Alpha1-Antitrypsin Deficiency

P Strnad, NG McElvaney… - New England Journal of …, 2020 - Mass Medical Soc
Alpha1-Antitrypsin Deficiency AAT is a protease inhibitor targeting neutrophil elastase. It
prevents the destruction of tissue, particularly in the lung, from elastase activity. AAT …

Neutrophil elastase and chronic lung disease

JA Voynow, M Shinbashi - Biomolecules, 2021 - mdpi.com
Neutrophil elastase (NE) is a major inflammatory protease released by neutrophils and is
present in the airways of patients with cystic fibrosis (CF), chronic obstructive pulmonary …

Alpha-1 antitrypsin inhibits TMPRSS2 protease activity and SARS-CoV-2 infection

L Wettstein, T Weil, C Conzelmann, JA Müller… - Nature …, 2021 - nature.com
SARS-CoV-2 is a respiratory pathogen and primarily infects the airway epithelium. As our
knowledge about innate immune factors of the respiratory tract against SARS-CoV-2 is …

The transmembrane protease TMPRSS2 as a therapeutic target for COVID-19 treatment

L Wettstein, F Kirchhoff, J Münch - International Journal of Molecular …, 2022 - mdpi.com
TMPRSS2 is a type II transmembrane protease with broad expression in epithelial cells of
the respiratory and gastrointestinal tract, the prostate, and other organs. Although the …

Engineering synthetic breath biomarkers for respiratory disease

LW Chan, MN Anahtar, TH Ong, KE Hern… - Nature …, 2020 - nature.com
Human breath contains many volatile metabolites. However, few breath tests are currently
used in the clinic to monitor disease due to bottlenecks in biomarker identification. Here we …

A review of alpha-1 antitrypsin binding partners for immune regulation and potential therapeutic application

ME O'Brien, G Murray, D Gogoi, A Yusuf… - International Journal of …, 2022 - mdpi.com
Alpha-1 antitrypsin (AAT) is the canonical serine protease inhibitor of neutrophil-derived
proteases and can modulate innate immune mechanisms through its anti-inflammatory …

Native and activated antithrombin inhibits TMPRSS2 activity and SARS‐CoV‐2 infection

L Wettstein, P Immenschuh, T Weil… - Journal of Medical …, 2023 - Wiley Online Library
Host cell proteases such as TMPRSS2 are critical determinants of severe acute respiratory
syndrome coronavirus 2 (SARS‐CoV‐2) tropism and pathogenesis. Here, we show that …

α1-Antitrypsin deficiency and chronic respiratory disorders

M Cazzola, D Stolz, P Rogliani… - European Respiratory …, 2020 - Eur Respiratory Soc
α1-antitrypsin deficiency (AATD) is a hereditary disorder associated with a risk of developing
liver disease and pulmonary emphysema, and other chronic respiratory disorders (mainly …

Augmentation therapy for severe alpha-1 antitrypsin deficiency improves survival and is decoupled from spirometric decline—a multinational registry analysis

DD Fraughen, AJ Ghosh, BD Hobbs… - American Journal of …, 2023 - atsjournals.org
Rationale: Intravenous plasma-purified alpha-1 antitrypsin (IV-AAT) has been used as
therapy for alpha-1 antitrypsin deficiency (AATD) since 1987. Previous trials (RAPID and …

Unique and shared systemic biomarkers for emphysema in Alpha-1 Antitrypsin deficiency and chronic obstructive pulmonary disease

KA Serban, KA Pratte, C Strange, RA Sandhaus… - …, 2022 - thelancet.com
Summary Background Alpha-1 Antitrypsin (AAT) deficiency (AATD), the most common
genetic cause of emphysema presents with unexplained phenotypic heterogeneity in …