Associated Papers
Idiopathic Pulmonary Fibrosis: A Comprehensive Review of Risk Factors, Genetics, Diagnosis, and Therapeutic Approaches.
Senhaji L et al.
Biomedicines, 2026
Surfactant Protein (SP)-A Benefits Over SP-A Mutant: A Preliminary Study for Interstitial Lung Disease Treatment.
Desroziers T et al.
American Journal of Respiratory Cell and Molecular Biology, 2025
A Test to Comprehensively Capture the Known Genetic Component of Familial Pulmonary Fibrosis.
Villeneuve J et al.
American Journal of Respiratory Cell and Molecular Biology, 2024
Functional assessment and phenotypic heterogeneity of SFTPA1 and SFTPA2 mutations in interstitial lung diseases and lung cancer.
Legendre M et al.
The European Respiratory Journal, 2020
Identification and functional characterization of a novel surfactant protein A2 mutation (p.N207Y) in a Chinese family with idiopathic pulmonary fibrosis.
Liu L et al.
Molecular Genetics & Genomic Medicine, 2020
Epigallocatechin-3-gallate (EGCG) inhibits aggregation of pulmonary fibrosis associated mutant surfactant protein A2 via a proteasomal degradation pathway.
Quan Y et al.
The International Journal of Biochemistry & Cell Biology, 2019
Chemical chaperone 4-phenylbutyric acid alleviates the aggregation of human familial pulmonary fibrosis-related mutant SP-A2 protein in part through effects on GRP78.
Jiang X et al.
Biochimica Et Biophysica Acta. Molecular Basis of Disease, 2018
Systematic review of drug effects in humans and models with surfactant-processing disease.
Klay D et al.
European Respiratory Review : an Official Journal of the European Respiratory Society, 2018
Genetics of idiopathic pulmonary fibrosis: from mechanistic pathways to personalised medicine.
Spagnolo P et al.
Journal of Medical Genetics, 2017
SFTPA2 Mutations in Familial and Sporadic Idiopathic Interstitial Pneumonia.
van Moorsel CH et al.
American Journal of Respiratory and Critical Care Medicine, 2015
Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease.
Mulugeta S et al.
American Journal of Physiology. Lung Cellular and Molecular Physiology, 2015
Mutations of rat surfactant protein A have distinct effects on its glycosylation, secretion, aggregation and degradation.
Yang W et al.
Life Sciences, 2014
Identification and Quantitation of Coding Variants and Isoforms of Pulmonary Surfactant Protein A.
Foster MW et al.
Journal of Proteome Research, 2014
Genome sequencing of idiopathic pulmonary fibrosis in conjunction with a medical school human anatomy course.
Kumar A et al.
Plos One, 2014
Human surfactant protein A2 gene mutations impair dimmer/trimer assembly leading to deficiency in protein sialylation and secretion.
Song Y et al.
Plos One, 2012
Idiopathic pulmonary fibrosis: update on genetic discoveries.
Garcia CK
Proceedings of the American Thoracic Society, 2011
Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress.
Maitra M et al.
The Journal of Biological Chemistry, 2010
Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer.
Wang Y et al.
American Journal of Human Genetics, 2009