Results 31 to 40 of about 3,774 (171)

Role of the P2 residue of human alpha 1-antitrypsin in determining target protease specificity. [PDF]

PLoS ONE, 2017
Alpha 1-antitrypsin (A1AT) is a serine protease inhibitor that mainly inhibits neutrophil elastase in the lungs. A variant of A1AT at the P1 position with methionine 358 to arginine (A1AT-Pittsburgh) is a rapid inhibitor of thrombin with greatly ...
Hye-Shin Chung, Ji-Sun Kim, Sang Mee Lee, Soon Jae Park   +3 more
doaj   +1 more source

Diagnosis and Management of Patients With α1-Antitrypsin (A1AT) Deficiency [PDF]

Clinical Gastroenterology and Hepatology, 2012
Alpha(1)-antitrypsin (A1AT) deficiency is an autosomal codominant disease that can cause chronic liver disease, cirrhosis, and hepatocellular carcinoma in children and adults and increases risk for emphysema in adults. The development of symptomatic disease varies; some patients have life-threatening symptoms in childhood, whereas others remain ...
David R, Nelson, Jeffrey, Teckman, Adrian M, Di Bisceglie, David A, Brenner   +3 more
openaire   +2 more sources

Therapy with plasma purified alpha1-antitrypsin (Prolastin®) induces time-dependent changes in plasma levels of MMP-9 and MPO. [PDF]

PLoS ONE, 2015
The common Z mutation (Glu342Lys) of α1-antitrypsin (A1AT) results in the polymerization and intracellular retention of A1AT protein. The concomitant deficiency of functional A1AT predisposes PiZZ subjects to early onset emphysema.
Janine Koepke, Marc Dresel, Severin Schmid, Timm Greulich, Björn Beutel, Bernd Schmeck, Claus Franz Vogelmeier, Sabina Janciauskiene, Andreas Rembert Koczulla   +8 more
doaj   +1 more source

P90 α-1 antitrypsin (a1AT) polymers cause extreme hepatocyte ageing [PDF]

Gut, 2011
Introduction a1AT, synthesised predominantly in the liver, is the archetypal inhibitor of the serpin protein family. a1AT deficiency is a common inherited disorder; Glu342Lys substitution causes abnormal folding of mutant protein, which may polymerise and aggregate in the endoplasmic reticulum.
M Mela, S Davies, S Verma, P Tachtatzis, E Miranda, D A Lomas, N Coleman, G Alexander   +7 more
openaire   +1 more source

Therapeutic Effects of α1-Antitrypsin on Psedumonas aeruginosa Infection in ENaC Transgenic Mice. [PDF]

PLoS ONE, 2015
Cystic fibrosis (CF) is a genetic disease with many airway pathological features, including aberrant epithelial sodium channel (ENaC) function, persistent Pseudomonas aeruginosa (PA) infection and neutrophil-dominant inflammation.
David P Nichols, Di Jiang, Carrie Happoldt, Reena Berman, Hong Wei Chu   +4 more
doaj   +1 more source

PROINFLAMMATORY CYTOKINE PROFILE IN PATIENTS WITH DIFFERENT ALPHA-1-ANTITRYPSIN PHENOTYPES

Медицинская иммунология, 2016
Alpha-1-antitrypsin (A1AT) exerts a wide spectrum of protective effects, being focused on reduction of secondary injury in inflammation. Moreover, A1AT inhibits some serine proteases, and down-regulates production of proinflammatory cytokines.
M. Yu. Pervakova, S. V. Lapin, E. A. Surkova, O. Yu. Tkachenko, A. I. Budkova, V. I. Guseva, O. N. Titova, V. L. Emanuel, Areg A. Totolian   +8 more
doaj   +1 more source

Linkage specific fucosylation of alpha-1-antitrypsin in liver cirrhosis and cancer patients: implications for a biomarker of hepatocellular carcinoma. [PDF]

PLoS ONE, 2010
We previously reported increased levels of protein-linked fucosylation with the development of liver cancer and identified many of the proteins containing the altered glycan structures. One such protein is alpha-1-antitrypsin (A1AT).
Mary Ann Comunale, Lucy Rodemich-Betesh, Julie Hafner, Mengjun Wang, Pamela Norton, Adrian M Di Bisceglie, Timothy Block, Anand Mehta   +7 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

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 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