Plants are increasingly being used as an expression system for complex recombinant proteins. However, our limited knowledge of the intrinsic factors that act along the secretory pathway, which may compromise product integrity, renders process design difficult in some cases. Here, we pursued the recombinant expression of the human protease inhibitor a1-antitrypsin (A1AT) in Nicotiana benthamiana...

BACKGROUND: 1-Antitrypsin (A1AT) deficiency is currently detectable by protein immunoassay, phenotyping, and genotyping of the S and Z mutations, but no fully automated method for standard biochemical analyzers is yet available. Here, we present a method that measures the antitryptic activity in serum. This method is rapid, automated, and allows the easy evaluation of a large cohort of patients.

α1-antitrypsin deficiency is the most widely recognised genetic disorder causing chronic obstructive pulmonary disease (COPD). Mutant Z α1-antitrypsin expression has previously been linked to intracellular accumulation and polymerisation of this proteinase inhibitor. Subsequently, this has been described to underlie an exaggerated endoplasmic reticulum stress response and enhanced nuclear facto...

BACKGROUND Alpha 1-antitrypsin (A1AT) is a 52 kDa serine protease inhibitor produced largely by hepatocytes but also by mononuclear phagocytes. A1AT chiefly inhibits neutrophil elastase and proteinase-3 but has also been reported to have immune modulatory functions including the ability to inhibit caspases. Its clinical availability for infusion suggests that A1AT therapy might modulate caspase...

Background The human designer cell line AGE1.HN represents a promising production system for biopharmaceuticals, particularly for those needing human-type post-translational modifications [1,2]. For further rational improvement of the cell line and the cultivation process a detailed understanding of the metabolism and metabolic changes during glycoprotein production is desirable. Metabolism and...

evidence acquisition elevated serum leptin levels are regarded as an established marker of obesity. it is also reported that obese asthmatic patients have maximum serum leptin levels compared to other groups such as non-obese asthmatics, and normal obese and non obese subjects without asthma. in addition to having an appetite suppressing effect, leptin also regulates certain acute-phase protein...

The last two decades efforts have been made in investigation of genes that encode proteins involved in pathogenesis of emphysema and chronic obstructive pulmonary disease (COPD). So far, SERPINA1 gene which encodes protein alpha-1-antitrypsin (A1AT) is the only defined genetic risk factor associated with early development of emphysema. The A1AT is dominant protein of 1 electrophoretic fraction...

Introduction: α1-antitrypsin deficiency (α1-ATD) is one of the most common genetic disorders in white race, a usual cause of liver disease in children, and hepatopulmonary involvement in children and adult. The aim of this case description is presenting a child with early lung disease without liver parenchymal disorder. Case presentation: We describe a 13 year old boy because of exertional dysp...