ABC family transporters

X Liu - Drug transporters in drug disposition, effects and …, 2019 - Springer
The transport of specific molecules across lipid membranes is an essential function of all
living organisms. The processes are usually mediated by specific transporters. One of the …

European protocols for the diagnosis and initial treatment of interstitial lung disease in children

A Bush, S Cunningham, J De Blic, A Barbato… - Thorax, 2015 - thorax.bmj.com
Interstitial lung disease in children (chILD) is rare, and most centres will only see a few
cases/year. There are numerous possible underlying diagnoses, with specific and non …

European Respiratory Society statement on familial pulmonary fibrosis

R Borie, C Kannengiesser, K Antoniou… - European …, 2023 - Eur Respiratory Soc
Genetic predisposition to pulmonary fibrosis has been confirmed by the discovery of several
gene mutations that cause pulmonary fibrosis. Although genetic sequencing of familial …

[HTML][HTML] Research progress in the molecular mechanisms, therapeutic targets, and drug development of idiopathic pulmonary fibrosis

H Ma, X Wu, Y Li, Y Xia - Frontiers in Pharmacology, 2022 - frontiersin.org
Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease. Recent studies have
identified the key role of crosstalk between dysregulated epithelial cells, mesenchymal …

Lung disease caused by ABCA3 mutations

C Kröner, T Wittmann, S Reu, V Teusch, M Klemme… - Thorax, 2017 - thorax.bmj.com
Background Knowledge about the clinical spectrum of lung disease caused by variations in
the ATP binding cassette subfamily A member 3 (ABCA3) gene is limited. Here we describe …

[HTML][HTML] Contributions of alveolar epithelial cell quality control to pulmonary fibrosis

J Katzen, MF Beers - The Journal of clinical investigation, 2020 - Am Soc Clin Investig
Epithelial cell dysfunction has emerged as a central component of the pathophysiology of
diffuse parenchymal diseases including idiopathic pulmonary fibrosis (IPF). Alveolar type 2 …

[HTML][HTML] Large-scale RNA-Seq transcriptome analysis of 4043 cancers and 548 normal tissue controls across 12 TCGA cancer types

L Peng, XW Bian, DK Li, C Xu, GM Wang, QY Xia… - Scientific reports, 2015 - nature.com
Abstract The Cancer Genome Atlas (TCGA) has accrued RNA-Seq-based transcriptome
data for more than 4000 cancer tissue samples across 12 cancer types, translating these …

Tissue remodelling in pulmonary fibrosis

L Knudsen, C Ruppert, M Ochs - Cell and tissue research, 2017 - Springer
Many lung diseases result in fibrotic remodelling. Fibrotic lung disorders can be divided into
diseases with known and unknown aetiology. Among those with unknown aetiology …

Genetic disorders of the surfactant system: focus on adult disease

CHM van Moorsel, JJ van der Vis… - European Respiratory …, 2021 - Eur Respiratory Soc
Genes involved in the production of pulmonary surfactant are crucial for the development
and maintenance of healthy lungs. Germline mutations in surfactant-related genes cause a …

The role of genetic testing in pulmonary fibrosis: a perspective from the pulmonary fibrosis foundation genetic testing work group

CA Newton, JM Oldham, C Applegate, N Carmichael… - Chest, 2022 - Elsevier
Patients with familial pulmonary fibrosis represent a subset of patients with pulmonary
fibrosis in whom inherited gene variation predisposes them to disease development. In the …