Alpha-1-Antitrypsin Enhances Primary Human Macrophage Immunity Against Non-tuberculous Mycobacteria

Conclusion: AAT augments macrophage control of M. intracellulare infection through enhancing phagosome-lysosome fusion and autophagy.
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research

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Background: Alpha-1 antitrypsin (AAT) deficiency is a genetic disorder. Most of case of severe AAT are caused by Z and S mutations in the SERPINA1 gene, that reduces serum AAT concentrations till to 15% of normal levels. Mutations causing total absence of serum AAT (Null mutations) are very rare. Individuals homozygouse for Null mutations should be considered a subgroup at particularly high risk of emphysema, but their clinical phenotype is poorly described.Objectives: We would like to evaluate the differences between clinical history of AATD in subject with the most prevalent deficient genotype (PI*ZZ) and those with rare...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Molecular pathology and funct. genomics Source Type: research
Introduction: The deficit of alpha 1 antitrypsin (A1AT) is caused by mutations in the A1AT gene (long arm chromosome 14). The aim of this study is to analyze the prevalence of mutations in this gene in patients with pulmonary emphysema without severe A1AT deficiency (
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Monitoring airway disease Source Type: research
Conclusions: To the best of our knowledge, this is the first controlled study to assess the relationship between AATD and ascending aortic diameter, and suggests AATD is associated with accelerated aortic wall degeneration.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Genes and environment Source Type: research
Rationale: Alpha-1 Antitrypsin Deficiency (AATD) is a rare genetic disease that can lead to the emphysema. Evidence suggests that small airways (SA) disease precedes emphysema. We compared traditional lung function and SA tests to CT evidence of emphysema in AATD subjects with mild or no COPD.Methods: 70 non-smokers with severe (PiZZ) AATD underwent lung function testing and high resolution CT (HRCT) scan. They were categorised based on HRCT data (Gevenois, P.A., et al. ERJ 1995; 5: 843–848); No emphysema (PD15> -910HU, n=16), microscopic emphysema (PD15 -950 to -910HU, n=48) and macroscopic emphysema (PD15
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Respiratory function technologists/scient. Source Type: research
α1-Antitrypsin deficiency (AATD) is a genetic disorder that predisposes to the development of early pulmonary emphysema, especially in smokers. Episodes of exacerbations are frequent in patients with emphysema due to AATD and are associated with a deficient antiprotease screen in the airways compared with that of non-deficient COPD patients [1]. As a consequence, exacerbations have great impact on the evolution of the lung disease in AATD, measured in terms of decline in gas transfer [2], in health status [2, 3], and in lung function over time [4, 5].
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Editorials Source Type: research
Conclusions: We have identified a genetic signature that differentiates the different risk groups and the presence of emphysema and hepatopathy in AATD patients.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Molecular pathology and funct. genomics Source Type: research
This study included 9,053 Japanese participants (male 2,962; female 6,091), in whom 7,525 (male 2,397; female 5,128) were included in the longitudinal analysis. The change in FEV1 was calculated by dividing the difference between the baseline and follow-up by follow-up periods (years). The change in FEV1 was categorized into two groups: rapid decline (change in FEV1
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Clinical Problems Source Type: research
We report on the 6 &12-month results of the AATD arm of the EMPROVE study.20 AATD subjects on optimal medical management with hyperinflation, severe dyspnea were treated with SVS valves at 12 centers. Target lobe selection was done using high resolution computed tomography. Targeted lobe was a lower lobe in 85% and an upper lobe in 15% of patients.A mean of 4.4 valves per patient were used to isolate the target lobe, with an average procedure time of 20 minutes. Results are presented as baseline and mean changes in FEV1, Target Lobe Volume (TLV), disease specific quality of life measures – St. George’s Resp...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Interventional pulmonology Source Type: research
Abstract Although chronic obstructive pulmonary disease (COPD) risk is strongly influenced by cigarette smoking, genetic factors are also important determinants of COPD. In addition to Mendelian syndromes such as alpha-1 antitrypsin deficiency, many genomic regions that influence COPD susceptibility have been identified in genome-wide association studies. Similarly, multiple genomic regions associated with COPD-related phenotypes, such as quantitative emphysema measures, have been found. Identifying the functional variants and key genes within these association regions remains a major challenge. However, newly ide...
Source: Annual Review of Physiology - Category: Physiology Authors: Tags: Annu Rev Physiol Source Type: research
Authors: Pye A, Turner AM Abstract Introduction Alpha-1 antitrypsin deficiency (AATD) is most often associated with chronic lung disease, early onset emphysema and liver disease. The standard of care in lung disease due to AATD is alpha-1 antitrypsin augmentation but there are several new and emerging treatment options under investigation for both lung and liver manifestations. Areas covered We review therapeutic approaches to lung and liver disease in alpha-1 antitrypsin deficiency (AATD) and the agents in clinical development according to their mode of action. The focus is on products in clinical trials, but data...
Source: Expert Opinion on Investigational Drugs - Category: Drugs & Pharmacology Tags: Expert Opin Investig Drugs Source Type: research
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