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Human genes for the alpha and beta chains of complement C4b-binding protein are closely linked in a head-to-tail arrangement.

作者信息

Pardo-Manuel F, Rey-Campos J, Hillarp A, Dahlbäck B, Rodriguez de Cordoba S

机构信息

Unidad de Immunologia, Centro de Investigaciones Biologicas (Consejo Superior de Investigaciones Cientificas), Madrid, Spain.

出版信息

Proc Natl Acad Sci U S A. 1990 Jun;87(12):4529-32. doi: 10.1073/pnas.87.12.4529.

DOI:10.1073/pnas.87.12.4529
PMID:2352933
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC54149/
Abstract

C4b-binding protein (C4BP) is an important component in the regulation of the complement system and also binds the anticoagulant vitamin K-dependent protein S. These activities are performed by distinct, although structurally related, polypeptides of 70 kDa (alpha chain) and 45 kDa (beta chain), respectively. In this report we have investigated the genetic relationships between these polypeptides. Using pulsed field gel electrophoresis analysis we demonstrate that the genes coding for the alpha (C4BP alpha) and beta (C4BP beta) chains are closely linked within the regulator of complement activation gene cluster. In addition, we have determined that the 3' end of the C4BP beta gene lies 3.5-5 kilobases from the 5' end of the C4BP alpha gene. These findings support the concept that the C4BP alpha and C4BP beta genes are the result of a gene duplication event.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1239/54149/15766acd9a5f/pnas01037-0127-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1239/54149/013e91dc3e5c/pnas01037-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1239/54149/15766acd9a5f/pnas01037-0127-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1239/54149/013e91dc3e5c/pnas01037-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1239/54149/15766acd9a5f/pnas01037-0127-b.jpg

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1
Human genes for the alpha and beta chains of complement C4b-binding protein are closely linked in a head-to-tail arrangement.
Proc Natl Acad Sci U S A. 1990 Jun;87(12):4529-32. doi: 10.1073/pnas.87.12.4529.
2
Molecular cloning of rat C4b binding protein alpha- and beta-chains: structural and functional relationships among human, bovine, rabbit, mouse, and rat proteins.大鼠C4b结合蛋白α链和β链的分子克隆:人、牛、兔、小鼠和大鼠蛋白质之间的结构与功能关系
J Immunol. 1997 Feb 1;158(3):1315-23.
3
High affinity binding of human vitamin K-dependent protein S to a truncated recombinant beta-chain of C4b-binding protein expressed in Escherichia coli.人维生素K依赖性蛋白S与在大肠杆菌中表达的C4b结合蛋白截短重组β链的高亲和力结合。
J Biol Chem. 1993 Feb 15;268(5):3033-6.
4
Cloning of cDNA coding for the beta chain of human complement component C4b-binding protein: sequence homology with the alpha chain.
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5
Molecular analysis of the beta-chain of human C4b-binding protein.
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Isoforms of human C4b-binding protein. I. Molecular basis for the C4BP isoform pattern and its variations in human plasma.
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Isoforms of human C4b-binding protein. II. Differential modulation of the C4BPA and C4BPB genes by acute phase cytokines.人C4b结合蛋白的亚型。II. 急性期细胞因子对C4BPA和C4BPB基因的差异调节。
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Expression and characterization of a recombinant C4b-binding protein lacking the beta-chain.缺乏β链的重组C4b结合蛋白的表达与鉴定
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The gene coding for the beta-chain of C4b-binding protein (C4BPB) has become a pseudogene in the mouse.编码C4b结合蛋白β链(C4BPB)的基因在小鼠中已成为假基因。
Genomics. 1994 Jun;21(3):501-9. doi: 10.1006/geno.1994.1308.
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Genes for C4b-binding protein alpha- and beta-chains (C4BPA and C4BPB) are located on chromosome 1, band 1q32, in humans and on chromosome 13 in rats.
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本文引用的文献

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Purification and characterization of a macromolecular weight cofactor for C3b-inactivator, C4bC3bINA-cofactor, of human plasma.人血浆中C3b灭活因子C4bC3bINA辅助因子(一种高分子量辅助因子)的纯化与特性分析
Mol Immunol. 1980 Nov;17(11):1365-72. doi: 10.1016/0161-5890(80)90005-x.
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A technique for radiolabeling DNA restriction endonuclease fragments to high specific activity.一种将DNA限制性内切酶片段放射性标记至高比活度的技术。
Anal Biochem. 1983 Jul 1;132(1):6-13. doi: 10.1016/0003-2697(83)90418-9.
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Visualization of human C4b-binding protein and its complexes with vitamin K-dependent protein S and complement protein C4b.
西班牙人群中C4b结合蛋白基因多态性与非典型溶血性尿毒症综合征之间无关联。
Clin Exp Immunol. 2009 Jan;155(1):59-64. doi: 10.1111/j.1365-2249.2008.03798.x.
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Genetic determinants of variation in the plasma levels of the C4b-binding protein (C4BP) in Spanish families.西班牙家庭中C4b结合蛋白(C4BP)血浆水平变异的遗传决定因素。
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Complement biosynthesis by mononuclear phagocytes.单核吞噬细胞的补体生物合成
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C4BPAL2: a second duplication of the C4BPA gene in the human RCA gene cluster.
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7
Expression of the human gene coding for the alpha-chain of C4b-binding protein, C4BPA, is controlled by an HNF1-dependent hepatic-specific promoter.编码C4b结合蛋白α链(C4BPA)的人类基因的表达受HNF1依赖性肝脏特异性启动子的控制。
Biochem J. 1995 Jun 1;308 ( Pt 2)(Pt 2):613-21. doi: 10.1042/bj3080613.
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The human factor H-related gene 2 (FHR2): structure and linkage to the coagulation factor XIIIb gene.
Immunogenetics. 1995;42(4):268-74. doi: 10.1007/BF00176444.
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Structure of the gene coding for the alpha polypeptide chain of the human complement component C4b-binding protein.编码人补体成分C4b结合蛋白α多肽链的基因结构。
J Exp Med. 1991 May 1;173(5):1073-82. doi: 10.1084/jem.173.5.1073.
10
The interaction between complement component C4b-binding protein and the vitamin K-dependent protein S forms a link between blood coagulation and the complement system.补体成分C4b结合蛋白与维生素K依赖蛋白S之间的相互作用在血液凝固和补体系统之间形成了一种联系。
Biochem J. 1991 Aug 1;277 ( Pt 3)(Pt 3):581-92. doi: 10.1042/bj2770581.
人C4b结合蛋白及其与维生素K依赖性蛋白S和补体蛋白C4b复合物的可视化。
Proc Natl Acad Sci U S A. 1983 Jun;80(11):3461-5. doi: 10.1073/pnas.80.11.3461.
4
Molecular cloning and characterization of the cDNA coding for C4b-binding protein, a regulatory protein of the classical pathway of the human complement system.人补体系统经典途径调节蛋白C4b结合蛋白编码cDNA的分子克隆与特性分析
Biochem J. 1985 Aug 15;230(1):133-41. doi: 10.1042/bj2300133.
5
A complement receptor locus: genes encoding C3b/C4b receptor and C3d/Epstein-Barr virus receptor map to 1q32.一个补体受体基因座:编码C3b/C4b受体和C3d/爱泼斯坦-巴尔病毒受体的基因定位于1q32。
J Immunol. 1987 Jan 1;138(1):312-5.
6
cDNA structure of murine C4b-binding protein, a regulatory component of the serum complement system.小鼠C4b结合蛋白的cDNA结构,血清补体系统的一种调节成分。
Biochemistry. 1987 Jul 28;26(15):4668-74. doi: 10.1021/bi00389a012.
7
Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements.人补体蛋白B因子基因的细胞特异性表达:两个不同5'侧翼元件作用的证据
Cell. 1987 Jan 30;48(2):331-42. doi: 10.1016/0092-8674(87)90436-3.
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Separation of large DNA molecules by contour-clamped homogeneous electric fields.通过轮廓钳位均匀电场分离大型DNA分子。
Science. 1986 Dec 19;234(4783):1582-5. doi: 10.1126/science.3538420.
9
Human genes for three complement components that regulate the activation of C3 are tightly linked.调控C3激活的三种补体成分的人类基因紧密连锁。
J Exp Med. 1985 May 1;161(5):1189-95. doi: 10.1084/jem.161.5.1189.
10
Molecular analysis of the Duchenne muscular dystrophy region using pulsed field gel electrophoresis.使用脉冲场凝胶电泳对杜兴氏肌营养不良症区域进行分子分析。
Cell. 1987 Jan 30;48(2):351-7. doi: 10.1016/0092-8674(87)90438-7.