Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease (Q44478799)

3 December 2017

title

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

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3 December 2017

5

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3 December 2017

Helen Parfrey

1

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3 December 2017

Ravi Mahadeva

2

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3 December 2017

Neil A Ravenhill

3

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3 December 2017

Aiwu Zhou

4

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3 December 2017

Richard C Foreman

6

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3 December 2017

David A Lomas

7

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3 December 2017

language of work or name

English

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publication date

13 June 2003

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Journal of Biological Chemistry

3 December 2017

published in

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3 December 2017

volume

278

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3 December 2017

issue

35

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3 December 2017

page(s)

33060-33066

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3 December 2017

cites work

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Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (PiZZ).

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

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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

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A 2.1 A resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops

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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

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

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

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Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants

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

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The serpin inhibitory mechanism is critically dependent on the length of the reactive center loop

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

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

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Influenza hemagglutinin is spring-loaded by a metastable native conformation

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Regulation of protein function by native metastability

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Cavities of alpha(1)-antitrypsin that play structural and functional roles

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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

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Beta-sheet breaker peptides inhibit fibrillogenesis in a rat brain model of amyloidosis: implications for Alzheimer's therapy

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Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

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

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A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

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

21 January 2018

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Amino acid substitution Glu→Lys in α1-antitrypsin PiZ

21 January 2018

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The natural history of liver disease in alpha 1-antitrypsin deficient children

21 January 2018

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Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency

21 January 2018

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

21 January 2018

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Structural explanation for the deficiency of S alpha 1-antitrypsin

21 January 2018

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A thermostable mutation located at the hydrophobic core of alpha 1-antitrypsin suppresses the folding defect of the Z-type variant

21 January 2018

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Inhibitory mechanism of serpins. Mobility of the C-terminal region of the reactive-site loop

21 January 2018

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Metastability in the inhibitory mechanism of human alpha1-antitrypsin

21 January 2018

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Distribution of the native strain in human alpha 1-antitrypsin and its association with protease inhibitor function

21 January 2018

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Xenopusoocytes can synthesise but do not secrete the Z variant of human α1-antitrypsin

21 January 2018

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Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency

21 January 2018

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Arrest of beta-amyloid fibril formation by a pentapeptide ligand

21 January 2018

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21 January 2018

Identifiers

DOI

10.1074/JBC.M302646200

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3 December 2017

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