A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
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Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational diseaseA pro-cathepsin L mutant is a luminal substrate for endoplasmic-reticulum-associated degradation in C. elegansDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencySerpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerolTherapeutic target-site variability in α1-antitrypsin characterized at high resolutionThe molecular interactions of heat shock protein 47 (Hsp47) and their implications for collagen biosynthesisNeuroserpin binds Abeta and is a neuroprotective component of amyloid plaques in Alzheimer diseaseMutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulumLatent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodiesDiscovery of an inhibitor of Z-alpha1 antitrypsin polymerizationPolymerization of plasminogen activator inhibitor-1.Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization.A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.Defining the mechanism of polymerization in the serpinopathies.The tempered polymerization of human neuroserpin.The high resolution crystal structure of a native thermostable serpin reveals the complex mechanism underpinning the stressed to relaxed transition.Small Molecule Probes That Perturb A Protein-protein Interface In AntithrombinMechanisms of liver injury relevant to pediatric hepatology.The roles of helix I and strand 5A in the folding, function and misfolding of α1-antitrypsin.Aeropin from the extremophile Pyrobaculum aerophilum bypasses the serpin misfolding trap.Protein misfolding and the serpinopathies.Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.The selective advantage of alpha1-antitrypsin deficiency.IgE-tailpiece associates with α-1-antitrypsin (A1AT) to protect IgE from proteolysis without compromising its ability to interact with FcεRI.Deficiency Mutations of Alpha-1 Antitrypsin. Effects on Folding, Function, and Polymerization.Smoothing a rugged protein folding landscape by sequence-based redesign.alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.α(1)-antitrypsin deficiency and inflammation.Unravelling the twists and turns of the serpinopathies.Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy.Advances in alpha-1-antitrypsin deficiency liver disease.Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.An antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein.An integrative approach combining ion mobility mass spectrometry, X-ray crystallography, and nuclear magnetic resonance spectroscopy to study the conformational dynamics of α1 -antitrypsin upon ligand binding.The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization.The structural basis of serpin polymerization studied by hydrogen/deuterium exchange and mass spectrometry.Loop-sheet mechanism of serpin polymerization tested by reactive center loop mutations.Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema.Maintenance of α(1)-antitrypsin activity by means of co-application of hypochlorous acid-scavengers in vitro and in the supernatant of polymorphonuclear leukocytes: as a basis for a new drug delivery approach.
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A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
description
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
im April 1999 veröffentlichter wissenschaftlicher Artikel
@de
scientific article published in Journal of Biological Chemistry
@en
wetenschappelijk artikel
@nl
наукова стаття, опублікована у квітні 1999
@uk
name
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@en
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@nl
type
label
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@en
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@nl
prefLabel
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@en
A Kinetic Mechanism for the Polymerization of α1-Antitrypsin
@nl
P2093
P2860
P356
P1476
A kinetic mechanism for the polymerization of alpha1-antitrypsin
@en
P2093
P R Elliott
P Sivasothy
R Mahadeva
P2860
P304
P356
10.1074/JBC.274.14.9548
P407
P50
P577
1999-04-01T00:00:00Z