Alpha1-antitrypsin deficiency: current perspective on research, diagnosis, and management (Q37018904)

17 August 2017

2 references

24 March 2019

22 April 2019

Alpha1-antitrypsin deficiency: current perspective on research, diagnosis, and management (English)

1 reference

alpha-1-antitrypsin deficiency

17 August 2017

2 references

24 March 2019

22 April 2019

0 references

Jan Stolk

1

1 reference

17 August 2017

Niels Seersholm

series ordinal

2

1 reference

17 August 2017

Noor Kalsheker

series ordinal

3

1 reference

17 August 2017

language of work or name

English

0 references

publication date

1 January 2006

1 reference

International Journal of Chronic Obstructive Pulmonary Disease

17 August 2017

3 references

17 August 2017

24 March 2019

22 April 2019

1

1 reference

17 August 2017

2

1 reference

17 August 2017

151-160

1 reference

The electrophoretic α1-globulin pattern of serum in α1-antitrypsin deficiency. 1963.

17 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Short-term variability of biomarkers of proteinase activity in patients with emphysema associated with type Z alpha-1-antitrypsin deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Prolastin, a pharmaceutical preparation of purified human alpha1-antitrypsin, blocks endotoxin-mediated cytokine release

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Haplotypes of the alpha-1 antitrypsin gene in healthy controls and Z deficiency patients.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

High levels of desmosines in urine and plasma of patients with pseudoxanthoma elasticum

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Correlation between annual change in health status and computer tomography derived lung density in subjects with alpha1-antitrypsin deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Enhancement of endoplasmic reticulum (ER) degradation of misfolded Null Hong Kong alpha1-antitrypsin by human ER mannosidase I.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Differential requirements of novel A1PiZ degradation deficient (ADD)genes in ER-associated protein degradation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Genomewide linkage analysis of quantitative spirometric phenotypes in severe early-onset chronic obstructive pulmonary disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. NHLBI/WHO Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop summary

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Conformational properties of serine proteinase inhibitors (serpins) confer multiple pathophysiological roles

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Neonatal alpha1-antitrypsin screening: parents' views and reactions 20 years after the identification of the deficiency state

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Sustained secretion of human alpha-1-antitrypsin from murine muscle transduced with adeno-associated virus vectors

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Oncostatin M is a potent stimulator of alpha1-antitrypsin secretion in lung epithelial cells: modulation by transforming growth factor-beta and interferon-gamma

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Young adults with alpha 1-antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

The fetal liver in PiZZ alpha-1-antitrypsin deficiency: a report of five cases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Results of a case-detection programme for alpha1-antitrypsin deficiency in COPD patients

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Pattern of emphysema distribution in alpha1-antitrypsin deficiency influences lung function impairment

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Emphysema lung tissue gene expression profiling

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Influence of calibration on densitometric studies of emphysema progression using computed tomography

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Role of human neutrophil peptides in lung inflammation associated with α1-antitrypsin deficiency

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

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

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Alpha1-antitrypsin deficiency: a report from the 2nd meeting of the Alpha One International Registry, Rapallo (Genoa, Italy), 2001.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Genetic Loci Influencing Lung Function

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2706616

Prognosis of alpha-1-antitrypsin deficiency-related liver disease in the era of paediatric liver transplantation

4 December 2018

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Urinary desmosine excretion in smokers with and without rapid decline of lung function: the Normative Aging Study

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Gene regulation of the serine proteinase inhibitors α 1 -antitrypsin and α 1 -antichymotrypsin

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Update on research, diagnosis and management of alphal-antitrypsin deficiency

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Genome-wide linkage analysis of bronchodilator responsiveness and post-bronchodilator spirometric phenotypes in chronic obstructive pulmonary disease

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Less childhood obesity--less persistence of wheeze in teenage girls and boys?

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Globular clusters, Hipparcos, and the age of the galaxy

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Adolescents with alpha1-antitrypsin deficiency have high alpha2-macroglobulin and low neutrophil lipocalin and elastase levels in plasma

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor

31 March 2020

1 reference

https://api.crossref.org/works/10.2147/COPD.2006.1.2.151

Elastin and collagen degradation products in urine of smokers with and without chronic obstructive pulmonary disease

31 March 2020

1 reference

12 December 2020

The liver in adolescents with α-antitrypsin deficiency

1 reference

12 December 2020

Alpha 1-antitrypsin deficiency: memorandum from a WHO meeting

1 reference

12 December 2020

Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group

1 reference

12 December 2020

10.2147/COPD.2006.1.2.151

Identifiers

DOI

1 reference

17 August 2017

1 reference

17 August 2017

PubMed publication ID

18046892

1 reference