α1-antitrypsin, M
Quantum proteolysis by neutrophils: implications for pulmonary emphysema in α1 antitrypsin deficiency
... tions demonstrated that there was an inverse and strik- ingly nonlinear relationship between the size of the quantum proteolytic events and the serum AAT con- centration. Small changes in serum AAT below 10 µM were ...
9
Characterising the association of latency with α1 antitrypsin polymerisation using a novel monoclonal antibody
... in blocking buffer (1:200 for plasma samples) and incubated for 2 h. The wells were washed and incubated with the 1C12 MAb culture medium supernatant diluted in blocking buffer (1:50) for 2 h. Bound 1C12 antibody was ...
12
Endoplasmic reticulum stress in the lung : lessons from α1-antitrypsin deficiency
... For many years, the protease-antiprotease imbalance in the lungs was thought to be the main explanation for early-onset emphysema in patients with Z α 1 -antitrypsin deficiency. However, over the years, α 1 ...
232
Deconvolution of ion mobility mass spectrometry arrival time distributions using a genetic algorithm approach: application to α1 antitrypsin peptide binding
... energies. This confirms that the reduction in conformational lability (increase in kinetic stability) induced by TTAI binding previously described at higher energies by CIU-IM-MS holds true at the lowest energy (most ...
28
Therapeutic target site variability in α1 antitrypsin characterized at high resolution
... previously. In the other case (PDB entry 2qug; Pearce et al., 2008), similarly to the 2.1 A ˚ resolution structure (PDB entry 1hp7; Kim et al., 2001), this water is not seen and the canonical conformation of these ...
8
α1-Antitrypsin deficiency
... More than 100 different genetic variants of AAT have been identified. Differences in the speed of migration on gel electrophoresis have been used to identify the protein variants. Normal individuals are homozygous for ...
6
Prolastin, a pharmaceutical preparation of purified human α1-antitrypsin, blocks endotoxin-mediated cytokine release
... α1-antitrypsin (AAT) is a glycoprotein, which is the major inhibitor of neutrophil elastase and proteinase 3 [1,2]. AAT is mainly produced in liver cells, but also in extrahe- patic cells, such as ...
11
α1-antitrypsin mitigates NLRP3-inflammasome activation in amyloid β1–42-stimulated murine astrocytes
... After 3 h/6 h of stimulation, medium was removed and peqGOLD TriFast™ (Peqlab, Germany) was added to cells. RNA was isolated using phenol-chloroform extraction method as previously described [53]. Afterwards, RNA con- ...
15
Heteropolymerization of S, I, and Z α1 antitrypsin and liver cirrhosis
... 22. Eldering, E., Verpy, E., Roem, D., Meo, T., and Tosi, M. 1995. COOH-ter- minal substitutions in the serpin C1 inhibitor that cause loop overin- sertion and subsequent multimerization. J. Biol. Chem. ...
9
Danazol induced Augmentation of Serum α1 Antitrypsin Levels in Individuals with Marked Deficiency of this Antiprotease
... protease-antiprotease homeostasis within the lung parenchyma. Because danazol, an impeded androgen, elevates levels of C1 inhibitor in patients deficient of that serum antiprotease, we hypothesized that this agent might ...
7
Matrix metalloproteinase-9 predicts pulmonary status declines in α1-antitrypsin deficiency
... Our study was not designed to establish a causative role of MMP-9 in antitrypsin deficient emphysema pro- gression. Evidence from other studies suggest that MMP-9 degrades basement membrane type IV collagen, ...
11
α1-antitrypsin promotes SPLUNC1-mediated lung defense against Pseudomonas aeruginosa infection in mice
... 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 ...
7
Liver injury in α1 antitrypsin deficiency: an aggregated protein induces mitochondrial injury
... 22. Werner, E.D., Brodsky, J.L., and McCracken, A.A. 1996. Proteasome-depend- ent endoplasmic reticulum-associated protein degradation: an unconven- tional route to a familiar fate. Proc. Natl. Acad. Sci. USA. ...
6
Acute-Phase Protein α1-Antitrypsin Inhibits Neutrophil Calpain I and Induces Random Migration
... A rapid recruitment of neutrophils to sites of injury or infection is a hallmark of the inflammatory response and is required for ef- fective host defense against pathogenic stimuli. However, neutrophil-mediated ...
10
Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy
... Another landmark study in the area of AATD research was published by the Alpha-1-Antitrypsin Deficiency Registry Study Group [8]. In this study of 1129 individuals with severe AATD enrolled in the US National ...
6
Heparan sulfate and syndecan 1 are essential in maintaining murine and human intestinal epithelial barrier function
... Increased presNonstandard abbreviations used: AAT, α1-antitrypsin; CDG, congenital disorders of glycosylation; dp, degree of polymerization; 2/3-DS-H, 2,3-de-O-sulfated heparin; GAG, gly[r] ...
11
Targeted screening programmes in COPD: how to identify individuals with α1-antitrypsin deficiency
... for patients with AATD. The initiative involved workshops covering practical details, clinical issues and laboratory procedures, and also personal medical training for physicians, physiotherapists and nurses. Educational ...
6
α1 Antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
... during episodes of inflammation as part of the acute phase response. At these times the formation of poly- mers is likely to overwhelm the degradative pathway, thereby exacerbating the formation of hepatic inclu- sions ...
7
Regulation of Autophagy by α1-Antitrypsin: “A Foe of a Foe Is a Friend”
... Autophagy is involved in both the cell protective and the cell death process but its mechanism is largely unknown. The pres- ent work unravels a novel intracellular mechanism by which the serpin α 1 -antitrypsin ...
10
α1-Antitrypsin Deficiency in Early Childhood
... The clinical and laboratory results during the first half year of life made it convenient to divide the children into the following groups: group I includes infants with increased levels[r] ...
6