ArticleAmerican journal of respiratory cell and molecular biology2023
Sonic Hedgehog Signaling Is Essential for Pulmonary Ionocyte Specification in Human and Ferret Airway Epithelia.
Article in American journal of respiratory cell and molecular biology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 30 citations in OpenAlex.
- Host-pathogen pyrimidine metabolism drives cystic fibrosis lung disease.Nature communications · 2026Article
- Small molecule-directed differentiation of submerged-cultured human nasal airway epithelia for respiratory disease modeling.Cell reports. Medicine · 2026Article
- Cellular plasticity and regenerative mechanisms in the lung.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Next Decade Research in Asthma: Broad Omics-Based Exploration Versus Targeted Airway Epithelium Studies.Journal of clinical medicine · 2025Review
- Sorcin regulates alveolarization and airway tissue remodeling during lung morphogenesis.Cellular and molecular life sciences : CMLS · 2025Article
- Pulmonary Ionocytes at the Crossroads of Absorption and Secretion: Clues from the Ferret.American journal of respiratory and critical care medicine · 2025Article
- Ionocyte CFTR Coordinates Chloride Absorption and Secretion to Balance Airway Fluid.American journal of respiratory and critical care medicine · 2025Article
- Single cell profiling of human airway identifies tuft-ionocyte progenitor cells displaying cytokine-dependent differentiation bias in vitro.Nature communications · 2025Article
- Catalpol inhibits Hedgehog signaling pathway to suppress proliferation and promote lipid accumulation in rat meibomian gland epithelial cells.Cytotechnology · 2025Article
- Do pulmonary ionocytes absorb chloride or secrete chloride?American journal of physiology. Cell physiology · 2025Review
- Pulmonary Ionocytes Regulate Airway Surface Liquid pH in Primary Human Bronchial Epithelial Cells.American journal of respiratory and critical care medicine · 2024Article
- Pulmonary Ionocytes: What Are They Transporting and Which Way?American journal of respiratory and critical care medicine · 2024Article
- IL-13 induces loss of CFTR in ionocytes and reduces airway epithelial fluid absorption.The Journal of clinical investigation · 2024Article
- In vitro platform to model the function of ionocytes in the human airway epithelium.Respiratory research · 2024Article
- Association of Fetal Lung Development Disorders with Adult Diseases: A Comprehensive Review.Journal of personalized medicine · 2024Review
- Functional Consequences of CFTR Interactions in Cystic Fibrosis.International journal of molecular sciences · 2024Review
- Cystic Fibrosis: "Ionocyte Modulators"?American journal of respiratory cell and molecular biology · 2023Article
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Authors and funding
8 authors at 1 institution in 2 countries.
Funding
Abstract
Pulmonary ionocytes express high levels of cystic fibrosis transmembrane conductance regulator (CFTR), an anion channel that is critical for hydration of the airways and mucociliary clearance. However, the cellular mechanisms that govern ionocyte specification and function remain unclear. We observed that increased abundance of ionocytes in cystic fibrosis (CF) airway epithelium was associated with enhanced expression of Sonic Hedgehog (SHH) effectors. In this study, we evaluated whether the SHH pathway directly impacts ionocyte differentiation and CFTR function in airway epithelia. Pharmacological HPI1-mediated inhibition of SHH signaling component GLI1 significantly impaired human basal cell specification of ionocytes and ciliated cells but significantly enhanced specification of secretory cells. By contrast, activation of the SHH pathway effector smoothened (SMO) with the chemical agonist SAG significantly enhanced ionocyte specification. The abundance of CFTR
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