Results 31 to 40 of about 3,462 (201)

Functional characterization of a SNP (F51S) found in human alpha 1‐antitrypsin

Molecular Genetics & Genomic Medicine, 2019
Background Alpha 1‐antitrypsin (A1AT) deficiency is related to lung and liver diseases, including pulmonary emphysema and liver cirrhosis in humans. Genetic variations including single nucleotide polymorphisms (SNPs) of SERPINA1 are responsible for A1AT ...
Hong‐Nhung Trinh, Sei‐Heon Jang, ChangWoo Lee   +2 more
doaj   +1 more source

Alpha-1 antitrypsin supplementation improves alveolar macrophages efferocytosis and phagocytosis following cigarette smoke exposure. [PDF]

PLoS ONE, 2017
Cigarette smoking (CS), the main risk factor for COPD (chronic obstructive pulmonary disease) in developed countries, decreases alveolar macrophages (AM) clearance of both apoptotic cells and bacterial pathogens.
Karina A Serban, Daniela N Petrusca, Andrew Mikosz, Christophe Poirier, Angelia D Lockett, Lauren Saint, Matthew J Justice, Homer L Twigg, Michael A Campos, Irina Petrache   +9 more
doaj   +1 more source

Alpha-1 Antitrypsin Deficiency Targeted Testing and Augmentation Therapy: A Canadian Thoracic Society Clinical Practice Guideline

Canadian Respiratory Journal, 2012
Alpha-1 antitrypsin (A1AT) functions primarily to inhibit neutrophil elastase, and deficiency predisposes individuals to the development of chronic obstructive pulmonary disease (COPD).
DD Marciniuk, P Hernandez, M Balter, J Bourbeau, KR Chapman, GT Ford, JL Lauzon, F Maltais, DE O’Donnell, D Goodridge, C Strange, AJ Cave, K Curren, S Muthuri, Canadian Thoracic Society COPD Clinical Assembly Alpha-1 Antitrypsin Deficiency Expert Working Group   +14 more
doaj   +1 more source

Assessment of liver fibrosis by transient elastography (Fibroscan®) in patients with A1AT deficiency [PDF]

Clinics and Research in Hepatology and Gastroenterology, 2019
Alpha-1-antitrypsin deficiency (A1ATD) is a common genetic condition which predisposes to emphysema and liver disorders. It is estimated that 10-15% of homozygous individuals for the Z allele (PiZZ) may develop liver fibrosis. The optimal modalities to detect liver disease in PiZZ adult patients need to be defined. The aim of this prospective study was
Chapuis-Cellier, C., Lachaux, A., Mornex, Jean-François, Guillaud, O., Dumortier, J., Traclet, J., Restier, L., Joly, P.   +7 more
openaire   +4 more sources

A1AT trafficking across cultured pulmonary endothelial and epithelial bilayers.

, 2014
(A) Co-culture schematic showing pulmonary epithelial cells cultured on the bottom of the transwell membrane and pulmonary endothelial cells cultured on the top. Endothelial cells only were exposed to A1AT.
Houssam Oueini (553977), Daniela N. Petrusca (553980), Kelly S. Schweitzer (553982), Karina A. Serban (553981), Mary Beth Brown (553974), Miryam A. Fragoso (129475), Irina Petrache (553985), Michael Campos (553984), Robert G. Presson Jr. (553983), Angelia D. Lockett (553973), Nieves Santos-Falcon (553975), Esther Bolanis (553979), Amber J. Oberle (553978), Natalia I. Rush (553976)   +13 more
core   +1 more source

Alpha-1-antitripsin deficiency: the need of a new diagnostic algorithm for improving the diagnostic ability of perinatologists and pediatricians

Journal of Pediatric and Neonatal Individualized Medicine, 2015
Caution should be taken in considering immunoelectrofocusing (IEF) as the best method for the diagnosis of alpha-1-antitrypsin (A1AT) deficiency, particularly in some population, including Sardinians, in which a M-like variant represents the most ...
Gavino Faa
doaj   +1 more source

Polarity of A1AT trafficking across cultured pulmonary endothelial monolayers.

, 2014
(A) Fraction (%) of FITC-Dextran (Dex) of 20 kDa (light gray bars) or 250 kDa (dark gray bars) or of A1AT (100 µg/mL; black bars) that crossed confluent endothelial cell monolayers grown on 0.4 µm transwells.
Houssam Oueini (553977), Daniela N. Petrusca (553980), Kelly S. Schweitzer (553982), Karina A. Serban (553981), Mary Beth Brown (553974), Miryam A. Fragoso (129475), Irina Petrache (553985), Michael Campos (553984), Robert G. Presson Jr. (553983), Angelia D. Lockett (553973), Nieves Santos-Falcon (553975), Esther Bolanis (553979), Amber J. Oberle (553978), Natalia I. Rush (553976)   +13 more
core   +1 more source

a-d. Molecular profile of heat-treated purified A1AT.

, 2015
a. Purified plasma A1AT (2.5 mg/ml) was heated at 60°C and aliquots were removed at indicated time points for analysis by a non-denaturing PAGE followed by Western blotting using rabbit polyclonal anti-A1AT antibody. b.
Marc Dresel (687824), Marina Gorrini (783011), Maurizio Luisetti (142908), Stefania Ottaviani (142904), Luana Cavallon (783013), Sabina Janciauskiene (264288), Ilaria Ferrarotti (142893), Rembert Koczulla (783009), Joanna Chorostowska-Wynimko (783014), Giorgia Sala (783012), Raffaele Baldo (783010), Tobias Welte (87188), Maria Teresa Trevisan (783008), Beata Poplawska-Wisniewska (783007), Janine Koepke (687823)   +14 more
core   +1 more source

Validation of the candidate substrate A1AT of HTRA1.

, 2014
Purified A1AT from human plasma was incubated with HTRA1 at 37°C for 17 hours. (A) 2-D gel of A1AT in the absence of HTRA1 incubation time 0 h and SDS-PAGE of A1AT and HTRA1. The A1AT preparation contains only very small amount of cleaved A1AT.
Violette Frochaux (647168), Diana Hildebrand (140686), Hartmut Schlüter (140692), Anja Talke (647169), Michael W. Linscheid (118496)   +4 more
core   +1 more source

FBG1 knockout increases A1AT-Z accumulation in the mouse liver.

, 2015
Livers were harvested from 6-month-old FBG1 +/0 A1AT-Z tg/0 and FBG1 +/+ A1AT-Z tg/0 mice. Total cell lysates were separated on SDS-PAGE gels and probed with the following antibodies against A1AT and stained for total protein (loading control) (n = 3 ...
Kevin A. Glenn (785982), Katherine N. Gibson-Corley (283085), Hsiang Wen (785981), John H. Wen (785980)   +3 more
core   +1 more source