Respiratory Research, 2023 Background Alpha-1-antitrypsin (AAT) deficiency (AATD) is a genetic disorder that can manifest as lung disease. A delay between onset of symptoms and diagnosis of AATD is common and associated with worse clinical status and more advanced disease stage ...
Tobias Meischl +12 more
doaj +1 more source
An antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein. [PDF]
, 2015 A monoclonal antibody (mAb) that binds to a transient intermediate may act as a catalyst for the corresponding reaction; here we show this principle can extend on a macro-molecular scale to the induction of mutant-like oligomerisation in a wild-type ...
Faull, SV +7 more
core +4 more sources
Alpha-1 antitrypsin deficiency [PDF]
Archives of Disease in Childhood, 2001 α-1 antitrypsin is synthesised in the liver and protects lung alveolar tissues from destruction by neutrophil elastase. α-1 antitrypsin deficiency is a common autosomal recessive condition (1:1600 to 1:1800) in which liver disease results from retention of abnormal polymerised α-1 antitrypsin in the endoplasmic reticulum of hepatocytes, and emphysema ...
Primhak, R.A., Tanner, M.S.
openaire +4 more sources
Alpha-1-antitrypsin: a novel human high temperature requirement protease A1 (HTRA1) substrate in human placental tissue. [PDF]
PLoS ONE, 2014 The human serine protease high temperature requirement A1 (HTRA1) is highly expressed in the placental tissue, especially in the last trimester of gestation. This suggests that HTRA1 is involved in placental formation and function.
Violette Frochaux +4 more
doaj +1 more source
Alpha 1 Antitrypsin is an Inhibitor of the SARS-CoV-2-Priming Protease TMPRSS2
bioRxiv, 2020 Host proteases have been suggested to be crucial for dissemination of MERS, SARS-CoV, and SARS-CoV-2 coronaviruses, but the relative contribution of membrane versus intracellular proteases remains controversial.
N. Azouz +11 more
semanticscholar +1 more source
AASLD practice guidance on drug, herbal, and dietary supplement–induced liver injury
, 2022 Hepatology, EarlyView.
Robert J. Fontana +6 more
wiley +1 more source
Case-finding for alpha1-antitrypsin deficiency in Kazakh patients with COPD
Multidisciplinary Respiratory Medicine, 2017 Background Alpha-1-antitrypsin deficiency (AATD) is an under-diagnosed condition in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to screen for AATD in Kazakh patients with COPD using dried blood spot specimens ...
Ardak Zhumagaliyeva +12 more
doaj +1 more source
Liver Transplantation for Advanced Liver Disease with Alpha-1antitrypsin Deficiency [PDF]
, 1980 ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson.1 In 1969, Sharp2 described the first cases of alpha-1-antitrypsin-deficiency disease in children with cirrhosis. Since then,
Allan G. Redeker +26 more
core +1 more source
The undiagnosed disease burden associated with alpha-1 antitrypsin deficiency genotypes
European Respiratory Journal, 2020 Alpha-1 antitrypsin deficiency (AATD), mainly due to the PI*ZZ genotype in SERPINA1, is one of the most common inherited diseases. Since it is associated with a high disease burden and partially prevented by smoking cessation, identification of PI*ZZ ...
T. Nakanishi +7 more
semanticscholar +1 more source
REVERSAL OF HEPATIC ALPHA-1-ANTITRYPSIN DEPOSITION AFTER PORTACAVAL SHUNT [PDF]
, 1983 End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 31/2 to almost 7 years.
Aagenaes +17 more
core +1 more source