Expression of mutant Sftpc in murine alveolar epithelia drives spontaneous lung fibrosis

Monocyte-derived alveolar macrophages drive lung fibrosis and persist in the lung over the life span

(2017) Alexander V. Misharin et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Tamoxifen for induction of Cre-recombination may confound fibrosis studies in female mice

(2017) Lucas L. Falke et al.   Journal of Cell Communication and Signaling

MAP1LC3B overexpression protects against Hermansky-Pudlak syndrome type-1-induced defective autophagy in vitro

(2016) Saket Ahuja et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Plasma Surfactant Protein-D, Matrix Metalloproteinase-7, and Osteopontin Index Distinguishes Idiopathic Pulmonary Fibrosis from Other Idiopathic Interstitial Pneumonias

(2016) Eric S. White et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report

(2016) Harold R. Collard et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Targeted Type 2 Alveolar Cell Depletion. A Dynamic Functional Model for Lung Injury Repair

(2016) Orquidea Garcia et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

First Data on Efficacy and Safety of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis and Forced Vital Capacity of ≤50 % of Predicted Value

(2016) Wim A. Wuyts et al.   LUNG

Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease

(2015) Surafel Mulugeta et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy

(2015) Arie Hawkins et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy

(2015) Tunda Hidvegi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Tamoxifen Inhibits TGF-β-Mediated Activation of Myofibroblasts by Blocking Non-Smad Signaling Through ERK1/2

(2015) Jon M. Carthy et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Telomere dysfunction causes alveolar stem cell failure

(2015) Jonathan K. Alder et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Sex-specific differences in hyperoxic lung injury in mice: Role of cytochrome P450 (CYP)1A

(2015) Krithika Lingappan et al.   TOXICOLOGY

Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction

(2015) Juan M. Povedano et al.   Cell Reports

Revealing the Pathogenic and Aging-related Mechanisms of the Enigmatic Idiopathic Pulmonary Fibrosis. An Integral Model

(2014) Moisés Selman et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis

(2014) Talmadge E. King et al.   NEW ENGLAND JOURNAL OF MEDICINE

An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias

(2013) William D. Travis et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

(2013) Geoffrey Kurland et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Future Directions in Idiopathic Pulmonary Fibrosis Research. An NHLBI Workshop Report

(2013) Timothy S. Blackwell et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Flow Cytometric Analysis of Macrophages and Dendritic Cell Subsets in the Mouse Lung

(2013) Alexander V. Misharin et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Animal Models of Fibrotic Lung Disease

(2013) Bethany B. Moore et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Type 2 alveolar cells are stem cells in adult lung

(2013) Christina E. Barkauskas et al.   JOURNAL OF CLINICAL INVESTIGATION

Targeting of αv integrin identifies a core molecular pathway that regulates fibrosis in several organs

(2013) Neil C Henderson et al.   NATURE MEDICINE

The Alveolar Epithelium Determines Susceptibility to Lung Fibrosis in Hermansky-Pudlak Syndrome

(2012) Lisa R. Young et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

4-Phenylbutyric Acid Treatment Rescues Trafficking and Processing of a Mutant Surfactant Protein–C

(2012) Gareth A. Stewart et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis

(2012) J. A. Kropski et al.   Disease Models & Mechanisms

Prednisone, Azathioprine, andN-Acetylcysteine for Pulmonary Fibrosis

(2012)   NEW ENGLAND JOURNAL OF MEDICINE

Age and sex dimorphisms contribute to the severity of bleomycin-induced lung injury and fibrosis

(2011) Elizabeth F. Redente et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Ly6ChiMonocytes Direct Alternatively Activated Profibrotic Macrophage Regulation of Lung Fibrosis

(2011) Michael A. Gibbons et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Early Alveolar Epithelial Dysfunction Promotes Lung Inflammation in a Mouse Model of Hermansky-Pudlak Syndrome

(2011) Elena N. Atochina-Vasserman et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Gene-targeted embryonic stem cells: real-time PCR assay for estimation of the number of neomycin selection cassettes

(2011) Cecilia Mancini et al.   BIOLOGICAL PROCEDURES ONLINE

Multiple ways to die: Delineation of the unfolded protein response and apoptosis induced by Surfactant Protein C BRICHOS mutants

(2011) Jean Ann Maguire et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Idiopathic pulmonary fibrosis

(2011) Talmadge E King et al.   LANCET

Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs

(2011) W. E. Lawson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Nonaggregating Surfactant Protein C Mutant Is Misdirected to Early Endosomes and Disrupts Phospholipid Recycling

(2011) Michael F. Beers et al.   TRAFFIC

Surfactant Protein C Mutations Are the Basis of a Significant Portion of Adult Familial Pulmonary Fibrosis in a Dutch Cohort

(2010) Coline H. M. van Moorsel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Endoplasmic Reticulum Stress Induced by Surfactant Protein C BRICHOS Mutants Promotes Proinflammatory Signaling by Epithelial Cells

(2010) Jean Ann Maguire et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Identification of Early Interstitial Lung Disease in an Individual With Genetic Variations in ABCA3 and SFTPC

(2010) Peter F. Crossno et al.   CHEST

Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome

(2010) J. W. Song et al.   EUROPEAN RESPIRATORY JOURNAL

Bronchoalveolar lavage in idiopathic pulmonary fibrosis: What does it tell us?

(2010) A. Pesci et al.   RESPIRATORY MEDICINE

Targeted Injury of Type II Alveolar Epithelial Cells Induces Pulmonary Fibrosis

(2009) Thomas H. Sisson et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Aberrant Processing Forms of Lung Surfactant Proteins SP–B and SP–C Revealed by High-Resolution Mass Spectrometry

(2009) Dmitry Galetskiy et al.   EUROPEAN JOURNAL OF MASS SPECTROMETRY

Familial interstitial disease with I73T mutation: A mid- and long-term study

(2009) Rola Abou Taam et al.   PEDIATRIC PULMONOLOGY

A Novel Mechanism for CCR4 in the Regulation of Macrophage Activation in Bleomycin-Induced Pulmonary Fibrosis

(2008) Glenda Trujillo et al.   AMERICAN JOURNAL OF PATHOLOGY

Epithelial Endoplasmic Reticulum Stress and Apoptosis in Sporadic Idiopathic Pulmonary Fibrosis

(2008) Martina Korfei et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy

(2008) Anja Fritsch et al.   JOURNAL OF CLINICAL INVESTIGATION