Published in J Mol Biol on October 29, 1999
An overview of the serpin superfamily. Genome Biol (2006) 2.14
Defining the mechanism of polymerization in the serpinopathies. Proc Natl Acad Sci U S A (2010) 1.48
The yeast prion Ure2p retains its native alpha-helical conformation upon assembly into protein fibrils in vitro. EMBO J (2002) 1.31
The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. Hum Mol Genet (2008) 1.20
Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology. Thorax (2004) 1.18
Insights from bacterial subtilases into the mechanisms of intramolecular chaperone-mediated activation of furin. Methods Mol Biol (2011) 1.10
Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function. Mol Biol Cell (2007) 1.08
Gene therapy for alpha-1 antitrypsin deficiency. Hum Mol Genet (2011) 1.04
Prolastin, a pharmaceutical preparation of purified human alpha1-antitrypsin, blocks endotoxin-mediated cytokine release. Respir Res (2005) 1.01
Loop-sheet mechanism of serpin polymerization tested by reactive center loop mutations. J Biol Chem (2010) 0.94
Crystallographic and cellular characterisation of two mechanisms stabilising the native fold of alpha1-antitrypsin: implications for disease and drug design. J Mol Biol (2009) 0.93
Allosteric modulation of hormone release from thyroxine and corticosteroid-binding globulins. J Biol Chem (2011) 0.87
Biochemical characterization of a neuroserpin variant associated with hereditary dementia. Am J Pathol (2001) 0.87
Structural dynamics associated with intermediate formation in an archetypal conformational disease. Structure (2012) 0.86
Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway. Biosci Rep (2013) 0.86
Fluorescence correlation spectroscopic study of serpin depolymerization by computationally designed peptides. J Mol Biol (2007) 0.85
Plasminogen activator inhibitor-1 polymers, induced by inactivating amphipathic organochemical ligands. Biochem J (2003) 0.85
Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization. Biochem J (2014) 0.83
Identification and analysis of serpin-family genes by homology and synteny across the 12 sequenced Drosophilid genomes. BMC Genomics (2009) 0.82
Serpins show structural basis for oligomer toxicity and amyloid ubiquity. FEBS Lett (2008) 0.81
The mechanism of fibril formation of a non-inhibitory serpin ovalbumin revealed by the identification of amyloidogenic core regions. J Biol Chem (2010) 0.80
The role of strand 1 of the C beta-sheet in the structure and function of alpha(1)-antitrypsin. Protein Sci (2001) 0.78
Structure and aggregation mechanism of beta(2)-microglobulin (83-99) peptides studied by molecular dynamics simulations. Biophys J (2007) 0.78
The shapes of Z-α1-antitrypsin polymers in solution support the C-terminal domain-swap mechanism of polymerization. Biophys J (2014) 0.77
Mutagenesis of the bovSERPINA3-3 demonstrates the requirement of aspartate-371 for intermolecular interaction and formation of dimers. Protein Sci (2012) 0.76
The human serpin proteinase inhibitor-9 self-associates at physiological temperatures. Protein Sci (2004) 0.76
Crystallography & NMR system: A new software suite for macromolecular structure determination. Acta Crystallogr D Biol Crystallogr (1998) 169.28
Incorporation of prior phase information strengthens maximum-likelihood structure refinement. Acta Crystallogr D Biol Crystallogr (1998) 6.47
The mechanism of Z alpha 1-antitrypsin accumulation in the liver. Nature (1992) 5.71
Not your average density. Structure (1997) 5.40
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
Conformational disease. Lancet (1997) 4.53
Evaluation of COPD Longitudinally to Identify Predictive Surrogate End-points (ECLIPSE). Eur Respir J (2008) 4.53
Structure and variation of human alpha 1-antitrypsin. Nature (1982) 4.13
The dead-end elimination theorem and its use in protein side-chain positioning. Nature (1992) 4.11
Structure of a serpin-protease complex shows inhibition by deformation. Nature (2000) 4.02
Cross-validated maximum likelihood enhances crystallographic simulated annealing refinement. Proc Natl Acad Sci U S A (1997) 3.64
Implications of the three-dimensional structure of alpha 1-antitrypsin for structure and function of serpins. Biochemistry (1989) 3.18
The anticoagulant activation of antithrombin by heparin. Proc Natl Acad Sci U S A (1997) 3.09
Shiga-like toxins are neutralized by tailored multivalent carbohydrate ligands. Nature (2000) 2.94
The estimation of red cell superoxide dismutase activity. J Lab Clin Med (1975) 2.75
Accumulating evidence suggests that several AB-toxins subvert the endoplasmic reticulum-associated protein degradation pathway to enter target cells. Biochemistry (1997) 2.59
Intraneuronal Abeta, non-amyloid aggregates and neurodegeneration in a Drosophila model of Alzheimer's disease. Neuroscience (2005) 2.54
The crystal structure of pertussis toxin. Structure (1994) 2.41
Structure of the shiga-like toxin I B-pentamer complexed with an analogue of its receptor Gb3. Biochemistry (1998) 2.38
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
Crystal structure of the cell-binding B oligomer of verotoxin-1 from E. coli. Nature (1992) 2.32
What do dysfunctional serpins tell us about molecular mobility and disease? Nat Struct Biol (1995) 2.30
Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study. BMJ (1998) 2.22
Siblings of patients with severe chronic obstructive pulmonary disease have a significant risk of airflow obstruction. Am J Respir Crit Care Med (2001) 2.11
Variations in the structure of human haemoglobin. With particular reference to the unstable haemoglobins. Br Med Bull (1969) 2.07
Smoking, lung function, and alpha 1-antitrypsin deficiency. Lancet (1985) 1.99
Structure of the complex of Streptomyces griseus protease B and the third domain of the turkey ovomucoid inhibitor at 1.8-A resolution. Biochemistry (1983) 1.95
Familial dementia caused by polymerization of mutant neuroserpin. Nature (1999) 1.88
A simple method for the detection of unstable haemoglobins. Br J Haematol (1972) 1.87
Crystal and molecular structures of the complex of alpha-chymotrypsin with its inhibitor turkey ovomucoid third domain at 1.8 A resolution. J Mol Biol (1987) 1.71
A kinetic mechanism for the polymerization of alpha1-antitrypsin. J Biol Chem (1999) 1.70
Crystal structure of Pseudomonas aeruginosa PAK pilin suggests a main-chain-dominated mode of receptor binding. J Mol Biol (2000) 1.67
Reactions involving superoxide and normal and unstable haemoglobins. Biochem J (1976) 1.66
Inhibitory conformation of the reactive loop of alpha 1-antitrypsin. Nat Struct Biol (1996) 1.66
CTLA4 gene polymorphisms are associated with chronic bronchitis. Eur Respir J (2009) 1.62
alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage. J Clin Invest (1986) 1.61
Polymorphisms in the superoxide dismutase-3 gene are associated with emphysema in COPD. COPD (2010) 1.60
Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis. J Clin Invest (1999) 1.60
Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease. Protein Sci (2000) 1.58
Crystallographic analysis of oxygenated and deoxygenated states of arthropod hemocyanin shows unusual differences. Proteins (1994) 1.58
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
A circulating variant of human proalbumin. Nature (1978) 1.52
Crystal structure of uncleaved ovalbumin at 1.95 A resolution. J Mol Biol (1991) 1.52
Critical evaluation of comparative model building of Streptomyces griseus trypsin. Biochemistry (1984) 1.49
Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin. Biochemistry (1993) 1.49
Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema. Am J Respir Cell Mol Biol (1998) 1.47
Hormone binding globulins undergo serpin conformational change in inflammation. Nature (1988) 1.47
Impact of non-linear smoking effects on the identification of gene-by-smoking interactions in COPD genetics studies. Thorax (2010) 1.46
Abnormal haem binding and globin SH group blockade in unstable haemoglobins. Nature (1968) 1.44
Plakalbumin, alpha 1-antitrypsin, antithrombin and the mechanism of inflammatory thrombosis. Nature (1985) 1.44
Structure of a pilin monomer from Pseudomonas aeruginosa: implications for the assembly of pili. J Biol Chem (2001) 1.43
Pathogenic alpha 1-antitrypsin polymers are formed by reactive loop-beta-sheet A linkage. J Biol Chem (2000) 1.43
Crystal structure of ovalbumin as a model for the reactive centre of serpins. Nature (1990) 1.42
Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization. J Biol Chem (1993) 1.41
Activated oxygen and haemolysis. Br J Haematol (1975) 1.41
The active conformation of plasminogen activator inhibitor 1, a target for drugs to control fibrinolysis and cell adhesion. Structure (1999) 1.40
A new hemoglobin variant resembling hemoglobin E. Hemoglobin E Saskatoon: beta-22 Glu replaced by Lys. Can J Biochem (1967) 1.40
Studies of hemoglobin denaturation and Heinz body formation in the unstable hemoglobins. J Clin Invest (1974) 1.39
Extending the limits of molecular replacement through combined simulated annealing and maximum-likelihood refinement. Acta Crystallogr D Biol Crystallogr (1999) 1.37
Imprisonment of J-P Allain. Lancet (1993) 1.34
Haemoglobin Köln (beta-98 valine--methionine): an unstable protein causing inclusion-body anaemia. Nature (1966) 1.33
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
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
Neuroserpin: a serpin to think about. Cell Mol Life Sci (2006) 1.32
Effects of mutations in the hinge region of serpins. Biochemistry (1993) 1.29
Molecular pathology of X linked retinoschisis: mutations interfere with retinoschisin secretion and oligomerisation. Br J Ophthalmol (2006) 1.28
The unstable haemoglobin haemolytic anaemias. Semin Hematol (1969) 1.27
The 2.6 A structure of antithrombin indicates a conformational change at the heparin binding site. J Mol Biol (1997) 1.27
Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation. J Mol Biol (1998) 1.26
Structure of a pertussis toxin-sugar complex as a model for receptor binding. Nat Struct Biol (1994) 1.24
Structural and functional characterization of the abnormal Z alpha 1-antitrypsin isolated from human liver. FEBS Lett (1984) 1.24
Oxidation of human haemoglobin by copper. Mechanism and suggested role of the thiol group of residue beta-93. Biochem J (1977) 1.22
The serpin inhibitory mechanism is critically dependent on the length of the reactive center loop. J Biol Chem (2001) 1.22
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 (2001) 1.17
Genetics and respiratory disease. 2. Alpha 1-antitrypsin deficiency, cirrhosis and emphysema. Thorax (1998) 1.17
The significance of monoclonal gammopathy in a normal population. Aust N Z J Med (1971) 1.16
Basis of the defect in alpha-1-antitrypsin deficiency. Nature (1973) 1.16
A multiple-start Monte Carlo docking method. Proteins (1992) 1.15
Preparation and characterization of latent alpha 1-antitrypsin. J Biol Chem (1995) 1.15
Smoking related COPD and facial wrinkling: is there a common susceptibility? Thorax (2006) 1.15
Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp) J Clin Invest (1994) 1.14
Prevalence of antithrombin deficiency in the healthy population. Br J Haematol (1994) 1.13
Yeast KEX2 protease has the properties of a human proalbumin converting enzyme. Science (1987) 1.13
A new doubly substituted sickling haemoglobin: HbS-Oman. Br J Haematol (1989) 1.12
Conformational changes in serpins: II. The mechanism of activation of antithrombin by heparin. J Mol Biol (2000) 1.12