Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
1
|
The mechanism of Z alpha 1-antitrypsin accumulation in the liver.
|
Nature
|
1992
|
5.71
|
2
|
The serpins are an expanding superfamily of structurally similar but functionally diverse proteins. Evolution, mechanism of inhibition, novel functions, and a revised nomenclature.
|
J Biol Chem
|
2001
|
4.81
|
3
|
Structure and variation of human alpha 1-antitrypsin.
|
Nature
|
1982
|
4.13
|
4
|
Structure of a serpin-protease complex shows inhibition by deformation.
|
Nature
|
2000
|
4.02
|
5
|
Implications of the three-dimensional structure of alpha 1-antitrypsin for structure and function of serpins.
|
Biochemistry
|
1989
|
3.18
|
6
|
The anticoagulant activation of antithrombin by heparin.
|
Proc Natl Acad Sci U S A
|
1997
|
3.09
|
7
|
The estimation of red cell superoxide dismutase activity.
|
J Lab Clin Med
|
1975
|
2.75
|
8
|
Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder.
|
N Engl J Med
|
1983
|
2.36
|
9
|
What do dysfunctional serpins tell us about molecular mobility and disease?
|
Nat Struct Biol
|
1995
|
2.30
|
10
|
Variations in the structure of human haemoglobin. With particular reference to the unstable haemoglobins.
|
Br Med Bull
|
1969
|
2.07
|
11
|
Smoking, lung function, and alpha 1-antitrypsin deficiency.
|
Lancet
|
1985
|
1.99
|
12
|
Familial dementia caused by polymerization of mutant neuroserpin.
|
Nature
|
1999
|
1.88
|
13
|
A simple method for the detection of unstable haemoglobins.
|
Br J Haematol
|
1972
|
1.87
|
14
|
Reactions involving superoxide and normal and unstable haemoglobins.
|
Biochem J
|
1976
|
1.66
|
15
|
Inhibitory conformation of the reactive loop of alpha 1-antitrypsin.
|
Nat Struct Biol
|
1996
|
1.66
|
16
|
alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage.
|
J Clin Invest
|
1986
|
1.61
|
17
|
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 U S A
|
2000
|
1.54
|
18
|
A circulating variant of human proalbumin.
|
Nature
|
1978
|
1.52
|
19
|
Crystal structure of uncleaved ovalbumin at 1.95 A resolution.
|
J Mol Biol
|
1991
|
1.52
|
20
|
Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin.
|
Biochemistry
|
1993
|
1.49
|
21
|
Hormone binding globulins undergo serpin conformational change in inflammation.
|
Nature
|
1988
|
1.47
|
22
|
Abnormal haem binding and globin SH group blockade in unstable haemoglobins.
|
Nature
|
1968
|
1.44
|
23
|
Plakalbumin, alpha 1-antitrypsin, antithrombin and the mechanism of inflammatory thrombosis.
|
Nature
|
1985
|
1.44
|
24
|
Crystal structure of ovalbumin as a model for the reactive centre of serpins.
|
Nature
|
1990
|
1.42
|
25
|
Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization.
|
J Biol Chem
|
1993
|
1.41
|
26
|
Activated oxygen and haemolysis.
|
Br J Haematol
|
1975
|
1.41
|
27
|
A new hemoglobin variant resembling hemoglobin E. Hemoglobin E Saskatoon: beta-22 Glu replaced by Lys.
|
Can J Biochem
|
1967
|
1.40
|
28
|
The active conformation of plasminogen activator inhibitor 1, a target for drugs to control fibrinolysis and cell adhesion.
|
Structure
|
1999
|
1.40
|
29
|
Studies of hemoglobin denaturation and Heinz body formation in the unstable hemoglobins.
|
J Clin Invest
|
1974
|
1.39
|
30
|
Imprisonment of J-P Allain.
|
Lancet
|
1993
|
1.34
|
31
|
Haemoglobin Köln (beta-98 valine--methionine): an unstable protein causing inclusion-body anaemia.
|
Nature
|
1966
|
1.33
|
32
|
alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization.
|
J Biol Chem
|
1995
|
1.33
|
33
|
Differences between alpha- and beta-chain mutants of human haemoglobin and between alpha- and beta-thalassaemia. Possible duplication of the alpha-chain gene.
|
Br Med J
|
1968
|
1.33
|
34
|
Effects of mutations in the hinge region of serpins.
|
Biochemistry
|
1993
|
1.29
|
35
|
The unstable haemoglobin haemolytic anaemias.
|
Semin Hematol
|
1969
|
1.27
|
36
|
The 2.6 A structure of antithrombin indicates a conformational change at the heparin binding site.
|
J Mol Biol
|
1997
|
1.27
|
37
|
A 2.6 A structure of a serpin polymer and implications for conformational disease.
|
J Mol Biol
|
1999
|
1.26
|
38
|
Structural and functional characterization of the abnormal Z alpha 1-antitrypsin isolated from human liver.
|
FEBS Lett
|
1984
|
1.24
|
39
|
Oxidation of human haemoglobin by copper. Mechanism and suggested role of the thiol group of residue beta-93.
|
Biochem J
|
1977
|
1.22
|
40
|
The serpin inhibitory mechanism is critically dependent on the length of the reactive center loop.
|
J Biol Chem
|
2001
|
1.22
|
41
|
The significance of monoclonal gammopathy in a normal population.
|
Aust N Z J Med
|
1971
|
1.16
|
42
|
Basis of the defect in alpha-1-antitrypsin deficiency.
|
Nature
|
1973
|
1.16
|
43
|
Preparation and characterization of latent alpha 1-antitrypsin.
|
J Biol Chem
|
1995
|
1.15
|
44
|
Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)
|
J Clin Invest
|
1994
|
1.14
|
45
|
Prevalence of antithrombin deficiency in the healthy population.
|
Br J Haematol
|
1994
|
1.13
|
46
|
Yeast KEX2 protease has the properties of a human proalbumin converting enzyme.
|
Science
|
1987
|
1.13
|
47
|
A new doubly substituted sickling haemoglobin: HbS-Oman.
|
Br J Haematol
|
1989
|
1.12
|
48
|
Conformational changes in serpins: II. The mechanism of activation of antithrombin by heparin.
|
J Mol Biol
|
2000
|
1.12
|
49
|
The amino acid sequence of the alpha chain of the major haemoglobin of the rat (Rattus norvegicus).
|
Biochem J
|
1975
|
1.11
|
50
|
alpha 1-Antitrypsin microheterogeneity. Isolation and physiological significance of isoforms.
|
Biochim Biophys Acta
|
1982
|
1.10
|
51
|
Mechanism of oxyhaemoglobin breakdown on reaction with acetylphenylhydrazine.
|
Biochem J
|
1978
|
1.06
|
52
|
Inhibition of plasmin, urokinase, tissue plasminogen activator, and C1S by a myxoma virus serine proteinase inhibitor.
|
J Biol Chem
|
1993
|
1.06
|
53
|
Dysfunctional C1-inhibitor(At), isolated from a type II hereditary-angio-oedema plasma, contains a P1 'reactive centre' (Arg444----His) mutation.
|
Biochem J
|
1988
|
1.06
|
54
|
Alpha-1-antitrypsin-Pittsburgh. A potent inhibitor of human plasma factor XIa, kallikrein, and factor XIIf.
|
J Clin Invest
|
1986
|
1.05
|
55
|
Antithrombins Wibble and Wobble (T85M/K): archetypal conformational diseases with in vivo latent-transition, thrombosis, and heparin activation.
|
Blood
|
1998
|
1.04
|
56
|
Structural explanation for the deficiency of S alpha 1-antitrypsin.
|
Nat Struct Biol
|
1996
|
1.04
|
57
|
Implications for function and therapy of a 2.9 A structure of binary-complexed antithrombin.
|
J Mol Biol
|
1998
|
1.03
|
58
|
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
|
2000
|
1.03
|
59
|
Importance of the release of strand 1C to the polymerization mechanism of inhibitory serpins.
|
Protein Sci
|
1997
|
1.03
|
60
|
Human alpha 1-antitrypsin: carbohydrate attachment and sequence homology.
|
FEBS Lett
|
1981
|
1.02
|
61
|
Polymerization of plasminogen activator inhibitor-1.
|
J Biol Chem
|
2000
|
1.00
|
62
|
Latent antithrombin and its detection, formation and turnover in the circulation.
|
J Thromb Haemost
|
2004
|
1.00
|
63
|
Heparin-dependent modification of the reactive center arginine of antithrombin and consequent increase in heparin binding affinity.
|
J Biol Chem
|
1997
|
1.00
|
64
|
Conformational changes in serpins: I. The native and cleaved conformations of alpha(1)-antitrypsin.
|
J Mol Biol
|
2000
|
1.00
|
65
|
Functional abnormality of proalbumin Christchurch.
|
Biochim Biophys Acta
|
1980
|
1.00
|
66
|
Hydrolink gels: a rapid and simple approach to the detection of DNA mutations in thromboembolic disease.
|
J Clin Pathol
|
1992
|
1.00
|
67
|
The structural basis for neutrophil inactivation of C1 inhibitor.
|
Biochem J
|
1989
|
0.99
|
68
|
A genetically engineered mutant of alpha 1-antitrypsin protects connective tissue from neutrophil damage and may be useful in lung disease.
|
Lancet
|
1984
|
0.98
|
69
|
Ovalbumin and angiotensinogen lack serpin S-R conformational change.
|
Biochem J
|
1989
|
0.98
|
70
|
Haemoglobin sydney: Beta-67 (E11) valine modified to alanine: an emerging pattern of unstable haemoglobins.
|
Nature
|
1967
|
0.98
|
71
|
Increased proteolytic cleavage of alpha 1-antitrypsin (alpha 1-proteinase inhibitor) in knee-joint synovial fluid from patients with rheumatoid arthritis.
|
Biochem Soc Trans
|
1990
|
0.98
|
72
|
The control of neutrophil chemotaxis by inhibitors of cathepsin G and chymotrypsin.
|
J Biol Chem
|
1995
|
0.98
|
73
|
Heparin binding defect in a new antithrombin III variant: Rouen, 47 Arg to His.
|
Blood
|
1987
|
0.97
|
74
|
Formation of the antithrombin heterodimer in vivo and the onset of thrombosis.
|
Blood
|
1999
|
0.97
|
75
|
Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.
|
J Biol Chem
|
1995
|
0.95
|
76
|
Probing serpin reactive-loop conformations by proteolytic cleavage.
|
Biochem J
|
1996
|
0.95
|
77
|
Protein C deficiency and portal thrombosis in liver transplantation in children.
|
Lancet
|
1988
|
0.95
|
78
|
Haemoglobin Genova: beta-28 (B10) leucine replaced by proline.
|
Nature
|
1967
|
0.95
|
79
|
Three cysteine residues in the alpha chain of rat haemoglobin (albino Rattus norvegicus): 13 (A 11), 104 (G 11) and 111 (G 18).
|
Biochim Biophys Acta
|
1974
|
0.94
|
80
|
The effect of the Z mutation on the ability of alpha 1-antitrypsin to prevent neutrophil mediated tissue damage.
|
Biochim Biophys Acta
|
1994
|
0.93
|
81
|
Translation and processing of normal (PiMM) and abnormal (PiZZ) human alpha 1-antitrypsin.
|
FEBS Lett
|
1983
|
0.92
|
82
|
Haemoglobin J Tongariki (alpha-115 alanine--aspartic acid): the first new haemoglobin variant found in a Pacific (Melanesian) population.
|
J Med Genet
|
1967
|
0.91
|
83
|
Köln haemoglobinopathy. Further data and a comparison with other hereditary Heinz body anaemias.
|
Br J Haematol
|
1967
|
0.91
|
84
|
Apolipoprotein E2-Christchurch (136 Arg----Ser). New variant of human apolipoprotein E in a patient with type III hyperlipoproteinemia.
|
J Clin Invest
|
1987
|
0.91
|
85
|
Antithrombin Glasgow, 393 Arg to His: a P1 reactive site variant with increased heparin affinity but no thrombin inhibitory activity.
|
FEBS Lett
|
1988
|
0.91
|
86
|
Mutations in the shutter region of antithrombin result in formation of disulfide-linked dimers and severe venous thrombosis.
|
J Thromb Haemost
|
2004
|
0.90
|
87
|
The effect of a reducing-end extension on pentasaccharide binding by antithrombin.
|
J Biol Chem
|
2000
|
0.90
|
88
|
Conformational changes in serpins: I. The native and cleaved conformations of alpha(1)-antitrypsin.
|
J Mol Biol
|
2000
|
0.90
|
89
|
Kinetic studies on the interaction of alpha 1-proteinase inhibitor (Pittsburgh) with trypsin-like serine proteinases.
|
Biol Chem Hoppe Seyler
|
1986
|
0.89
|
90
|
Proteolysis of human native and oxidised alpha 1-proteinase inhibitor by matrilysin and stromelysin.
|
Biochim Biophys Acta
|
1994
|
0.89
|
91
|
Active site of alpha 1-antitrypsin: homologous site in antithrombin-III.
|
Biochem Biophys Res Commun
|
1980
|
0.88
|
92
|
New carbohydrate site in mutant antithrombin (7 Ile----Asn) with decreased heparin affinity.
|
FEBS Lett
|
1988
|
0.88
|
93
|
Proposed heparin binding site in antithrombin based on arginine 47. A new variant Rouen-II, 47 Arg to Ser.
|
J Clin Invest
|
1988
|
0.88
|
94
|
Preparative induction and characterization of L-antithrombin: a structural homologue of latent plasminogen activator inhibitor-1.
|
Biochemistry
|
1997
|
0.88
|
95
|
Detection of variants of haemoglobin D in Australia: haemoglobin D Camperdown alpha 2 A beta 2 121 Glu leads to Val and haemoglobin D Punjab alpha 2 A beta 2 121 Glu leads to Gin.
|
Med J Aust
|
1974
|
0.88
|
96
|
Alzheimer's disease. Enter a protease inhibitor.
|
Nature
|
1988
|
0.87
|
97
|
Alpha-1-antitrypsin variants in New Zealand.
|
N Z Med J
|
1975
|
0.87
|
98
|
Angiotensinogen cleavage by renin: importance of a structurally constrained N-terminus.
|
FEBS Lett
|
1998
|
0.87
|
99
|
Development of a novel recombinant serpin with potential antithrombotic properties.
|
J Biol Chem
|
1995
|
0.87
|
100
|
Molecular genetics of human antithrombin deficiency.
|
Hum Mutat
|
1996
|
0.86
|
101
|
The serpins: nature's molecular mousetraps.
|
Sci Prog
|
2001
|
0.86
|
102
|
The attachment of Heinz bodies to the red cell membrane.
|
Br J Haematol
|
1973
|
0.86
|
103
|
Circulating proalbumin associated with a variant proteinase inhibitor.
|
Biochim Biophys Acta
|
1984
|
0.85
|
104
|
alpha-1-Antitrypsin: sequence of the Z variant tryptic peptide.
|
FEBS Lett
|
1977
|
0.85
|
105
|
Mechanisms of antithrombin polymerisation and heparin activation probed by the insertion of synthetic reactive loop peptides.
|
Biol Chem
|
1997
|
0.85
|
106
|
In vitro synthesis of M and Z forms of human alpha 1-antitrypsin.
|
FEBS Lett
|
1982
|
0.84
|
107
|
Proteolytic inactivation of human alpha 1 antitrypsin by human stromelysin.
|
FEBS Lett
|
1991
|
0.84
|
108
|
Antithrombin Rouen-IV 24 Arg----Cys. The amino-terminal contribution to heparin binding.
|
FEBS Lett
|
1990
|
0.84
|
109
|
Protease specificity and heparin binding and activation of recombinant protease nexin I.
|
J Biol Chem
|
1991
|
0.83
|
110
|
A protein structural approach to the solution of biological problems: alpha 1-antitrypsin as a recent example.
|
Am J Physiol
|
1993
|
0.83
|
111
|
Oxidative interactions between haemoglobin and membrane lipid. A liposome model.
|
Biochem J
|
1984
|
0.83
|
112
|
CpG dinucleotides are "hotspots" for mutation in the antithrombin III gene. Twelve variants identified using the polymerase chain reaction.
|
Mol Biol Med
|
1989
|
0.83
|
113
|
Characterization of the alpha chain core of human haemoglobin variants.
|
Biochim Biophys Acta
|
1968
|
0.83
|
114
|
alpha 1-Antitrypsin Christchurch, 363 Glu----Lys: mutation at the P'5 position does not affect inhibitory activity.
|
Biochim Biophys Acta
|
1986
|
0.83
|
115
|
Genetic engineering and the serpins.
|
Bioessays
|
1988
|
0.82
|
116
|
Antithrombin Dublin (-3 Val----Glu): an N-terminal variant which has an aberrant signal peptidase cleavage site.
|
FEBS Lett
|
1990
|
0.82
|
117
|
Bassen-Kornzweig and Refsum's syndromes. Some comments on their recognition among the N.Z. population.
|
N Z Med J
|
1966
|
0.82
|
118
|
The incidence of dysfunctional antithrombin variants: four cases in 210 patients with thromboembolic disease.
|
Br J Haematol
|
1991
|
0.82
|
119
|
Characterization of Heinz bodies in unstable haemoglobin haemolytic anaemia.
|
Nature
|
1972
|
0.81
|
120
|
The abnormality of the S variant of human alpha-1-antitrypsin.
|
Biochim Biophys Acta
|
1976
|
0.81
|
121
|
Haemoglobin Camperdown beta104(G6) arginine leads to serine.
|
Biochim Biophys Acta
|
1975
|
0.81
|
122
|
Recombinant antitrypsin Pittsburgh undergoes proteolytic cleavage during E. coli sepsis and fails to prevent the associated coagulopathy in a primate model.
|
Thromb Haemost
|
1998
|
0.81
|
123
|
Commercial plasma alpha1-antitrypsin (Prolastin) contains a conformationally inactive, latent component.
|
Eur Respir J
|
1997
|
0.80
|
124
|
The reactive site of alpha 1-antitrypsin is C-terminal, not N-terminal.
|
Biochim Biophys Acta
|
1983
|
0.80
|
125
|
Hemoglobin Brisbane: beta68 Leu replaced by His. A new high oxygen affinity variant.
|
Hemoglobin
|
1981
|
0.80
|
126
|
Hemoglobin Palmerston North beta 23 (B5) Val replaced by Phe. A new variant identified in a patient with polycythemia.
|
Hemoglobin
|
1982
|
0.79
|
127
|
Haemoglobin F auckland G gamma 7 Asp leads to Asn: further evidence for multiple genes for the gamma chain.
|
Biochim Biophys Acta
|
1974
|
0.79
|
128
|
Alpha-1-antitrypsin deficiency in New Zealand.
|
N Z Med J
|
1975
|
0.79
|
129
|
Zinc acetate as a precipitant of unstable haemoglobins.
|
J Clin Pathol
|
1981
|
0.79
|
130
|
Haemoglobin F Melbourne Ggamma 16 Gly leads to Arg and haemoglobin F carlton Ggamma 121 Glu leads to Lys. Further evidence for varied activity of gamma-chain genes.
|
Biochim Biophys Acta
|
1977
|
0.79
|
131
|
Alpha 1-antitrypsin, emphysema and smoking.
|
N Z Med J
|
1984
|
0.79
|
132
|
Retracted
Heparin binding site, conformational change, and activation of antithrombin.
|
Biochemistry
|
1992
|
0.79
|
133
|
Human alpha 1-antitrypsin expression in Xenopus oocytes. Secretion of the normal (PiM) and abnormal (PiZ) forms.
|
Eur J Biochem
|
1985
|
0.79
|
134
|
Human Z alpha 1-antitrypsin accumulates intracellularly and stimulates lysosomal activity when synthesised in the Xenopus oocyte.
|
FEBS Lett
|
1985
|
0.79
|
135
|
The molecular pathology of human alpha 1-antitrypsin.
|
Biochem Soc Symp
|
1984
|
0.78
|
136
|
The precipitation of hemoglobin by zinc: its application to the isolation of a minor hemoglobin fraction (HbB2 delta 16 Gly replaced by Arg) from lysed whole blood.
|
Hemoglobin
|
1982
|
0.78
|
137
|
Alpha 1-antitrypsin and serum albumin mRNA accumulation in normal, acute phase and ZZ human liver.
|
FEBS Lett
|
1985
|
0.78
|
138
|
Characterization of Hb Aalborg, a new unstable hemoglobin variant, by fast atom bombardment mass spectrometry.
|
Hemoglobin
|
1990
|
0.78
|
139
|
Detecting iron deficiency by serum tests.
|
Clin Chem
|
1985
|
0.78
|
140
|
Two novel antithrombin variants, Asn187Asp and Asn187Lys, indicate a functional role for asparagine 187.
|
Blood Coagul Fibrinolysis
|
1995
|
0.78
|
141
|
A pilot study of antithrombin replacement in intensive care management: the effects on mortality, coagulation and renal function.
|
Transfus Med
|
1991
|
0.78
|
142
|
Characterization of antithrombins produced by active site mutagenesis of human alpha 1-antitrypsin expressed in yeast.
|
Blood
|
1989
|
0.78
|
143
|
Serpins: implications of a mobile reactive centre.
|
Curr Opin Biotechnol
|
1992
|
0.78
|
144
|
Heterozygous alpha 1-antitrypsin deficiency: a longitudinal lung function study.
|
N Z Med J
|
1981
|
0.77
|
145
|
Five antithrombin variants, four associated with thrombosis.
|
Blood Coagul Fibrinolysis
|
1997
|
0.77
|
146
|
Haemoglobin M Hyde Park: a hereditary methaemoglobinaemia in a Caucasian child.
|
N Z Med J
|
1968
|
0.77
|
147
|
Screening for heparin binding variants of antithrombin.
|
J Clin Pathol
|
1991
|
0.77
|
148
|
Antithrombin Cambridge, 384 Ala to Pro: a new variant identified using the polymerase chain reaction.
|
FEBS Lett
|
1989
|
0.77
|
149
|
Insertions/deletions in the antithrombin gene: 3 mutations associated with non-expression.
|
Thromb Haemost
|
1992
|
0.77
|
150
|
Intracellular serpins in haemopoietic and peripheral blood cells.
|
Br J Haematol
|
2001
|
0.77
|
151
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