Serpin conformations

Molecular and Cellular Aspects of the Serpinopathies and Disorders in Serpin Activity

Volumn , Issue , 2007, Pages 35-66

  Pearce, Mary C ^a   Pike, Robert N ^a   Lesk, Arthur M ^b   Bottomley, Stephen P ^c  

^a MONASH UNIVERSITY   (Australia)

^b PENNSYLVANIA STATE UNIVERSITY   (United States)

^c MONASH UNIVERSITY   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84967373605     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1142/9789812707543_0002     Document Type: Chapter

References (101)

1. Whisstock JC, Pike RN, Jin L, Skinner R, Pei XY, Carrell RWand LeskAM(2000) Conformational changes in serpins: II. The mechanism of activation of antithrombin by heparin. J Mol Biol 301:1287-1305.
2. Whisstock JC, Skinner R, Carrell RW and LeskAM(2000) Conformational changes in serpins: I. The native and cleaved conformations of alpha(1)- antitrypsin. J Mol Biol 295:651-665.
3. Lawrence DA, Olson ST, Palaniappan S and Ginsburg D(1994) Serpin reactive center loop mobility is required for inhibitor function but not for enzyme recognition. J Biol Chem 269:27657-27662.
4. Lawrence DA, Olson ST, Palaniappan S and Ginsburg D(1994) Engineering plasminogen activator inhibitor 1 mutants with increased functional stability. Biochemistry 33:3643-3648.
5. Horvath AJ, Irving JA, Rossjohn J, Law RH, Bottomley SP, Quinsey NS, Pike RN, Coughlin PB and Whisstock JC(2005) The murine orthologue of human antichymotrypsin: A structural paradigm for clade A3 serpins.J Biol Chem.
6. Irving JA, Cabrita LD, Rossjohn J, Pike RN, Bottomley SP and Whisstock JC(2003) The 1.5A crystal structure of a prokaryote serpin: controlling conformational change in a heated environment. Structure (Camb) 11:387-397.
7. Dafforn TR, Mahadeva R, Elliott PR, Sivasothy P and Lomas DA(1999) A kinetic mechanism for the polymerization of alpha1-antitrypsin. J Biol Chem 274:9548-9555.
8. Pearce MC, Cabrita LD, Rubin H, Gore MG and Bottomley SP(2004) Identification of residual structure within denatured antichymotrypsin:implications for serpin folding and misfolding. Biochem Biophys Res Commun 324:729-735.
9. Onda M, BelorgeyD, Sharp LK and LomasDA(2005) Latent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodies. J Biol Chem 280:13735-13741.
10. Cabrita LD, Whisstock JC and Bottomley SP(2002) Probing the role of the F-helix in serpin stability through a single tryptophan substitution. Biochemistry 41:4575-4581.
11. Stein PE, Leslie AG, Finch JT and Carrell RW(1991) Crystal structure of uncleaved ovalbumin at 1.95 A resolution. J Mol Biol 221:941-959.
12. Bruch M, Weiss V and Engel J(1988) Plasma serine proteinase inhibitors (serpins) exhibit major conformational changes and a large increase in conformational stability upon cleavage at their reactive sites. J Biol Chem 263:16626-16630.
13. Herve M and Ghelis C(1990) Conformational changes in intact and papainmodified alpha 1-proteinase inhibitor induced by guanidinium chloride. Eur J Biochem 191:653-658.
14. Gooptu B, Hazes B, Chang WS, Dafforn TR, Carrell RW, Read RJ and Lomas DA(2000) Inactive conformation of the serpin alpha(1)- antichymotrypsin indicates two-stage insertion of the reactive loop:implications for inhibitory function and conformational disease. Proc Natl Acad Sci USA 97:67-72.
15. Stein PE and Carrell RW(1995) What do dysfunctional serpins tell us about molecular mobility and disease? Nat Struct Biol 2:96-113.
16. Huntington JA, Pannu NS, Hazes B, Read RJ, Lomas DA and Carrell RW(1999) A 2.6A structure of a serpin polymer and implications for conformational disease. J Mol Biol 293:449-455.
17. Dunstone MA, Dai W, Whisstock JC, Rossjohn J, Pike RN, Feil SC, Le Bonniec BF, Parker MW and Bottomley SP(2000) Cleaved antitrypsin polymers at atomic resolution. Protein Sci 9:417-420.
18. Nar H, Bauer M, Stassen JM, Lang D, Gils A and Declerck PJ(2000) Plasminogen activator inhibitor 1. Structure of the native serpin, comparison to its other conformers and implications for serpin inactivation. J Mol Biol 297:683-695.
19. Marszal E, Danino D and Shrake A(2003) A novel mode of polymerization of alpha1-proteinase inhibitor. J Biol Chem 278:19611-19618.
20. Carrell RW, Stein PE, Fermi G andWardell MR(1994) Biological implications of a 3 A structure of dimeric antithrombin. Structure 2:257-270.
21. Tew DJ and Bottomley SP(2001) Probing the equilibrium denaturation of the serpin alpha(1)-antitrypsin with single tryptophan mutants: Evidence for structure in the urea unfolded state. J Mol Biol 313:1161-1169.
22. Loebermann H, Tokuoka R, Deisenhofer J and Huber R(1984) Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function. J Mol Biol 177:531-557.
23. Elliott PR, LomasDA, CarrellRWandAbrahams JP(1996) Inhibitory conformation of the reactive loop of alpha 1-antitrypsin. Nat Struct Biol 3:676-681.
24. Stein PE and Carrell RW(1995) What do dysfunctional serpins tell us about molecular mobility and disease? Nat Struct Biol 2:96-113.
25. Whisstock JC, Skinner R, Carrell RW and LeskAM(2000) Conformational changes in serpins: I. The native and cleaved conformations of alpha(1)- antitrypsin. J Mol Biol 296:685-699.
26. Hopkins PC, Carrell RW and Stone SR(1993) Effects of mutations in the hinge region of serpins. Biochemistry 32:7650-7657.
27. Bottomley SP, Lawrenson ID, Tew D, Dai W, Whisstock JC and Pike RN(2001) The role of strand 1 of the C beta-sheet in the structure and function of alpha(1)-antitrypsin. Protein Sci 10:2518-2524.
28. Wang Q and Shaltiel S (2003) Distal hinge of plasminogen activator inhibitor-1 involves its latency transition and specificities toward serine proteases. BMC Biochem 4:5.
29. He S, Yang JC, Tsang S, Sim RB and Whaley K(1997) Role of the distal hinge region of C1-inhibitor in the regulation of C1s activity. FEBS Lett 412:506-510.
30. Bijnens AP, Gils A, Stassen JM, Komissarov AA, Knockaert I, Brouwers E, Shore JD and Declerck PJ(2001) The distal hinge of the reactive site loop and its proximity: a target to modulate plasminogen activator inhibitor-1 activity. J Biol Chem 276:44912-44918.
31. De Taeye B, Compernolle G, Dewilde M, Biesemans W and Declerck PJ(2003) Immobilization of the distal hinge in the labile serpin plasminogen activator inhibitor 1: Identification of a transition state with distinct conformational and functional properties. J Biol Chem 278:23899-23905.
32. Irving JA, Pike RN, Lesk AM and Whisstock JC(2000) Phylogeny of the serpin superfamily: Implications of patterns of amino acid conservation for structure and function. Genome Res 10:1845-1864.
33. Lomas DA, Evans DL, Finch JT and Carrell RW(1992) The mechanism of Z alpha 1-antitrypsin accumulation in the liver. Nature 357:605-607.
34. Mottonen J, Strand A, Symersky J, Sweet RM, Danley DE, Geoghegan KF, Gerard RD and Goldsmith EJ(1992) Structural basis of latency in plasminogen activator inhibitor-1. Nature 355:270-273.
35. Stein PE, Leslie AG, Finch JT, TurnellWG, McLaughlin PJ and Carrell RW(1990) Crystal structure of ovalbumin as a model for the reactive centre of serpins. Nature 347:99-102.
36. Skinner R, Abrahams JP, Whisstock JC, Lesk AM, Carrell RW and Wardell MR(1997) The 2.6 A structure of antithrombin indicates a conformational change at the heparin binding site. J Mol Biol 266:601-609.
37. Baglin TP, Carrell RW, Church FC, Esmon CT and Huntington JA(2002) Crystal structures of native and thrombin-complexedheparin cofactor II reveal a multistep allosteric mechanism. Proc Natl Acad Sci USA 99:11079-11084.
38. Fulton KF, BuckleAM, Cabrita LD, Irving JA, Butcher RE, Smith I, Reeve S, Lesk AM, Bottomley SP, Rossjohn J and Whisstock JC(2005) The high resolution crystal structure of a native thermostable serpin reveals the complex mechanism underpinning the stressed to relaxed transition. J Biol Chem 280:8435-8442.
39. Irving JA, Steenbakkers PJ, Lesk AM, Op den Camp HJ, Pike RN and Whisstock JC(2002) Serpins in prokaryotes. Mol Biol Evol 19:1881-1890.
40. Im H, Woo MS, Hwang KY and Yu MH(2002) Interactions causing the kinetic trap in serpin protein folding. J Biol Chem 277:46347-46354.
41. Im H andYu MH(2000) Role of Lys335 in the metastability and function of inhibitory serpins. Protein Sci 9:934-941.
42. Huntington JA, Fan B, Karlsson KE, Deinum J, LawrenceDAand Gettins PG(1997) Serpin conformational change in ovalbumin. Enhanced reactive center loop insertion through hinge region mutations. Biochemistry 36:5432-5440.
43. Chaillan-Huntington CE, Gettins PG, Huntington JA and Patston PA(1997) The P6-P2 region of serpins is critical for proteinase inhibition and complex stability. Biochemistry 36:9562-9570.
44. Jin L, Abrahams JP, Skinner R, Petitou M, Pike RN and Carrell RW(1997) The anticoagulant activation of antithrombin by heparin. Proc Natl Acad Sci USA 94:14683-14688.
45. Plotnick MI, Schechter NM, Wang ZM, Liu X and Rubin H(1997) Role of the P6-P3_ region of the serpin reactive loop in the formation and breakdown of the inhibitory complex. Biochemistry 36:14601-14608.
46. Plotnick MI, Samakur M, Wang ZM, Liu X, Rubin H, Schechter NM and Selwood T(2002) Heterogeneity in serpin-protease complexes as demonstrated by differences in the mechanism of complex breakdown. Biochemistry 41:334-342.
47. Mast AE, Enghild JJ and Salvesen G(1992) Conformation of the reactive site loop of alpha 1-proteinase inhibitor probed by limited proteolysis. Biochemistry 31:2720-2728.
48. Baumann U, Huber R, BodeW, Grosse D, Lesjak M and Laurell CB(1991) Crystal structure of cleaved human α1 antichymotrypsin at 2.7Å resolution and its comparison with other serpins. J Mol Biol 218:595-606.
49. Mourey L, Samama JP, Delarue M, Petitou M, Choay J and Moras D(1993) Crystal structure of cleaved bovine antithrombin III at 3.2A resolution. J Mol Biol 232:223-241.
50. Li W, Johnson DJ, Esmon CT and Huntington JA(2004) Structure of the antithrombin-thrombin-heparin ternary complex reveals the antithrombotic mechanism of heparin. Nat Struct Mol Biol 11:857-862.
51. Dementiev A, Petitou M, Herbert JM and Gettins PG(2004) The ternary complex of antithrombin-anhydrothrombin-heparin reveals the basis of inhibitor specificity. Nat Struct Mol Biol 11:863-867.
52. Ye S, Cech AL, Belmares R, Bergstrom RC, Tong Y, Corey DR, Kanost MR and Goldsmith EJ(2001) The structure of a Michaelis serpin-protease complex. Nat Struct Biol 8:979-983.
53. Huntington JA, Read RJ and CarrellRW(2000) Structure of a serpin-protease complex shows inhibition by deformation. Nature 407:923-926.
54. Stratikos E and Gettins PG(1997) Major proteinase movement upon stable serpin-proteinase complex formation. Proc Natl Acad Sci USA 94:453-458.
55. Stratikos E and Gettins PG(1998) Mapping the serpin-proteinase complex using single cysteine variants of alpha1-proteinase inhibitor Pittsburgh. J Biol Chem 273:15582-15589.
56. Stratikos E and Gettins PG(1999) Formation of the covalent serpinproteinase complex involves translocation of the proteinase by more than 70 A and full insertion of the reactive center loop into beta-sheet A. Proc Natl Acad Sci USA 96:4808-4813.
57. Mellet P, Boudier C, Mely Y and Bieth JG(1998) Stopped flow fluorescence energy transfer measurement of the rate constants describing the reversible formation and the irreversible rearrangement of the elastasealpha1- proteinase inhibitor complex. J Biol Chem 273:9119-9123.
58. Carrell RW and Lomas DA(1997) Conformational disease. Lancet 350:134-138.
59. Davis RL, Shrimpton AE, Holohan PD, Bradshaw C, Feiglin D, Collins GH, Sonderegger P, Kinter J, Becker LM, Lacbawan F, Krasnewich D, MuenkeM, Lawrence DA, Yerby MS, Shaw CM, Gooptu B, Elliott PR, Finch JT, Carrell RW and Lomas DA(1999) Familial dementia caused by polymerization of mutant neuroserpin. Nature 401:376-379.
60. Rodriguez-Soriano J, Fidalgo I, Camarero C, Vallo A and Oliveros R(1978) Juvenile cirrhosis and membranous glomerulonephritis in a child with alpha1- antitrypsin deficiency PiSZ. Acta Paediatr Scand 67:793-796.
61. Beauchamp NJ, Pike RN, DalyM, Butler L, MakrisM, Dafforn TR, Zhou A, Fitton HL, Preston FE, Peake IR and Carrell RW(1998) AntithrombinsWibble andWobble (T85M/K): Archetypal conformational diseases with in vivo latent-transition, thrombosis and heparin activation. Blood 92:2696-2706.
62. Chang W-SW and Lomas DA(1998) Latent α1-antichymotrypsin. J Biol Chem 273:3695-3701.
63. Stout TJ, Graham H, Buckley DI and MatthewsDJ(2000) Structures of active and latent PAI-1: A possible stabilizing role for chloride ions. Biochemistry 39:8460-8469.
64. Lomas DA, Elliott PR, Chang WS, Wardell MR and Carrell RW(1995) Preparation and characterization of latent alpha 1-antitrypsin. J Biol Chem 270:5282-5288.
65. Lomas DA, Finch JT, Seyama K, Nukiwa T and Carrell RW(1993) Alpha 1-antitrypsin Siiyama (Ser53->Phe). Further evidence for intracellular loopsheet polymerization. J Biol Chem 268:15333-15335.
66. James EL and Bottomley SP(1998) The mechanism of alpha 1-antitrypsin polymerization probed by fluorescence spectroscopy. Arch Biochem Biophys 356:296-300.
67. Crowther DC, Belorgey D, Miranda E, Kinghorn KJ, Sharp LK and Lomas DA(2004) Practical genetics: Alpha-1-antitrypsin deficiency and the serpinopathies. Eur J Hum Genet 12:167-172.
68. LomasDA, BelorgeyD, MallyaM, Onda M, Kinghorn KJ, Sharp LK, Phillips RL, Page R, Crowther DC and Miranda E(2004) Polymerisation underlies alpha1-antitrypsin deficiency, dementia and other serpinopathies. Front Biosci 9:2873-2891.
69. Schulze AJ, Baumann U, Knof S, Jaeger E, Huber R and Laurell CB(1990) Structural transition of alpha 1-antitrypsin by a peptide sequentially similar to beta-strand s4A. Eur J Biochem 194:51-56.
70. Bottomley SP, Hopkins PC and Whisstock JC(1998) Alpha 1-antitrypsin polymerisation can occur by both loop A and C sheet mechanisms. Biochem Biophys Res Commun 251:1-5.
71. Chang WS, Whisstock J, Hopkins PC, Lesk AM, Carrell RW and Wardell MR(1997) Importance of the release of strand 1C to the polymerization mechanism of inhibitory serpins. Protein Sci 6:89-98.
72. Sharp AM, Stein PE, Pannu NS, Carrell RW, Berkenpas MB, Ginsburg D, Lawrence DA and Read RJ(1999) The active conformation of plasminogen activator inhibitor 1, a target for drugs to control fibrinolysis and cell adhesion. Struct Fold Des 7:111-118.
73. Zhou A, Faint R, Charlton P, Dafforn TR, Carrell RW and Lomas DA(2001) Polymerization of plasminogen activator inhibitor-1. J Biol Chem 276:9115-9122.
74. Devlin GL, Parfrey H, Tew DJ, Lomas DA and Bottomley SP(2001) Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency. Am J Respir Cell Mol Biol 24:727-732.
75. Chow MK, Devlin GL and Bottomley SP(2001) Osmolytes as modulators of conformational changes in serpins. Biol Chem 382:1593-1599.
76. Zhou A, Stein PE, Huntington JA and Carrell RW(2003) Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol. J Biol Chem 278:15116-15122.
77. Zhou A, Huntington JA, Pannu NS, Carrell RW and Read RJ(2003) How vitronectin binds PAI-1 to modulate fibrinolysis and cellmigration. Nat Struct Biol 10:541-544.
78. Elliott PR, Pei XY, Dafforn TR and Lomas DA(2000) Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease. Protein Sci 9:1274-1281.
79. Parfrey H, Mahadeva R, Ravenhill NA, Zhou A, Dafforn TR, Foreman RC and Lomas DA(2003) Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease. J Biol Chem 278:33060-33066.
80. Song HK, Lee KN, Kwon KS, Yu MH and Suh SW(1995) Crystal structure of an uncleaved alpha 1-antitrypsin reveals the conformation of its inhibitory reactive loop. FEBS Lett 377:150-154.
81. Kim S, Woo J, Seo EJ, Yu M and Ryu S(2001) A 2.1 Å resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops. J Mol Biol 306:109-119.
82. Zhou A, Stein PE, Huntington JA, Sivasothy P, Lomas DA and Carrell RW(2004) How small peptides block and reverse serpin polymerisation. J Mol Biol 342:931-941.
83. Dementiev A, Simonovic M, Volz K and Gettins PG(2003) Canonical inhibitor-like interactions explain reactivity of alpha1-proteinase inhibitor Pittsburgh and antithrombin with proteinases. J Biol Chem 278:37881-37887.
84. Huntington JA, McCoy A, Belzar KJ, Pei XY, Gettins PG and Carrell RW(2000) The conformational activation of antithrombin.A 2.85-A structure of a fluorescein derivative reveals an electrostatic link between the hinge and heparin binding regions. J Biol Chem 275:15377-15383.
85. Schreuder HA, de Boer B, Dijkema R, Mulders J, Theunissen HJ, Grootenhuis PDand HolWG(1994) The intact and cleaved human antithrombin III complex as a model for serpin-proteinase interactions. Nat Struct Biol 1:48-54.
86. Tucker HM, Mottonen J, Goldsmith EJ and Gerard RD(1995) Engineering of plasminogen activator inhibitor-1 to reduce the rate of latency transition. Nat Struct Biol 2:442-445.
87. Aertgeerts K, De Bondt HL, De Ranter C and Declerck PJ(1995) Crystallization and X-ray diffraction data of the cleaved form of plasminogen activator inhibitor-1. Proteins 23:118-121.
88. Harrop SJ, Jankova L, Coles M, Jardine D, Whittaker JS, Gould AR, Meister A, King GC, Mabbutt BC and Curmi PM(1999) The crystal structure of plasminogen activator inhibitor 2 at 2.0A resolution: Implications for serpin function. Struct Fold Des 7:43-54.
89. Jankova L, Harrop SJ, Saunders DN, Andrews JL, Bertram KC, Gould AR, Baker MS and Curmi PM(2001) Crystal structure of the complex of plasminogen activator inhibitor 2 with a peptide mimicking the reactive center loop. J Biol Chem 276:43374-43382.
90. Simonovic M, Gettins PGW and Volz K(2000) Crystal structure of viral serpin crmA provides insights into its mechanism of cysteine proteinase inhibition. Protein Sci 9:1423-1427.
91. Renatus M, Zhou Q, Stennicke HR, Snipas SJ, Turk D, Bankston LA, Liddington RC and Salvesen GS(2000) Crystal structure of the apoptotic suppressor CrmA in its cleaved form. Struct Fold Des 8:789-797.
92. Lukacs CM, Rubin H and Christianson DW(1998) Engineering an anion-binding cavity in antichymotrypsin modulates the "spring-loaded" serpin-protease interaction. Biochemistry 37:3297-3304.
93. Lukacs CM, Zhong JQ, Plotnick MI, Rubin H, Cooperman BS and Christianson DW(1996) Arginine substitutions in the hinge region of antichymotrypsin affect serpin beta-sheet rearrangement. Nat Struct Biol 3:888-893.
94. Huntington JA, Kjellberg M and Stenflo J(2003) Crystal structure of protein C inhibitor provides insights into hormone binding and heparin activation. Structure (Camb) 11:205-215.
95. Bos IG, Hack CE and Abrahams JP(2002) Structural and functional aspects of C1-inhibitor. Immunobiology 205:518-533.
96. Yamasaki M, Takahashi N and Hirose M(2003) Crystal structure of Sovalbumin as a non-loop-inserted thermostabilized serpin form. J Biol Chem 278:35524-35530.
97. Yamasaki M, Arii Y, Mikami B and Hirose M(2002) Loop-inserted and thermostabilized structure of P1-P1_ cleaved ovalbumin mutant R339T. J Mol Biol 315:113-120.
98. Briand C,KozlovSV, Sonderegger P and GrutterMG(2001) Crystal structure of neuroserpin:Aneuronal serpin involved in a conformational disease. FEBS Lett 505:18-22.
99. Simonovic M, Gettins PG and Volz K(2001) Crystal structure of human PEDF, a potent anti-angiogenic and neurite growth-promoting factor. Proc Natl Acad Sci USA 98:11131-11135.
100. Al-Ayyoubi M, Gettins PG and Volz K(2004) Crystal structure of human maspin, a serpin with antitumor properties: Reactive center loop of maspin is exposed but constrained. J Biol Chem 279:55540-55544.
101. Li J, Wang Z, Canagarajah B, Jiang H, Kanost M and Goldsmith EJ(1999) The structure of active serpin 1K from Manduca sexta. Struct Fold Des 7:103-109.