Structure, function and molecular genetics of human and murine C1r

Immunobiology

Volumn 205, Issue 4-5, 2002, Pages 365-382

  Arlaud, G J ^a   Gaboriaud, C ^a   Garnier, G ^b   Circolo, A ^b   Thielens, N M ^a   Budayova Spano, M ^a   Fontecilla Camps, J C ^a   Volanakis, J E ^b  

^a INSTITUT DE BIOLOGIE STRUCTURALE   (France)

^b BIOMEDICAL SCIENCES RESEARCH CENTER ALEXANDER FLEMING   (Greece)

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENT COMPONENT C1; COMPLEMENT COMPONENT C1R; COMPLEMENT COMPONENT C1S; ISOPROTEIN; MANNAN BINDING LECTIN; SERINE PROTEINASE;

CATALYSIS; COMPLEMENT ACTIVATION; CONTROLLED STUDY; HUMAN; MOLECULAR GENETICS; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN STRUCTURE; REVIEW; SEQUENCE HOMOLOGY; STRUCTURE ANALYSIS; THREE DIMENSIONAL IMAGING; TISSUE SPECIFICITY;

EID: 0036748155     PISSN: 01712985     EISSN: None     Source Type: Journal    
DOI: 10.1078/0171-2985-00139     Document Type: Article

References (78)

1. BORK, P., and A. BAIROCH. 1995. Extracellular protein modules: a proposed nomenclature. Trends Biochem. Sci. 20: C03.
2. COOPER, N. R. 1985. The classical pathway of complement: activation and regulation of the first complement component. Adv. Immunol. 37: 151.
3. SCHUMAKER, V. N., P. ZAVODSZKY, and P. H. POON. 1987. Activation of the first component of complement. Annu. Rev. Immunol. 5: 21.
4. ARLAUD, G. J., C. GABORIAUD, N. M. THIELENS, V. ROSSI, B. BERSCH, J.-F. HERNANDEZ, and J. C. FONTECILLA-CAMPS. 2001. Structural biology of C1: dissection of a complex molecular machinery. Immunol. Rev. 180: 136.
5. ARLAUD, G. J., J. E. VOLANAKIS, N. M. THIELENS, S. V. L. NARAYANA, V. ROSSI, and Y. Xu. 1998. The atypical serine proteases; of the complement system. Adv. Immunol. 69: 249.
6. LACROIX, M. B., C. EBEL, J. KARDOS, J. DOBO, P. GAL, P. ZAVODSZKY, G. J. ARLAUD, and N. M. THIELENS. 2001. Assembly and enzymatic properties of the catalytic domain of human complement protease C1r. J. Biol. Chem. 276: 36233.
7. KARDOS, J., P. GAL, L. SZILAGYI, N. M. THIELENS, K. SZILAGYI, Z. LORINCZ, P. KULCSAR, L. GRAF, G. J. ARLAUD, and P. ZAVODSZKY. 2001. The role of individual domains in the structure and function of the catalytic region of a modular serine protease, C1r. J. Immunol. 167: 5202.
8. BUDAYOVA-SPANO, M., M. LACROIX, N. M. THIELENS, G. J. ARLAUD, J. C. FONTECILLA-CAMPS, and C. GABORIAUD. 2002. The crystal structure of the zymogen catalytic domain of complement protease C1r reveals that a disruptive mechanical stress is required to trigger activation of the C1 complex. EMBO J. 21: 231.
9. GARNIER G., A. CIRCOLO, and J. E. VOLANAKIS. 2001. A novel murine complement-related gene encoding a C1r-like protein. Mol. Immunol. 38: 92 (abstract).
10. GARNIER, G., A. CIRCOLO, Y. XU, and J. E. VOLANAKIS. 2002. Complement C1r and C1s genes are duplicated in the mouse. Differential expression generates alternative isomorphs in the liver and the male reproductive system (submitted for publication).
11. BORK, P., and G. BECKMANN. 1993. The CUB domain. A widespread module in developmentally regulated proteins. J. Mol. Biol. 231: 539.
12. CAMPBELL, I. D., and P. BORK. 1993. Epidermal growth factor-like modules. Curr. Opin. Struct. Biol. 3: 385.
13. REID, K. B. M., D. R. BENTLEY, R. D. CAMPBELL, L. P. CHUNG, R. B. Sim, T. KRISTENSEN, and B. F. TACK. 1986. Complement system proteins which interact with C3b and C4b. A superfamily of structurally related proteins. Immunol. Today 7: 230.
14. ARLAUD, G. J., and J. GAGNON. 1983. Complete amino acid sequence of the catalytic chain of human complement subcomponent C1r. Biochem. 22: 1758.
15. JOURNET, A., and M. TOSI. 1986. Cloning and sequencing of full-length cDNA encoding the precursor of human complement component C1r. Biochem. J. 240: 783.
16. LEYTUS, S. P., K. KURACHI, K. S. SAKARIASSEN, and E. W. DAVIE. 1986. Nucleotide sequence of the cDNA coding for human complement C1r. Biochem. 25: 4855.
17. ARLAUD, G. J., A. C. WILLIS, and J. GAGNON. 1987. Complete amino acid sequence of the A chain of human complement-classical-pathway enzyme C1r. Biochem. J. 241: 711.
18. SATO, T., Y. ENDO, M. MATSUSHITA, and T. FUJITA. 1994. Molecular characterization of a novel serine protease involved in activation of the complement system by mannose-binding protein. Int. Immunol. 6: 665.
19. THIEL, S., T. VORUP-JENSEN, C. M. STOVER, W. SCHWAEBLE, S. B. LAURSEN, K. POULSEN, A. C. WILLIS, P. EGGLETON, S. HANSEN, U. HOLMSKOV, K. B. M. REID, and J. C. JENSENIUS. 1997. A second serine protease associated with mannan-binding lectin that activates complement. Nature 386: 506.
20. DAHL, M. R., S. THIEL, M. MATSUSHITA, T. FUJITA, A. C. WILLIS, T. CHRISTENSEN, T. VORUP-JENSEN, and J. C. JENSENIUS. 2001. MASP-3 and its association with distinct complexes of the mannan-binding lectin complement activation pathway. Immunity 15: 127.
21. BORK, P. 1991. Complement components C1r/C1s, bone morphogenetic protein-1 and Xenopus laevis developmentally regulated protein UVS.2 share common repeats. FEBS Lett. 282: 9.
22. ROMERO, A., M. J. ROMAO, P. F. VARELA, I. KÖLLN, J. M. DIAS, A. L. CARVALHO, L. SANZ, E. TÖPFER-PETERSEN, and J. J. CALVETE. 1997. The crystal structure of two spermadhesins reveal the CUB domain fold. Nature Struct. Biol. 4: 783.
23. VARELA, P. F., A. ROMERO, L. SANZ, M. J. ROMAO, E. TÖPFER-PETERSEN, and J. J. CALVETE. 1997. The 2.4 Å resolution crystal structure of boar seminal plasma PSP-I/PSP-II: a zona pellucida-binding glycoprotein heterodimer of the spermadhesin family built by a CUB domain architecture. J. Mol. Biol. 274: 635.
24. ROMAO, M. J., I. KÖLLN, J. M. DIAS, A. L. CARVALHO, A. ROMERO, P. F. VARELA, L. SANZ, E. TÖPFER-PETERSEN, and J. J. CALVETE. 1997. Crystal structure of acidic seminal fluid protein (aSFP) at 1.9 Å resolution: a bovine polypeptide ofthe spermadhesin family. J. Mol. Biol. 274: 650.
25. ARLAUD, G. J., A. VAN DORSSELAER, M. BELL, A. MANCINI, C. AUDE, and J. GAGNON. 1987. Identification of erythro-β-hydroxyasparagine in the EGF-like domain of human C1r. FEBS Lett. 222: 129.
26. THIELENS, N. M., A. VAN DORSSELAER, J. GAGNON, and G. J. ARLAUD. 1990. Chemical and functional characterization of a fragment of C1s containing the epidermal growth factor homology region. Biochem. 29: 3570.
27. HERNANDEZ, J.-F., B. BERSCH, Y. PÉTILLOT, J. GAGNON, and G. J. ARLAUD. 1997. Chemical synthesis and characterization of the epidermal growth factor-like module of human complement protease C1r. J. Peptide Res. 49: 221.
28. BERSCH, B., J.-F. HERNANDEZ, D. MARION, and G. J. ARLAUD. 1998. Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, an atypical member of the EGF family. Biochem. 37: 1204.
29. KAMBOH, M. I., and R. E. FERRELL. 1986. Genetic studies of low abundance human plasma proteins. III. Polymorphism of the C1r subcomponent of the first complement component. Am. J. Hum. Genet. 39: 826.
30. BORK, P., A. K. DOWNING, B. KIEFFER, and I. D. CAMPBELL. 1996. Structure and distribution of modules in extracellular proteins. Quart. Rev. Biophys. 29: 119.
31. GABORIAUD, C., V. ROSSI, I. BALLY, G. J. ARLAUD, and J. C. FONTECILLA-CAMPS. 2000. Crystal structure of the catalytic domain of human complement C1s: a serine protease with a handle. EMBO J. 19: 1755.
32. WILES, A. P., G. SHAW, J. BRIGHT, A. PERCZEL, I. D. CAMPBELL, and P. N. BARLOW. 1997. NMR studies of a viral protein that mimics the regulators of complement activation. J. Mol. Biol. 272: 253.
33. ARLAUD, G. J., and J. GAGNON. 1981. C1r and C1s subcomponents of human complement: two serine proteases lacking the "histidine loop" disulfide bridge. Biosci. Rep. 1: 779.
34. LACROIX, M., V. Rossi, C. GABORIAUD, S. CHEVALLIER, A JAQUINOD, N. M. THIELENS, J. GAGNON, and G. J. ARLAUD. 1997. Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling. Biochem. 36; 6270.
35. PERONA, J. J., and C. S. CRAIK. 1997. Evolutionary divergence of substrate specificity within the chymotrypsin-like serine protease fold. J. Biol. Chem. 272: 29987.
36. JING, H., K. J. MACON, D. MOORE, L. J. DELUCAS, J. E. VOLANAKIS, and S. V. L. NARAYANA. 1999. Structural basis of profactor D activation: from a highly flexible zymogen to a novel self-inhibited serine protease, complement factor D. EMBO J. 18: 804.
37. GABORIAUD, C., V. Rossi, J. C. FONTECILLA-CAMPS, and G. J. ARLAUD. 1998. Evolutionary conserved rigid module-domain interactions can be detected at the sequence level: the examples of complement and blood coagulation proteases. J. Mol. Biol. 282: 459.
38. VILLIERS, C. L., G. J. ARLAUD, and M. G. COLOMB. 1985. Domain structure and associated functions of subcomponents C1r and C1s of the first component of human complement. Proc. Natl. Acad. Sci. U.S.A. 82: 4477.
39. ARLAUD, G. J., C. L. VILLIERS, S. CHESNE, and M. G. COLOMB. 1980. Purified proenzyme C1r. Some characteristics of its activation and subsequent proteolytic cleavage. Biochim. Biophys. Acta 616: 116.
40. BUSBY, T. F., and K. C. INGHAM. 1987. Calcium-sensitive thermal transitions and domain structure of human complement subcomponent C1r. Biochem. 26: 5564.
41. 2-terminal α fragments of human complement proteases C1r and C1s. J. Biol. Chem. 265: 14469.
42. ZAVODSZKY, P., P. GAL, S. CSEH, and V. N. SCHUMAKER. 1993. Protein engineering studies on C1r and C1s. Behring Inst. Mitt. 93: 103.
43. CSEH, S., P. GAL, M. SARVARI, J. DOBO, Z. LORINCZ, V. N. SCHUMAKER, and P. ZAVODSZKY. 1996. Functional effects of domain deletions in a multidomain serine protease, C1r. Mol. Immunol. 33: 351.
44. 2+-binding epidermal growth factor-like domain: its role in protein-protein interactions. Cell 82: 131.
45. DOWNING, A. K., V. KNOTT, J. M. WERNER, C. M. CARDY, I. D. CAMPBELL, and P. A. HANDFORD. 1996. Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders. Cell 85: 597.
46. 2+ binding to the first EGF domain. A combined NMR-small angle X-ray scattering study. Biochem. 35: 11547.
47. 2+-dependent interaction with C1s. J. Biol. Chem. 274: 9149.
48. + -linked association of human complement C1s and C1r. Biochem. 33: 2341.
49. THIELENS, N. M., B. BERSCH, J.-F. HERNANDEZ, and G. J. ARLAUD. 1999. Structure and functions of the interaction domains of C1r and C1s: keystones of the architecture of the C1 complex. Immunopharmacology 42: 3.
50. 2-terminal calcium-binding domain of human complement C1s mediates the interaction of C1r with C1q. Biochem. 29: 4613.
51. 2+-dependent intramolecular control that is released in the C1 complex through a signal transmitted by C1q. Biochem. J. 301: 509.
52. TSAI, S.-W., P. H. POON, and V. N. SCHUMAKER. 1997. Expression and characterization of a 159 amino acid, N-terminal fragment of human complement component C1s. Mol. Immunol. 34: 1273.
53. LAKATOS, S. 1987. Subunit interactions in the first component of complement, C1. Biochem. Biophys. Res. Commun. 149: 378.
54. ILLY, C., N. M. THIELENS, and G. J. ARLAUD. 1993. Chemical characterization and location of ionic interactions involved in the assembly of the C1 complex of human complement. J. Prot. Chem. 12: 771.
55. WEISS, V., C. FAUSER, and J. ENGEL. 1986. Functional model of subcomponent C1 of human complement. J. Mol. Biol. 189: 573.
56. ARLAUD, G. J., J. GAGNON, C. L. VILLIERS, and M. G. COLOMB. 1986. Molecular characterization of the catalytic domains of human complement serine protease C1r. Biochem. 25: 5177.
57. LACROIX, M. B., C. A. AUDE, G. J. ARLAUD, and M. G. COLOMB. 1989. Isolation and functional characterization of the proenzyme form of the catalytic domains of human C1r. Biochem. J. 257: 885.
58. LACROIX, M., V. Rossi, C. GABORIAUD, S. CHEVALLIER, M. JAQUINOD, N. M. THIELENS, J. GAGNON, and G. J. ARLAUD. 1997. Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling. Biochem. 36: 6270.
59. SCHUMAKER, V. N., P. H. POON, G. W SEEGAN, and C. A. SMITH. 1981. Semi-flexible joint in the C1q subunit of the first component of human complement. J. Mol. Biol. 148: 191.
60. POON, P. H., V. N. SCHUMAKER, M. L. PHILLIPS, and C. J. STRANG. 1983. Conformation and restricted flexibility of C1, the first component of human complement. J. Mol. Biol. 168: 563.
61. STRANG, C. J., R. C. SIEGEL, M. L. PHILLIPS, P. H. POON, and V. N. SCHUMAKER. 1982. Ultrastructure of the first component of human complement: electron microscopy of the crosslinked complex. Proc. Natl. Acad. Sci. USA 79: 586.
62. MACKINNON, C. M., P. E. CARTER, S. J. SMYTH, B. DUNBAR, and J. E. FOTHERGILL. 1987. Molecular cloning of cDNA for human complement component C1s. The complete amino acid sequence. Eur. J. Biochem. 169: 547.
63. Tosi, M., C. DUPONCHEL, T. MEO, and C. JULIER 1987. Complete cDNA sequence of human complement C1s and close physical linkage of the homologous genes C1s and C1r. Biochem. 26: 8516.
64. NGUYEN, V. C., M. Tosi, M. S. GROSS, O. COHEN-HAGUENAUER, C. JEGOU-FOUBERT, M. F. DE TAND, T. MEO, and J. FREZAL. 1988. Assignment of the complement serine protease genes C1r and C1s to chromosome 12 region 12p13. Hum. Genet. 78: 363.
65. KUSUMOTO, H., S. HIROSAWA, J. P. SALIER, F. S. HAGEN, and K. KURACHI. 1988. Human genes for complement components C1r and C1s in a close tail-to-tail arrangement. Proc. Natl. Acad. Sci. USA. 85: 7307.
66. Tosi, M., C. DUPONCHEL, T. MEO, and E. COUTURE-TOSI. 1989. Complement genes C1r and C1s feature an intronless serine protease domain closely related to haptoglobin. J. Mol. Biol. 208: 709.
67. ENDO, Y., M. TAKAHASHI, M. NAKAO, H. SAIGA, H. SEKINE M. MATSUSHITA, M. NONAKA, and T. FUJITA. 1998. Two lineages of mannose-binding lectin-associated serine protease (MASP) in vertebrates. J. Immunol. 161: 4924.
68. KINOSHITA, H., H. SAKIYAMA, K. TOKUNAGA, S. IMAJOH-OHMI, Y. HAMADA, K. ISONO, and S. SAKIYAMA. 1989. Complete primary structure of calcium-dependent serine proteinase capable of degrading extracellular matrix proteins. FEBS Lett. 250: 411.
69. SAKAI, H., S. NAKASHIMA, S. YOSHIMURA, Y. NISHIMURA, N. SAKAI, and Y. NOZAWA. 1998. Molecular cloning of a cDNA encoding a serine protease homologous to complement C1s precursor from rat C6 glial cells and its expression during glial differentiation. Gene 209: 87.
70. BYUN, S. J., Y. Y. BAHK, Z. Y. RYOO, K. E. KIM, H. Y. HWANG, J. W. LEE, J. Y. YI, and T. Y. KIM. 2001. Identification of cDNA encoding a serine protease homologous to human complement C1r precursor from grafted mouse skin. J. Invest. Dermatol. 116: 374.
71. LAWSON, P. R., and K. B. M. REID. 2000. A novel PCR-based technique using expressed sequence tags and gene homology for murine genetic mapping: localization of the complement genes. Int. Immunol. 12: 231.
72. GARNIER, G., A. CIRCOLO, Y. XU, and J. E. VOLANAKIS. 2001. The murine C1r/C1s gene family cluster. Mol. Immunol. 38: 91 (abstract)
73. ENDO, Y., T. SATO, M. MATSUSHITA, and T. FUJITA. 1996. Exon structure of the gene encoding the human mannose-binding protein-associated serine protease light chain: comparison with complement C1r and C1s genes. Int. Immunol. 8: 1355.
74. STOVER, C., Y. ENDO, M. TAKAHASHI, N. J. LYNCH, C. CONSTANTINESCU, T. VORUP-JENSEN, S. THIEL, H. FRIEDL, T. HANKELN, R. HALL, S. GREGORY, T. FUJITA, and W. SCHWAEBLE. 2001. The human gene for mannan-binding lectin-associated serine protease-2 (MASP-2), the effector component of the lectin route of complement activation, is part of a tightly linked gene cluster on chromosome 1p36.2-3. Genes Immun. 2: 119.
75. ROGERS, J. 1985. Exon shuffling and intron insertion in serine protease genes. Nature 315: 458.
76. MORRIS, K. M., D. P. ADEN, B. B. KNOWLES, and H. R. COLTEN. 1982. Complement biosynthesis by the human hepatoma-derived cell line HepG2. J. Clin. Invest. 70: 906.
77. RAMADORI, G., H. P. HEINZ, H. MARTIN, K. H. MEYER ZUM BUSCHENFELDE, and M. Loos. 1986. Biosynthesis of the subcomponents C1q, C1r and C1s of the first component of complement (C1) by guinea pig hepatocyte primary cultures. Eur. J. Immunol. 16: 1137.
78. LIGOUDISTIANOU, C., G. GARNIER, A. CIRCOLO, and J. E. VOLANAKIS. 2001. C1r-like protein (C1r-LP): a novel human complement related protein. Abstracts of the Vth International Workshop on C1, the first component of complement, and collectins. Mainz, October 26-28, 2001.