(Q37882046)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21624056 AND SRC:MED&resulttype=core&format=json

2 February 2020

review article

1 reference

title

Unravelling the twists and turns of the serpinopathies (English)

1 reference

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2 February 2020

2

1 reference

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2 February 2020

Stefan Marciniak

11

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2 February 2020

Benoit D Roussel

1

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2 February 2020

Sheikh T. Rashid

9

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2 February 2020

10

Damian C Crowther

1 reference

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2 February 2020

author name string

Ugo I Ekeowa

3

1 reference

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2 February 2020

Didier Belorgey

4

1 reference

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2 February 2020

Imran Haq

5

1 reference

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2 February 2020

Adriana Ordóñez

6

1 reference

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2 February 2020

Antonina J Kruppa

7

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2 February 2020

Annelyse Duvoix

8

1 reference

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2 February 2020

David A Lomas

12

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2 February 2020

language of work or name

English

0 references

publication date

20 June 2011

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2 February 2020

1 reference

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2 February 2020

volume

278

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2 February 2020

issue

20

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2 February 2020

page(s)

3859-3867

1 reference

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Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

2 February 2020

cites work

1 reference

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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

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Structure of a serpin-protease complex shows inhibition by deformation

21 January 2018

1 reference

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Active site distortion is sufficient for proteinase inhibition by serpins: structure of the covalent complex of alpha1-proteinase inhibitor with porcine pancreatic elastase

21 January 2018

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Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy

21 January 2018

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

21 January 2018

1 reference

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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

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Familial encephalopathy with neuroserpin inclusion bodies

21 January 2018

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Familial dementia caused by polymerization of mutant neuroserpin

21 January 2018

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Cognitive deficits associated with a recently reported familial neurodegenerative disease: familial encephalopathy with neuroserpin inclusion bodies

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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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

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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

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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Neuroserpin mutation causes electrical status epilepticus of slow-wave sleep

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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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 disease

21 January 2018

1 reference

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What do dysfunctional serpins tell us about molecular mobility and disease?

21 January 2018

1 reference

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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/10.1111/J.1742-4658.2011.08201.X

Drosophila necrotic mutations mirror disease-associated variants of human serpins

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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Probing the local conformational change of alpha1-antitrypsin

21 January 2018

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Dimers initiate and propagate serine protease inhibitor polymerisation.

21 January 2018

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

21 January 2018

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

21 January 2018

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The Z type variation of human α1-antitrypsin causes a protein folding defect

21 January 2018

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A novel transcription factor regulates expression of the vacuolar H -ATPase B2 subunit through AP-2 sites during monocytic differentiation

21 January 2018

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

21 January 2018

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Defining the mechanism of polymerization in the serpinopathies

21 January 2018

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Biological implications of a 3 A structure of dimeric antithrombin

21 January 2018

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A structural basis for loop C-sheet polymerization in serpins

21 January 2018

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α1-Antitrypsin Polymerisation Can Occur by both Loop A and C Sheet Mechanisms

21 January 2018

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

21 January 2018

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Polymerization of plasminogen activator inhibitor-1.

21 January 2018

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

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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Refolding and Polymerization Pathways of Neuroserpin

21 January 2018

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Characterisation of serpin polymers in vitro and in vivo.

21 January 2018

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Molecular contortionism - on the physical limits of serpin 'loop-sheet' polymers

21 January 2018

1 reference

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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

1 reference

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

21 January 2018

1 reference

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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

1 reference

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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

1 reference

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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

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

Sugar and alcohol molecules provide a therapeutic strategy for the serpinopathies that cause dementia and cirrhosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

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/10.1111/J.1742-4658.2011.08201.X

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

Lithium induces autophagy by inhibiting inositol monophosphatase

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

Alpha1-antitrypsin deficiency and autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1111/J.1742-4658.2011.08201.X

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

21 January 2018

1 reference

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