(Q37855954)

24 August 2017

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title

α(1)-antitrypsin deficiency and inflammation (English)

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24 August 2017

2

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24 August 2017

Ugo I Ekeowa

1

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24 August 2017

David A Lomas

series ordinal

3

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24 August 2017

publication date

1 March 2011

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Expert Review of Clinical Immunology

24 August 2017

published in

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24 August 2017

volume

7

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24 August 2017

issue

2

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24 August 2017

page(s)

243-252

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Expression of the alpha 1-proteinase inhibitor gene in human monocytes and macrophages

24 August 2017

cites work

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Biosynthesis of alpha1-proteinase inhibitor by human lung-derived epithelial cells

21 January 2018

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The serpins are an expanding superfamily of structurally similar but functionally diverse proteins. Evolution, mechanism of inhibition, novel functions, and a revised nomenclature

21 January 2018

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Molecular mousetraps and the serpinopathies

21 January 2018

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Distribution of alpha1-antitrypsin PI S and PI Z frequencies in countries outside Europe: a meta-analysis

21 January 2018

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

21 January 2018

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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

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The structural basis of serpin polymerization studied by hydrogen/deuterium exchange and mass spectrometry

21 January 2018

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

21 January 2018

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

21 January 2018

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Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency

21 January 2018

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A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

21 January 2018

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Endoplasmic reticulum stress: cell life and death decisions.

21 January 2018

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Endoplasmic reticulum stress signaling in disease

21 January 2018

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Signal integration in the endoplasmic reticulum unfolded protein response

21 January 2018

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Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

21 January 2018

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The molecular aetiology of the serpinopathies

21 January 2018

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

21 January 2018

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Characterization of an ERAD gene as VPS30/ATG6 reveals two alternative and functionally distinct protein quality control pathways: one for soluble Z variant of human alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ.

21 January 2018

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Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease

21 January 2018

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

21 January 2018

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Aging as a catabolic malfunction

21 January 2018

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Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.

21 January 2018

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The ER-overload response: activation of NF-kappa B.

21 January 2018

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The evolution of vertebrate Toll-like receptors

21 January 2018

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Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency

21 January 2018

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Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

21 January 2018

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Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.

21 January 2018

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Interaction of the smooth endoplasmic reticulum and mitochondria

21 January 2018

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Endoplasmic reticulum stress-induced cell death mediated by the proteasome

21 January 2018

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Elastin fragments drive disease progression in a murine model of emphysema

21 January 2018

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

21 January 2018

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

21 January 2018

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Polymers and inflammation: disease mechanisms of the serpinopathies

21 January 2018

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Alpha1-antitrypsin protects beta-cells from apoptosis

21 January 2018

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alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis

21 January 2018

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A novel antiapoptotic role for alpha1-antitrypsin in the prevention of pulmonary emphysema

21 January 2018

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Evidence for unfolded protein response activation in monocytes from individuals with alpha-1 antitrypsin deficiency

21 January 2018

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IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA

21 January 2018

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The selective advantage of alpha1-antitrypsin deficiency

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Interleukin-8 processing by neutrophil elastase, cathepsin G and proteinase-3.

21 January 2018

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Converting enzyme-independent release of tumor necrosis factor alpha and IL-1beta from a stimulated human monocytic cell line in the presence of activated neutrophils or purified proteinase 3

21 January 2018

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

21 January 2018

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

21 January 2018

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Alpha1-antitrypsin deficiency

21 January 2018

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alpha1-Antitrypsin deficiency . 5: intravenous augmentation therapy: current understanding

21 January 2018

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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

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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

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

21 January 2018

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Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model

21 January 2018

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Defining the mechanism of polymerization in the serpinopathies

21 January 2018

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Mechanisms of emphysema in alpha1-antitrypsin deficiency: molecular and cellular insights

21 January 2018

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A polymorphism of the alpha1-antitrypsin gene represents a risk factor for liver disease

21 January 2018

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A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

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Pathogenesis of chronic obstructive pulmonary disease

21 January 2018

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21 January 2018

Identifiers

DOI

10.1586/ECI.10.95

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24 August 2017

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