Associated Papers
Divergent pathways of Surfactant Protein C maturation for disease-associated isoforms.
Bui S et al.
The Journal of Biological Chemistry, 2026
Aberrant intermediate alveolar epithelial cells promote pathogenic activation of lung fibroblasts in preclinical fibrosis models.
Hoffman ET et al.
Nature Communications, 2025
Clinical characteristics of SFTPC gene mutations in children: a single-center experience.
Yang T et al.
Italian Journal of Pediatrics, 2025
Progressive lung fibrosis: reprogramming a genetically vulnerable bronchoalveolar epithelium.
Bridges JP et al.
The Journal of Clinical Investigation, 2025
Engraftment of wild-type alveolar type II epithelial cells in surfactant protein C deficient mice.
Predella C et al.
Research Square, 2024
Humanized L184Q Mutated Surfactant Protein C Gene Alters Alveolar Type 2 Epithelial Cell Fate.
Jain KG et al.
International Journal of Molecular Sciences, 2024
Novel surfactant C variant c.325-47_374del without response to hydroxychloroquine treatment.
Drobnakova S et al.
Respiratory Medicine Case Reports, 2024
Chronic Expression of a Clinical SFTPC Mutation Causes Murine Lung Fibrosis with Idiopathic Pulmonary Fibrosis Features.
Rodriguez L et al.
American Journal of Respiratory Cell and Molecular Biology, 2023
Disruption of proteostasis causes IRE1 mediated reprogramming of alveolar epithelial cells.
Katzen J et al.
Proceedings of the National Academy of Sciences of the United States of America, 2022
Gene Therapeutics for Surfactant Dysfunction Disorders: Targeting the Alveolar Type 2 Epithelial Cell.
Sitaraman S et al.
Human Gene Therapy, 2022
Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease.
Alysandratos KD et al.
Cell Reports, 2021
Surfactant protein C mutation links postnatal type 2 cell dysfunction to adult disease.
Sitaraman S et al.
Jci Insight, 2021
An SFTPC BRICHOS mutant links epithelial ER stress and spontaneous lung fibrosis.
Katzen J et al.
Jci Insight, 2019
Interstitial lung disease of infancy caused by a new NKX2-1 mutation.
Safi KH et al.
Clinical Case Reports, 2017
A novel surfactant protein C gene mutation associated with progressive respiratory failure in infancy.
Litao MK et al.
Pediatric Pulmonology, 2017
Genotype alone does not predict the clinical course of SFTPC deficiency in paediatric patients.
Kröner C et al.
The European Respiratory Journal, 2015
Successful treatment of neonatal respiratory failure caused by a novel surfactant protein C p.Cys121Gly mutation with hydroxychloroquine.
Hepping N et al.
Journal of Perinatology : Official Journal of the California Perinatal Association, 2013