Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy (Q24554310)

Journal of Clinical Investigation

Europe PubMed Central

title

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

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author name string

David A Lomas

series ordinal

1

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Ravi Mahadeva

series ordinal

2

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language of work or name

English

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publication date

December 2002

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published in

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volume

110

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issue

11

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page(s)

1585-90

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cites work

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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

24 March 2017

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

24 March 2017

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24 March 2017

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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

24 March 2017

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Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis

24 March 2017

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The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes

27 September 2017

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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

27 September 2017

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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

27 September 2017

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

27 September 2017

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27 September 2017

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27 September 2017

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30 May 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

30 May 2018

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

28 November 2018

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Lung lavage fluid from patients with alpha 1-proteinase inhibitor deficiency or chronic obstructive bronchitis: anti-elastase function and cell profile

28 November 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

28 November 2018

1 reference

12 December 2020

Soluble aggregates of the human PiZ alpha 1-antitrypsin variant are degraded within the endoplasmic reticulum by a mechanism sensitive to inhibitors of protein synthesis

1 reference

12 December 2020

Structural explanation for the deficiency of S alpha 1-antitrypsin

1 reference

12 December 2020

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

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12 December 2020

Identifiers

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release_px445w65tffdnm72lsn7g7ume4

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Fatcat

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24 November 2022

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PubMed publication ID

12464660

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