When Development of the Alveolar Gas Exchange Unit Fails Universal Single-Cell Lessons from Rare Monogenic Disorders

Article Biochemistry & Molecular Biology

An integrated cell atlas of the lung in health and disease

Lisa Sikkema et al.

Summary: Single-cell technologies are used to study human tissues, but individual studies have limitations in terms of sample size and cell type definitions. The integrated Human Lung Cell Atlas (HLCA) combines 49 datasets from 486 individuals to create a comprehensive atlas of the human respiratory system. The HLCA provides new annotations for different cell types and allows for the identification of gene modules associated with demographic variables and changes in gene expression.

NATURE MEDICINE (2023)

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Article Multidisciplinary Sciences

Lung endothelial cells regulate pulmonary fibrosis through FOXF1/R-Ras signaling

Fenghua Bian et al.

Summary: This study reveals the inhibitory role of endothelial cells in pulmonary fibrosis, and shows that endothelial-specific delivery of FOXF1 gene therapy reduces lung fibrosis in mice.

NATURE COMMUNICATIONS (2023)

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Review Physiology

Lung Cell Atlases in Health and Disease

Taylor S. Adams et al.

Summary: The human lung cellular portfolio is highly diverse with over 40 cell types and a complex branching structure. Changes in lung cellular content can occur in response to exposure, injury, and infection, leading to progressive lung disease. The rise of single-cell profiling technologies has allowed for detailed measurements and the development of comprehensive cell atlases, which have greatly contributed to our understanding of human lung biology in health and disease.

ANNUAL REVIEW OF PHYSIOLOGY (2023)

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Article Critical Care Medicine

Single Cell Multiomics Identifies Cells and Genetic Networks Underlying Alveolar Capillary Dysplasia

Minzhe Guo et al.

Summary: This study reveals the cellular and molecular mechanisms underlying the pathogenesis of Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a lethal disorder of lung development. The deficiency of FOXF1 transcription factor disrupts lung formation, leading to changes in cell types, gene expression, and cell-cell interactions in ACDMPV lungs. These findings provide potential targets for the development of therapies for ACDMPV and other diffuse lung diseases of infancy.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2023)

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Article Pathology

A Morphomolecular Approach to Alveolar Capillary Dysplasia

Jan C. Kamp et al.

Summary: This study analyzed the microvascular morphology and molecular background of ACD, revealing characteristics such as altered capillary network, high prevalence of intussusceptive angiogenesis, and increased activity of key signaling pathways in ACD patients.

AMERICAN JOURNAL OF PATHOLOGY (2022)

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Article Cardiac & Cardiovascular Systems

Integrated Single-Cell Atlas of Endothelial Cells of the Human Lung

Jonas C. Schupp et al.

Summary: This study provides a comprehensive reference atlas of endothelial cells in the lungs, identifying over 15,000 vascular endothelial cells from 73 individuals. The research reveals previously unrecognized subpopulations of lung endothelial cells and confirms the maintenance of endothelial diversity in pulmonary hypertension.

CIRCULATION (2021)

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Article Cell Biology