-
1
-
-
0343193210
-
Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease
-
P.R. Elliott, and X.Y. Pei D.A. Lomas Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease Protein Sci. 9 2000 1274 1281
-
(2000)
Protein Sci.
, vol.9
, pp. 1274-1281
-
-
Elliott, P.R.1
Pei, X.Y.2
Lomas, D.A.3
-
2
-
-
0034687422
-
Structure of a serpin-protease complex shows inhibition by deformation
-
J.A. Huntington, R.J. Read, and R.W. Carrell Structure of a serpin-protease complex shows inhibition by deformation Nature 407 2000 923 926
-
(2000)
Nature
, vol.407
, pp. 923-926
-
-
Huntington, J.A.1
Read, R.J.2
Carrell, R.W.3
-
3
-
-
3042617720
-
How do proteins avoid becoming too stable? Biophysical studies into metastable proteins
-
L.D. Cabrita, and S.P. Bottomley How do proteins avoid becoming too stable? Biophysical studies into metastable proteins Eur. Biophys. J. 33 2004 83 88
-
(2004)
Eur. Biophys. J.
, vol.33
, pp. 83-88
-
-
Cabrita, L.D.1
Bottomley, S.P.2
-
4
-
-
0021076101
-
The isolation of a clone for human α1-antitrypsin and the detection of α1-antitrypsin in mRNA from liver and leukocytes
-
J. Rogers, and N. Kalsheker S.E. Humphries The isolation of a clone for human α1-antitrypsin and the detection of α1-antitrypsin in mRNA from liver and leukocytes Biochem. Biophys. Res. Commun. 116 1983 375 382
-
(1983)
Biochem. Biophys. Res. Commun.
, vol.116
, pp. 375-382
-
-
Rogers, J.1
Kalsheker, N.2
Humphries, S.E.3
-
5
-
-
0024842004
-
The α1-antitrypsin gene and its deficiency states
-
R.G. Crystal The α1-antitrypsin gene and its deficiency states Trends Genet. 5 1989 411 417
-
(1989)
Trends Genet.
, vol.5
, pp. 411-417
-
-
Crystal, R.G.1
-
6
-
-
0026755363
-
The mechanism of Z α1-antitrypsin accumulation in the liver
-
D.A. Lomas, and D.L. Evans R.W. Carrell The mechanism of Z α1-antitrypsin accumulation in the liver Nature 357 1992 605 607
-
(1992)
Nature
, vol.357
, pp. 605-607
-
-
Lomas, D.A.1
Evans, D.L.2
Carrell, R.W.3
-
7
-
-
0021288078
-
Role of connective tissue proteases in the pathogenesis of chronic inflammatory lung disease
-
J.E. Gadek, and G.A. Fells R.G. Crystal Role of connective tissue proteases in the pathogenesis of chronic inflammatory lung disease Environ. Health Perspect. 55 1984 297 306
-
(1984)
Environ. Health Perspect.
, vol.55
, pp. 297-306
-
-
Gadek, J.E.1
Fells, G.A.2
Crystal, R.G.3
-
8
-
-
0023003588
-
Aggregation of plasma Z type α1-antitrypsin suggests basic defect for the deficiency
-
D.W. Cox, G.D. Billingsley, and J.W. Callahan Aggregation of plasma Z type α1-antitrypsin suggests basic defect for the deficiency FEBS Lett. 205 1986 255 260
-
(1986)
FEBS Lett.
, vol.205
, pp. 255-260
-
-
Cox, D.W.1
Billingsley, G.D.2
Callahan, J.W.3
-
10
-
-
78049239444
-
Defining the mechanism of polymerization in the serpinopathies
-
U.I. Ekeowa, and J. Freeke D.A. Lomas Defining the mechanism of polymerization in the serpinopathies Proc. Natl. Acad. Sci. USA 107 2010 17146 17151
-
(2010)
Proc. Natl. Acad. Sci. USA
, vol.107
, pp. 17146-17151
-
-
Ekeowa, U.I.1
Freeke, J.2
Lomas, D.A.3
-
11
-
-
0036882160
-
Acid denaturation of α1-antitrypsin: Characterization of a novel mechanism of serpin polymerization
-
G.L. Devlin, and M.K. Chow S.P. Bottomley Acid denaturation of α1-antitrypsin: characterization of a novel mechanism of serpin polymerization J. Mol. Biol. 324 2002 859 870
-
(2002)
J. Mol. Biol.
, vol.324
, pp. 859-870
-
-
Devlin, G.L.1
Chow, M.K.2
Bottomley, S.P.3
-
12
-
-
0027473016
-
Effect of the Z mutation on the physical and inhibitory properties of α1-antitrypsin
-
D.A. Lomas, and D.L. Evans R.W. Carrell Effect of the Z mutation on the physical and inhibitory properties of α1-antitrypsin Biochemistry 32 1993 500 508
-
(1993)
Biochemistry
, vol.32
, pp. 500-508
-
-
Lomas, D.A.1
Evans, D.L.2
Carrell, R.W.3
-
13
-
-
0036510604
-
6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z α(1)-antitrypsin-related cirrhosis
-
R. Mahadeva, and T.R. Dafforn D.A. Lomas 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z α(1)-antitrypsin-related cirrhosis J. Biol. Chem. 277 2002 6771 6774
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 6771-6774
-
-
Mahadeva, R.1
Dafforn, T.R.2
Lomas, D.A.3
-
14
-
-
0026532063
-
Conformation of the reactive site loop of α1-proteinase inhibitor probed by limited proteolysis
-
A.E. Mast, J.J. Enghild, and G. Salvesen Conformation of the reactive site loop of α1-proteinase inhibitor probed by limited proteolysis Biochemistry 31 1992 2720 2728
-
(1992)
Biochemistry
, vol.31
, pp. 2720-2728
-
-
Mast, A.E.1
Enghild, J.J.2
Salvesen, G.3
-
15
-
-
80053561166
-
Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer
-
M. Yamasaki, and T.J. Sendall J.A. Huntington Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer EMBO Rep. 12 2011 1011 1017
-
(2011)
EMBO Rep.
, vol.12
, pp. 1011-1017
-
-
Yamasaki, M.1
Sendall, T.J.2
Huntington, J.A.3
-
16
-
-
0032529612
-
The mechanism of α1-antitrypsin polymerization probed by fluorescence spectroscopy
-
E.L. James, and S.P. Bottomley The mechanism of α1-antitrypsin polymerization probed by fluorescence spectroscopy Arch. Biochem. Biophys. 356 1998 296 300
-
(1998)
Arch. Biochem. Biophys.
, vol.356
, pp. 296-300
-
-
James, E.L.1
Bottomley, S.P.2
-
17
-
-
0033515559
-
A kinetic mechanism for the polymerization of α1-antitrypsin
-
T.R. Dafforn, and R. Mahadeva D.A. Lomas A kinetic mechanism for the polymerization of α1-antitrypsin J. Biol. Chem. 274 1999 9548 9555
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 9548-9555
-
-
Dafforn, T.R.1
Mahadeva, R.2
Lomas, D.A.3
-
18
-
-
77449144020
-
Kinetic instability of the serpin Z α1-antitrypsin promotes aggregation
-
A.S. Knaupp, and V. Levina S.P. Bottomley Kinetic instability of the serpin Z α1-antitrypsin promotes aggregation J. Mol. Biol. 396 2010 375 383
-
(2010)
J. Mol. Biol.
, vol.396
, pp. 375-383
-
-
Knaupp, A.S.1
Levina, V.2
Bottomley, S.P.3
-
20
-
-
46249092554
-
GROMACS 4: Algorithms for highly efficient, load-balanced, and scalable molecular simulation
-
B. Hess, and C. Kutzner E. Lindahl GROMACS 4: algorithms for highly efficient, load-balanced, and scalable molecular simulation J. Chem. Theory Comput. 4 2008 435 447
-
(2008)
J. Chem. Theory Comput.
, vol.4
, pp. 435-447
-
-
Hess, B.1
Kutzner, C.2
Lindahl, E.3
-
21
-
-
4444282928
-
A biomolecular force field based on the free enthalpy of hydration and solvation: The GROMOS force-field parameter sets 53A5 and 53A6
-
C. Oostenbrink, and A. Villa W.F. van Gunsteren A biomolecular force field based on the free enthalpy of hydration and solvation: the GROMOS force-field parameter sets 53A5 and 53A6 J. Comput. Chem. 25 2004 1656 1676
-
(2004)
J. Comput. Chem.
, vol.25
, pp. 1656-1676
-
-
Oostenbrink, C.1
Villa, A.2
Van Gunsteren, W.F.3
-
22
-
-
0002775934
-
Interaction models for water in relation to protein hydration
-
B. Pullman, Reidel Dordrecht, The Netherlands
-
H.J.C. Berendsen, J.P.M. Postma, and W.F. van Gunsteren Interaction models for water in relation to protein hydration B. Pullman, Intermolecular Forces 1981 Reidel Dordrecht, The Netherlands 331 342
-
(1981)
Intermolecular Forces
, pp. 331-342
-
-
Berendsen, H.J.C.1
Postma, J.P.M.2
Van Gunsteren, W.F.3
-
23
-
-
33750587438
-
Molecular dynamics with coupling to an external bath
-
H.J.C. Berendsen, and J.P.M. Postma J.R. Haak Molecular dynamics with coupling to an external bath J. Chem. Phys. 81 1984 3684 3690
-
(1984)
J. Chem. Phys.
, vol.81
, pp. 3684-3690
-
-
Berendsen, H.J.C.1
Postma, J.P.M.2
Haak, J.R.3
-
24
-
-
0000388705
-
LINCS: A linear constraint solver for molecular simulations
-
B. Hess, and H. Bekker J.G.E.M. Fraaije LINCS: a linear constraint solver for molecular simulations J. Comput. Chem. 18 1997 1463 1472
-
(1997)
J. Comput. Chem.
, vol.18
, pp. 1463-1472
-
-
Hess, B.1
Bekker, H.2
Fraaije, J.G.E.M.3
-
25
-
-
4544369164
-
A generalized reaction field method for molecular-dynamics simulations
-
I.G. Tironi, and R. Sperb W.F. Vangunsteren A generalized reaction field method for molecular-dynamics simulations J. Chem. Phys. 102 1995 5451 5459
-
(1995)
J. Chem. Phys.
, vol.102
, pp. 5451-5459
-
-
Tironi, I.G.1
Sperb, R.2
Vangunsteren, W.F.3
-
26
-
-
0035878765
-
Comparison of four methods to compute the dielectric permittivity of liquids from molecular dynamics simulations
-
T.N. Heinz, W.F. van Gunsteren, and P.H. Hunenberger Comparison of four methods to compute the dielectric permittivity of liquids from molecular dynamics simulations J. Chem. Phys. 115 2001 1125 1136
-
(2001)
J. Chem. Phys.
, vol.115
, pp. 1125-1136
-
-
Heinz, T.N.1
Van Gunsteren, W.F.2
Hunenberger, P.H.3
-
27
-
-
33846823909
-
Particle mesh Ewald - An N.log(N) method for Ewald sums in large systems
-
T. Darden, D. York, and L. Pedersen Particle mesh Ewald - an N.log(N) method for Ewald sums in large systems J. Chem. Phys. 98 1993 10089 10092
-
(1993)
J. Chem. Phys.
, vol.98
, pp. 10089-10092
-
-
Darden, T.1
York, D.2
Pedersen, L.3
-
28
-
-
33846231365
-
Influence of long-range electrostatic treatments on the folding of the N-terminal H4 histone tail peptide
-
R.D. Lins, and U. Rothlisberger Influence of long-range electrostatic treatments on the folding of the N-terminal H4 histone tail peptide J. Chem. Theory Comput. 2 2006 246 250
-
(2006)
J. Chem. Theory Comput.
, vol.2
, pp. 246-250
-
-
Lins, R.D.1
Rothlisberger, U.2
-
29
-
-
0001029719
-
On finite-size effects in computer simulations using the Ewald potential
-
F. Figueirido, G.S. Del Buono, and R.M. Levy On finite-size effects in computer simulations using the Ewald potential J. Chem. Phys. 103 1995 6133 6142
-
(1995)
J. Chem. Phys.
, vol.103
, pp. 6133-6142
-
-
Figueirido, F.1
Del Buono, G.S.2
Levy, R.M.3
-
30
-
-
0035964342
-
Electrostatics of nanosystems: Application to microtubules and the ribosome
-
N.A. Baker, and D. Sept J.A. McCammon Electrostatics of nanosystems: application to microtubules and the ribosome Proc. Natl. Acad. Sci. USA 98 2001 10037 10041
-
(2001)
Proc. Natl. Acad. Sci. USA
, vol.98
, pp. 10037-10041
-
-
Baker, N.A.1
Sept, D.2
McCammon, J.A.3
-
31
-
-
70349446583
-
Expression, purification and characterization of recombinant Z α1-antitrypsin - The most common cause of α1-antitrypsin deficiency
-
V. Levina, and W. Dai S.P. Bottomley Expression, purification and characterization of recombinant Z α1-antitrypsin - the most common cause of α1-antitrypsin deficiency Protein Expr. Purif. 68 2009 226 232
-
(2009)
Protein Expr. Purif.
, vol.68
, pp. 226-232
-
-
Levina, V.1
Dai, W.2
Bottomley, S.P.3
-
32
-
-
33744757719
-
The conformational dynamics of a metastable serpin studied by hydrogen exchange and mass spectrometry
-
Y. Tsutsui, and L. Liu P.L. Wintrode The conformational dynamics of a metastable serpin studied by hydrogen exchange and mass spectrometry Biochemistry 45 2006 6561 6569
-
(2006)
Biochemistry
, vol.45
, pp. 6561-6569
-
-
Tsutsui, Y.1
Liu, L.2
Wintrode, P.L.3
-
33
-
-
34447095724
-
Molecular gymnastics: Serpin structure, folding and misfolding
-
J.C. Whisstock, and S.P. Bottomley Molecular gymnastics: serpin structure, folding and misfolding Curr. Opin. Struct. Biol. 16 2006 761 768
-
(2006)
Curr. Opin. Struct. Biol.
, vol.16
, pp. 761-768
-
-
Whisstock, J.C.1
Bottomley, S.P.2
-
34
-
-
80053571955
-
The structural diversity in α1-antitrypsin misfolding
-
S.P. Bottomley The structural diversity in α1-antitrypsin misfolding EMBO Rep. 12 2011 983 984
-
(2011)
EMBO Rep.
, vol.12
, pp. 983-984
-
-
Bottomley, S.P.1
-
35
-
-
0034523345
-
Phylogeny of the serpin superfamily: Implications of patterns of amino acid conservation for structure and function
-
J.A. Irving, and R.N. Pike J.C. Whisstock Phylogeny of the serpin superfamily: implications of patterns of amino acid conservation for structure and function Genome Res. 10 2000 1845 1864
-
(2000)
Genome Res.
, vol.10
, pp. 1845-1864
-
-
Irving, J.A.1
Pike, R.N.2
Whisstock, J.C.3
-
36
-
-
80054866168
-
Structural change in β-sheet A of Z α1-antitrypsin is responsible for accelerated polymerization and disease
-
A.S. Knaupp, and S.P. Bottomley Structural change in β-sheet A of Z α1-antitrypsin is responsible for accelerated polymerization and disease J. Mol. Biol. 413 2011 888 898
-
(2011)
J. Mol. Biol.
, vol.413
, pp. 888-898
-
-
Knaupp, A.S.1
Bottomley, S.P.2
-
37
-
-
0024217519
-
Repair of the secretion defect in the Z form of α1-antitrypsin by addition of a second mutation
-
M. Brantly, M. Courtney, and R.G. Crystal Repair of the secretion defect in the Z form of α1-antitrypsin by addition of a second mutation Science 242 1988 1700 1702
-
(1988)
Science
, vol.242
, pp. 1700-1702
-
-
Brantly, M.1
Courtney, M.2
Crystal, R.G.3
-
38
-
-
0025047629
-
A mutant T4 lysozyme displays five different crystal conformations
-
H.R. Faber, and B.W. Matthews A mutant T4 lysozyme displays five different crystal conformations Nature 348 1990 263 266
-
(1990)
Nature
, vol.348
, pp. 263-266
-
-
Faber, H.R.1
Matthews, B.W.2
-
39
-
-
0024791087
-
Three-dimensional structure of a light chain dimer crystallized in water. Conformational flexibility of a molecule in two crystal forms
-
K.R. Ely, and J.N. Herron A.B. Edmundson Three-dimensional structure of a light chain dimer crystallized in water. Conformational flexibility of a molecule in two crystal forms J. Mol. Biol. 210 1989 601 615
-
(1989)
J. Mol. Biol.
, vol.210
, pp. 601-615
-
-
Ely, K.R.1
Herron, J.N.2
Edmundson, A.B.3
-
40
-
-
84886602275
-
The structure of triclinic lysozyme at 2-5 Å resolution
-
J. Moult, and A. Yonath A. Saya The structure of triclinic lysozyme at 2-5 Å resolution J. Mol. Biol. 100 1976 179 195
-
(1976)
J. Mol. Biol.
, vol.100
, pp. 179-195
-
-
Moult, J.1
Yonath, A.2
Saya, A.3
-
41
-
-
0026740792
-
Structural effects induced by mutagenesis affected by crystal packing factors: The structure of a 30-51 disulfide mutant of basic pancreatic trypsin inhibitor
-
C. Eigenbrot, M. Randal, and A.A. Kossiakoff Structural effects induced by mutagenesis affected by crystal packing factors: the structure of a 30-51 disulfide mutant of basic pancreatic trypsin inhibitor Proteins 14 1992 75 87
-
(1992)
Proteins
, vol.14
, pp. 75-87
-
-
Eigenbrot, C.1
Randal, M.2
Kossiakoff, A.A.3
-
42
-
-
0347912957
-
Influence of solvent accessibility and intermolecular contacts on atomic mobilities in hemerythrins
-
S. Sheriff, and W.A. Hendrickson L.H. Jensen Influence of solvent accessibility and intermolecular contacts on atomic mobilities in hemerythrins Proc. Natl. Acad. Sci. USA 82 1985 1104 1107
-
(1985)
Proc. Natl. Acad. Sci. USA
, vol.82
, pp. 1104-1107
-
-
Sheriff, S.1
Hendrickson, W.A.2
Jensen, L.H.3
-
43
-
-
0025037796
-
Comparison of the dynamics of myoglobin in different crystal forms
-
G.N. Phillips Jr. Comparison of the dynamics of myoglobin in different crystal forms Biophys. J. 57 1990 381 383
-
(1990)
Biophys. J.
, vol.57
, pp. 381-383
-
-
Phillips, Jr.G.N.1
-
44
-
-
80053626537
-
Accessing protein conformational ensembles using room-temperature x-ray crystallography
-
J.S. Fraser, and H. van den Bedem T. Alber Accessing protein conformational ensembles using room-temperature x-ray crystallography Proc. Natl. Acad. Sci. USA 108 2011 16247 16252
-
(2011)
Proc. Natl. Acad. Sci. USA
, vol.108
, pp. 16247-16252
-
-
Fraser, J.S.1
Van Den Bedem, H.2
Alber, T.3
-
45
-
-
80054866168
-
Structural change in β-sheet A of Z α(1)-antitrypsin is responsible for accelerated polymerization and disease
-
A.S. Knaupp, and S.P. Bottomley Structural change in β-sheet A of Z α(1)-antitrypsin is responsible for accelerated polymerization and disease J. Mol. Biol. 413 2011 888 898
-
(2011)
J. Mol. Biol.
, vol.413
, pp. 888-898
-
-
Knaupp, A.S.1
Bottomley, S.P.2
-
46
-
-
84862673659
-
Z-type α-1-antitrypsin (Aat) is less competent than M1-type Aat as an inhibitor of neutrophil elastase
-
F. Ogushi, and G. Fells R. Crystal Z-type α-1-antitrypsin (Aat) is less competent than M1-type Aat as an inhibitor of neutrophil elastase Am. Rev. Respir. Dis. 133 1986 A218
-
(1986)
Am. Rev. Respir. Dis.
, vol.133
, pp. 218
-
-
Ogushi, F.1
Fells, G.2
Crystal, R.3
-
47
-
-
0024498368
-
Disruption of the 290-342 salt bridge is not responsible for the secretory defect of the PiZ α1-antitrypsin variant
-
R.N. Sifers, C.P. Hardick, and S.L.C. Woo Disruption of the 290-342 salt bridge is not responsible for the secretory defect of the PiZ α1-antitrypsin variant J. Biol. Chem. 264 1989 2997 3001
-
(1989)
J. Biol. Chem.
, vol.264
, pp. 2997-3001
-
-
Sifers, R.N.1
Hardick, C.P.2
Woo, S.L.C.3
-
48
-
-
46149105683
-
Hereditary α-1-antitrypsin deficiency and its clinical consequences
-
L. Fregonese, and J. Stolk Hereditary α-1-antitrypsin deficiency and its clinical consequences Orphanet J. Rare Dis. 3 2008 16 24
-
(2008)
Orphanet J. Rare Dis.
, vol.3
, pp. 16-24
-
-
Fregonese, L.1
Stolk, J.2
-
49
-
-
20744450475
-
α1-antitrypsin deficiency
-
J.K. Stoller, and L.S. Aboussouan α1-antitrypsin deficiency Lancet 365 2005 2225 2236
-
(2005)
Lancet
, vol.365
, pp. 2225-2236
-
-
Stoller, J.K.1
Aboussouan, L.S.2
-
50
-
-
0035224263
-
342Lys mutation in α1-antitrypsin on its structure, studied by molecular modeling methods
-
342Lys mutation in α1-antitrypsin on its structure, studied by molecular modeling methods Acta Biochim. Pol. 48 2001 65 75
-
(2001)
Acta Biochim. Pol.
, vol.48
, pp. 65-75
-
-
Jezierski, G.1
Pasenkiewicz-Gierula, M.2
|