變加速因子相關的疾病;在維基數據上查看/編輯參考. 
  2. ^ 2.0 2.1 2.2 GRCh38: Ensembl release 89: ENSG00000196352 - Ensembl, May 2017
  3. ^ 3.0 3.1 3.2 GRCm38: Ensembl release 89: ENSMUSG00000026399 - Ensembl, May 2017
  4. ^ Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
  5. ^ Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
  6. ^ 6.0 6.1 Medof ME, Lublin DM, Holers VM, Ayers DJ, Getty RR, Leykam JF, Atkinson JP, Tykocinski ML. Cloning and characterization of cDNAs encoding the complete sequence of decay-accelerating factor of human complement. Proc. Natl. Acad. Sci. U.S.A. April 1987, 84 (7): 2007–11. PMC 304572可免费查阅. PMID 2436222. doi:10.1073/pnas.84.7.2007. 
  7. ^ 7.0 7.1 7.2 CD55. UNIPROT. [2018-05-01]. (原始内容存档于2020-11-06). 
  8. ^ 8.0 8.1 Brodbeck WG, Kuttner-Kondo L, Mold C, Medof ME. Structure/function studies of human decay-accelerating factor. immunology. Sep 2000, 101 (1): 104–11. PMC 2327052可免费查阅. PMID 11012760. doi:10.1046/j.1365-2567.2000.00086.x. 
  9. ^ Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005, 106 (12): 3699–709 [2018-05-01]. PMC 1895106可免费查阅. PMID 16051736. doi:10.1182/blood-2005-04-1717. (原始内容存档于2009-09-18). 
  10. ^ Karnauchow TM, Tolson DL, Harrison BA, Altman E, Lublin DM, Dimock K. The HeLa cell receptor for enterovirus 70 is decay-accelerating factor (CD55). J. Virol. August 1996, 70 (8): 5143–52. PMC 190469可免费查阅. PMID 8764022. 
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衰变加速因子
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