OBJECTIVE α-1 antitrypsin (AAT) is an anti-protease, anti-inflammatory and tissue-protective molecule. Normal circulating levels are <3.5 mg/dl and rise during pregnancy. Although AAT deficiency is associated with several pregnancy and placental disorders, little is known regarding AAT levels and preeclampsia. Since unopposed inflammation might contribute to preeclampsia, we studied whether pre...

In this issue the Journal is reprinting the Executive Summary of the new evidence-based standards regarding the management of individuals with alpha-1 antitrypsin (AAT) deficiency.1,2 Upon reading the summary, respiratory therapists (RTs), like all clinicians, will ask themselves several important questions about AAT deficiency, namely: 1. How well do I understand AAT deficiency? 2. What is the...

Tolerogenic IL-10-positive CCR7-positive dendritic cells (DC) promote T regulatory (Treg) cell differentiation upon CCR7-dependent migration to draining lymph nodes (DLN). Indeed, in human DC deficiencies, Treg levels are low. α-1 antitrypsin (AAT) has been shown to reduce inflammatory markers, promote a semimature LPS-induced DC phenotype, facilitate Treg expansion, and protect pancreatic isle...

AAT: a1-antitrypsin A lpha1-antitrypsin (AAT) is a serine protease inhibitor that prevents enzymatic degradation of normal human tissue. The Z allele of the AAT gene locus (designated PI ) is the most frequent deficient mutation, with the PiZZ phenotype responsible for 95% of severe AAT deficiency cases. Severe deficiency is often underdiagnosed and classically leads to pulmonary and hepatic di...

Alpha-1 antitrypsin (AAT) is an inhibitor of neutrophil elastase and a member of the serine proteinase inhibitor (serpin) superfamily, and little is known about its activity in sickle cell disease (SCD). We hypothesize that AAT may undergo changes in SCD because of the high oxidative stress and inflammation associated with the disease. We have found high AAT levels in SCD patients compared to c...

Chronic obstructive pulmonary disease (COPD) is a prevalent and preventable disease associated with high morbidity and mortality. Severe and intermediate alpha1-antitrypsin (AAT) deficiency (serum levels <11 and 11-20 micromol.L(-1), respectively) increase the risk of COPD in active smokers. However, little is known about the interaction of severe and intermediate AAT deficiency with modifiable...

BACKGROUND The major concept behind augmentation therapy with human α(1)-antitrypsin (AAT) is to raise the levels of AAT in patients with protease inhibitor phenotype ZZ (Glu342Lys)-inherited AAT deficiency and to protect lung tissues from proteolysis and progression of emphysema. OBJECTIVE To evaluate the short-term effects of augmentation therapy (Prolastin) on plasma levels of AAT, C-react...

Human alpha-1 antitrypsin (AAT) is an abundant serum protein present at a concentration of 1.0-1.5 g L(-1). AAT deficiency is a genetic disease that manifests with emphysema and liver cirrhosis due to the accumulation of a misfolded AAT mutant in hepatocytes. Lung AAT amount is inversely correlated with chronic obstructive pulmonary disease (COPD), a serious and often deadly condition, with inc...

Several isoenzymic forms of aspartate aminotransferase (AAT) have been identified in protein extracts from carrot (Daucus carota) cell suspension cultures. The cellular location of the major form (form I) of AAT in carrot suspension cultures was determined by heat inactivation, subcellular fractionation, and amino acid sequence analysis. In mammalian systems, there are two forms of AAT, a heat-...