(Q36378692)

13 August 2017

1 reference

title

The selective advantage of alpha1-antitrypsin deficiency (English)

1 reference

13 August 2017

David A Lomas

1

1 reference

PubMed ID

16439713

13 August 2017

language of work or name

English

0 references

publication date

26 January 2006

1 reference

American Journal of Respiratory and Critical Care Medicine

13 August 2017

1 reference

13 August 2017

173

1 reference

13 August 2017

10

1 reference

13 August 2017

1072-1077

1 reference

Estimated numbers and prevalence of PI*S and PI*Z alleles of alpha1-antitrypsin deficiency in European countries.

13 August 2017

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Alpha 1-antitrypsin-deficient variant Siiyama (Ser53[TCC] to Phe53[TTC]) is prevalent in Japan. Status of alpha 1-antitrypsin deficiency in Japan

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Prevalence and phenotype of subjects carrying rare variants in the Italian registry for alpha1-antitrypsin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

DNA restriction fragments associated with alpha 1-antitrypsin indicate a single origin for deficiency allele PI Z.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Alpha-1-antitrypsin PI phenotypes S and Z in Europe: an analysis of the published surveys

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Factors associated with advanced liver disease in adults with alpha1-antitrypsin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Emphysema lung tissue gene expression profiling

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Sputum chemotactic activity in chronic obstructive pulmonary disease: effect of alpha(1)-antitrypsin deficiency and the role of leukotriene B(4) and interleukin 8

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Expression of the alpha-1-antitrypsin gene in mononuclear phagocytes of normal and alpha-1-antitrypsin-deficient individuals

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Divergent effects of alpha1-antitrypsin on neutrophil activation, in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

A family study of the variability of pulmonary function in alpha 1-antitrypsin deficiency. Quantitative phenotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

alpha 1-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha 1-antitrypsin administration and liver transplantation

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

The global smoking epidemic: a history and status report

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Dissecting glycoprotein quality control in the secretory pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

Protease inhibitor (Pi) locus, fertility and twinning

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200511-1797PP

10.1164/RCCM.200511-1797PP

21 January 2018

Identifiers

DOI

1 reference

13 August 2017

1 reference

PubMed ID

16439713