A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency. (Q34123062)

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6 February 2020

title

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

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6 February 2020

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6 February 2020

Bibek Gooptu

6

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6 February 2020

Jeffrey Teckman

11

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6 February 2020

Stefan Marciniak

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6 February 2020

author name string

Juan Pérez

2

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6 February 2020

Ugo I Ekeowa

3

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6 February 2020

Nedim Hadzic

4

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6 February 2020

Noor Kalsheker

5

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6 February 2020

Bernard Portmann

7

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6 February 2020

Didier Belorgey

8

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6 February 2020

Marian Hill

9

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6 February 2020

Susan Chambers

10

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6 February 2020

Graeme J Alexander

12

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6 February 2020

David A Lomas

14

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language of work or name

English

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publication date

1 September 2010

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6 February 2020

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6 February 2020

volume

52

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issue

3

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6 February 2020

page(s)

1078-1088

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

6 February 2020

cites work

1 reference

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

21 January 2018

1 reference

Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency

21 January 2018

1 reference

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

21 January 2018

1 reference

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

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Probing neuroserpin polymerization and interaction with amyloid-beta peptides using single molecule fluorescence.

21 January 2018

1 reference

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

21 January 2018

1 reference

Differential detection of PAS-positive inclusions formed by the Z, Siiyama, and Mmalton variants of alpha1-antitrypsin.

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Quantitative isolation of alphalAT mutant Z protein polymers from human and mouse livers and the effect of heat

21 January 2018

1 reference

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

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

21 January 2018

1 reference

Molecular characterization of the new defective P(brescia) alpha1-antitrypsin allele.

21 January 2018

1 reference

Novel serpinopathy in rat kidney and pancreas induced by overexpression of megsin

21 January 2018

1 reference

Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol

21 January 2018

1 reference

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

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

21 January 2018

1 reference

Detection of circulating and endothelial cell polymers of Z and wild type alpha 1-antitrypsin by a monoclonal antibody

21 January 2018

1 reference

inferred from DOI database lookup

7 January 2021

based on heuristic

Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin

1 reference

inferred from DOI database lookup

7 January 2021

based on heuristic

Preparation and characterization of latent alpha 1-antitrypsin

1 reference