(Q38092851)

28 August 2017

title

Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency (English)

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28 August 2017

David A Lomas

1

1 reference

28 August 2017

language of work or name

English

0 references

publication date

1 March 2013

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COPD: Journal of Chronic Obstructive Pulmonary Disease

28 August 2017

published in

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28 August 2017

volume

10 Suppl 1

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28 August 2017

page(s)

17-25

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Polymers and inflammation: disease mechanisms of the serpinopathies

28 August 2017

cites work

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Conformational pathology of the serpins: themes, variations, and therapeutic strategies

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The serpinopathies studying serpin polymerization in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The Electrophoretic α;1-Globulin Pattern of Serum in α;1-Antitrypsin Deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The native strains in the hydrophobic core and flexible reactive loop of a serine protease inhibitor: crystal structure of an uncleaved alpha1-antitrypsin at 2.7 A

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Structure of a serpin-protease complex shows inhibition by deformation

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The mechanism of Z alpha 1-antitrypsin accumulation in the liver

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Implications for function and therapy of a 2.9 A structure of binary-complexed antithrombin

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The Z type variation of human alpha 1-antitrypsin causes a protein folding defect

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The mechanism of alpha 1-antitrypsin polymerization probed by fluorescence spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Alpha 1-antitrypsin polymerization: a fluorescence correlation spectroscopic study.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Different conformational changes within the F-helix occur during serpin folding, polymerization, and proteinase inhibition

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Probing the local conformational change of alpha1-antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Crystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α1-Antitrypsin: Implications for Disease and Drug Design

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Structural dynamics associated with intermediate formation in an archetypal conformational disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Structural transition of alpha 1-antitrypsin by a peptide sequentially similar to beta-strand s4A.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Acid Denaturation of alpha1-antitrypsin: characterization of a novel mechanism of serpin polymerization.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Biological implications of a 3 A structure of dimeric antithrombin

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

A structural basis for loop C-sheet polymerization in serpins

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The active conformation of plasminogen activator inhibitor 1, a target for drugs to control fibrinolysis and cell adhesion

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

X-ray crystal structure of MENT: evidence for functional loop-sheet polymers in chromatin condensation

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Dynamic local unfolding in the serpin α-1 antitrypsin provides a mechanism for loop insertion and polymerization

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Defining the mechanism of polymerization in the serpinopathies

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Characterisation of serpin polymers in vitro and in vivo.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The inhibitory complex of human alpha 1-proteinase inhibitor and human leukocyte elastase is a neutrophil chemoattractant

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Oxidation of Z α1-antitrypsin by cigarette smoke induces polymerization: a novel mechanism of early-onset emphysema.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Modeling inherited metabolic disorders of the liver using human induced pluripotent stem cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Genetically distinct subsets within ANCA-associated vasculitis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The selective advantage of alpha1-antitrypsin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Neutrophilic inflammation and IL-8 levels in induced sputum of alpha-1-antitrypsin PiMZ subjects

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Antithrombin Phe229Leu: a new homozygous variant leading to spontaneous antithrombin polymerization in vivo associated with severe childhood thrombosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The molecular basis of alpha 1-antichymotrypsin deficiency in a heterozygote with liver and lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

A leucine-to-proline substitution causes a defective alpha 1-antichymotrypsin allele associated with familial obstructive lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Homozygous deficiency of heparin cofactor II: relevance of P17 glutamate residue in serpins, relationship with conformational diseases, and role in thrombosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Neuroserpin, a brain-associated inhibitor of tissue plasminogen activator is localized primarily in neurons. Implications for the regulation of motor learning and neuronal survival

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Association between conformational mutations in neuroserpin and onset and severity of dementia

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Neuroserpin mutation causes electrical status epilepticus of slow-wave sleep

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Inhibiting polymerization: new therapeutic strategies for Z alpha1-antitrypsin-related emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

How small peptides block and reverse serpin polymerisation.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Lack of effect of oral 4-phenylbutyrate on serum alpha-1-antitrypsin in patients with alpha-1-antitrypsin deficiency: a preliminary study

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Targeted gene correction of α1-antitrypsin deficiency in induced pluripotent stem cells

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

Pathogenic α1-Antitrypsin Polymers Are Formed by Reactive Loop-β-Sheet A Linkage

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

ANCA-associated vasculitis is linked to carriage of the Z allele of 1 antitrypsin and its polymers

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F15412555.2013.764401

10.3109/15412555.2013.764401

21 January 2018

Identifiers

DOI

1 reference

28 August 2017

1 reference