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 …

Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews

M Miravitlles, M Herepath, A Priyendu… - European …, 2022 - Eur Respiratory Soc
Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder characterised by reduced
levels of circulating alpha-1 antitrypsin and an increased risk of lung and liver disease …

2022 年GOLD 慢性阻塞性肺疾病诊断, 治疗, 管理及预防全球策略更新要点解读

陈亚红 - 中国全科医学, 2022 - chinagp.net
慢性阻塞性肺疾病(慢阻肺) 全球倡议(global initiative for chronic obstructive lung disease,
GOLD) 2022 年修订版于2021-11-15 发布, 总体来说其对于慢阻肺的诊断, 评估 …

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 …

Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency

M Fromme, CV Schneider, V Pereira, K Hamesch… - Gut, 2022 - gut.bmj.com
Objective Alpha-1 antitrypsin deficiency (AATD) is a common, potentially lethal inborn
disorder caused by mutations in alpha-1 antitrypsin (AAT). Homozygosity for the 'Pi …

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 …

Alpha-1 antitrypsin deficiency: current therapy and emerging targets

OF McElvaney, DD Fraughen… - Expert Review of …, 2023 - Taylor & Francis
Introduction Alpha1 antitrypsin deficiency (AATD), a common hereditary disorder affecting
mainly lungs, liver and skin has been the focus of some of the most exciting therapeutic …

Alpha-1 antitrypsin deficiency associated COPD

C Strange - Clinics in Chest Medicine, 2020 - chestmed.theclinics.com
Alpha-1 antitrypsin (AAT) is the most abundant serine proteinase inhibitor in human plasma.
When Laurell and Erikson 1 first noted the association between a deficiency of the protein …

Alpha1-antitrypsin deficiency: An updated review

JF Mornex, J Traclet, O Guillaud, M Dechomet… - La Presse Médicale, 2023 - Elsevier
Abstract Alpha1-antitrypsin deficiency (AATD) is a rare autosomal recessive disease
associated with the homozygous Z variant of the SERPINA1 gene. Clinical expression of …

Alpha-1 antitrypsin deficiency and tobacco smoking: exploring risk factors and smoking cessation in a registry population

AN Franciosi, MA Alkhunaizi, A Woodsmith… - COPD: Journal of …, 2020 - Taylor & Francis
The ZZ genotype of alpha-1 antitrypsin deficiency (AATD) is strongly associated with COPD,
even in never-smokers. Moderate AATD genotypes (MZ and SZ) have been shown to …