BACKGROUND Pseudomonas aeruginosa (PA) infection is involved in various lung diseases such as cystic fibrosis and chronic obstructive pulmonary disease. However, treatment of PA infection is not very effective in part due to antibiotic resistance. α1-antitrypsin (A1AT) has been shown to reduce PA infection in humans and animals, but the underlying mechanisms remain unclear. The goal of our stud...

Hepatitis C virus (HCV) infection represents a serious health problem. The -174 G/C mutation in the pro inflammatory cytokine interleukin-6 (IL-6) is associated with developing liver diseases. Likewise, the S and Z mutations in the serine protease inhibitor α1-antitrypsin (A1AT) are associated with pulmonary emphysema and/or liver cirrhosis. We explored the distribution of the single nucleotide...

In vitro-transcribed (IVT) mRNA has come into focus in recent years as a potential therapeutic approach for the treatment of genetic diseases. The nebulized formulations IVT-mRNA-encoding alpha-1-antitrypsin (A1AT-mRNA) would be highly acceptable and tolerable remedy protein replacement therapy deficiency future. Here we show that lipoplexes containing A1AT-mRNA prepared optimum conditions coul...

INTRODUCTION Chronic obstructive pulmonary disease (COPD) has been recently defined as a systemic pulmonary inflammatory disease, and congenital α1 antitrypsin deficiency is one of the well-established genetic risk factors for chronic obstructive pulmonary disease. The aim of our study was to evaluate the possible associations of α1 antitrypsin with inflammatory markers - CRP, sCD14, TNF-α, sTN...

www.thelancet.com Published online May 28, 2015 http://dx.doi.org/10.1016/S0140-6736(15)60036-8 1 α1 antitrypsin is a serine antiprotease secreted by the liver. Its main function is inhibition of neutrophil elastase, a non-specifi c protease released by activated or dying neutrophils. In individuals with healthy concentrations of α1 antitrypsin, the fragile alveolar structures of the lung paren...

The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener's granulomatosis). The aim of our study was to e...

The patient’s serum -1 globulins were reduced at 1.3 g/L (reference interval, 2.1–3.5 g/L). Potential causes include malnutrition, excessive excretion, decreased production, and 1-antitrypsin (A1AT) deficiency. The clinical history was consistent with A1AT deficiency (1–3 ). The diagnosis should be confirmed by direct quantification of circulating A1AT by immunoassay and confirmation of the pre...

Liver disease due to alpha-1-antitrypsin deficiency (A1ATD) is associated with hepatic iron overload in a subgroup of patients. The underlying cause for this association is unknown. The aim of the present study was to define the genetics of this correlation and the effect of alpha-1-antitrypsin (A1AT) on the expression of the iron hormone hepcidin. Full exome and candidate gene sequencing were ...

OBJECTIVE To investigate the relationship between alpha 1-antitrypsin (A1AT), adiponectin, leptin, blood glucose, and insulin protein levels in human serum and obesity. METHODS Forty-eight subjects with normal blood glucose levels and average age of 32.4+/-5.1 years were enrolled in this study. The study took place in the Reproductive Center, The Second Hospital of Jilin University, Changchun...