Results 21 to 30 of about 42,616 (247)
Frontiers in Molecular Biosciences, 2022 Background: Alpha-1 antitrypsin deficiency (A1ATD) is a progressive lung disease caused by inherited pathogenic variants in the SERPINA1 gene. However, their actual role in maintenance of structural and functional characteristics of the corresponding α-1
Noor Ahmad Shaik +14 more
doaj +1 more source
Testing for alpha-1 antitrypsin in COPD in outpatient respiratory clinics in Spain: A multilevel, cross-sectional analysis of the EPOCONSUL study [PDF]
, 2018 Background Alpha-1 antitrypsin deficiency (AATD) is the most common hereditary disorder in adults, but is under-recognized. In Spain, the number of patients diagnosed with AATD is much lower than expected according to epidemiologic studies.
Alcázar Navarrete, Bernardino +6 more
core +1 more source
Does urinary peptide content differ between COPD patients with and without inherited alpha-1 antitrypsin deficiency? [PDF]
, 2017 Differentiating between chronic obstructive pulmonary disease (COPD) patients with normal (PiMM) or deficient (PiZZ) genetic variants of alpha-1 antitrypsin (A1AT) is important not only for understanding the pathobiology of disease progression but also ...
Carleo, Alfonso +7 more
core +2 more sources
PLoS ONE, 2020 BACKGROUND:Alpha-1-antitrypsin is a protein involved in avoidance of different processes that are seen in diabetic retinopathy pathogenesis. These processes include apoptosis, extracellular matrix remodeling and damage of vessel walls and capillaries ...
María Constanza Potilinski +6 more
doaj +1 more source
Delving into Vertebrate Serpins for Understanding their Evolution [PDF]
, 2009 The superfamily of serine proteinase inhibitors (serpins) is involved in an array of fundamental biological processes such as blood coagulation, cell differentiation, cell migration, complement activation, embryo implantation, fibrinolysis, angiogenesis,
Abhishek Kumar
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ALPHA-1-ANTITRYPSIN DEFICIENCY IN CHILDREN
Медицинский совет, 2017 Hereditary deficiency of the alpha-1-antitrypsin occupies a leading position among the causes of chronic nonspecific lung diseases with emphysema formation.
S. I. MELNIK +5 more
doaj +1 more source
Respiratory Medicine Case Reports, 2022 Alpha-1-antitrypsin deficiency (AATD) is a rare cause of noncystic fibrosis (CF) bronchiectasis. The benefits of augmentation therapy in patients with chronic obstructive pulmonary disease (COPD) and pulmonary emphysema are well established.
Emanuel Buck +6 more
doaj +1 more source
An antibody that prevents serpin polymerisation acts by inducing a novel allosteric behavior [PDF]
, 2016 Serpins are important regulators of proteolytic pathways with an antiprotease activity that involves a conformational transition from a metastable to a hyperstable state. Certain mutations permit the transition to occur in the absence of a protease; when
Faull, Sarah V. +14 more
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The skeletal phenotype of chondroadherin deficient mice [PDF]
, 2013 Chondroadherin, a leucine rich repeat extracellular matrix protein with functions in cell to matrix interactions, binds cells via their a2b1 integrin as well as via cell surface proteoglycans, providing for different sets of signals to the cell ...
A Franzen +44 more
core +8 more sources
Clinical features in patients with severe Alpha-1 antitrypsin deficiency due to rare genotypes
PulmonologyAlpha-1 Antitrypsin Deficiency (AATD) is a co-dominant condition associated with an increased risk of lung and liver disease. Since it is commonly thought that 95% of severe cases of AATD have PI*ZZ genotype, most studies about AATD have been focused on ...
Ilaria Ferrarotti +18 more
doaj +1 more source