Heterozygous germline mutations in BMPR2, encoding a TGF-β receptor, cause familial primary pulmonary hypertension

Kirk B. Lane, Rajiv D. Machado, Michael W. Pauciulo, +5

Nature Genetics

HypertensionPulmonary Arterial HypertensionTgf-β ReceptorGenetic DisorderGenetics +9

Heterozygosity for a Surfactant Protein C Gene Mutation Associated with Usual Interstitial Pneumonitis and Cellular Nonspecific Interstitial Pneumonitis in One Kindred

Alan Q. Thomas, Kirk B. Lane, John A. Phillips, +11

American Journal of Respiratory and Critical Care Medicine

BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension

Rajiv D. Machado, Michael W. Pauciulo, Jennifer R. Thomson, +20

The American Journal of Human Genetics

HypertensionPulmonary Arterial HypertensionPulmonary CirculationPrimary Pulmonary HypertensionInherited Molecular Mechanism +10

Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection

William E. Lawson, Peter F. Crossno, Vasiliy V. Polosukhin, +10

American Journal of Physiology-Lung Cellular and Molecular Physiology

ImmunologyViral PathogenesisPulmonary Alveolar ProteinosisPathologyAec Dysfunction +17

Mutation in the Gene for Bone Morphogenetic Protein Receptor II as a Cause of Primary Pulmonary Hypertension in a Large Kindred

John H. Newman, Lisa Wheeler, Kirk B. Lane, +4

New England Journal of Medicine

Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs

William E. Lawson, Dong‐Sheng Cheng, Amber L. Degryse, +11

Proceedings of the National Academy of Sciences

Pulmonary Hypertension in Transgenic Mice Expressing a Dominant-Negative BMPRII Gene in Smooth Muscle

James West, Karen Fagan, Wolfgang Steudel, +8

Circulation Research

SclerostinGeneticsDominant-negative BmpriiPulmonary HypertensionBone Morphogenetic Peptides +18

Increased and Prolonged Pulmonary Fibrosis in Surfactant Protein C-Deficient Mice Following Intratracheal Bleomycin

William E. Lawson, Vasiliy V. Polosukhin, Georgios T. Stathopoulos, +12

American Journal Of Pathology

Acute Lung InjuryInflammationProlonged Pulmonary FibrosisFibrosisInflammatory Lung Disease +10

Interaction of interleukin-6 and the BMP pathway in pulmonary smooth muscle

Moira Hagen, Karen Fagan, Wolfgang Steudel, +3

American Journal of Physiology-Lung Cellular and Molecular Physiology

Inflammatory Lung DiseaseLung InflammationImmunologyCellular PhysiologyInflammation +16

Pulmonary Veno-occlusive Disease Caused by an Inherited Mutation in Bone Morphogenetic Protein Receptor II

James Runo, Cindy L. Vnencak‐Jones, Melissa Prince, +8

American Journal of Respiratory and Critical Care Medicine