ArticleMolecules and cells2018
Increased Primary Cilia in Idiopathic Pulmonary Fibrosis.
Article in Molecules and cells, 2018. 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, 25 citations in OpenAlex.
- Primary cilia as regulators of fibrosis and potential therapeutic targets.Nature reviews. Rheumatology · 2026Review
- Dynamics of primary cilia in endothelial and mesenchymal cells throughout mouse lung development.Developmental dynamics : an official publication of the American Association of Anatomists · 2025Article
- Whole genome DNA methylation patterns in tissue and cfDNA associated with fibrosis reflect the complex signature of MASLD.PloS one · 2025Article
- Spatiotemporal dynamics of primary and motile cilia throughout lung development.bioRxiv : the preprint server for biology · 2024Article
- Modelling and targeting mechanical forces in organ fibrosis.Nature reviews bioengineering · 2024Article
- Multi-omics integration reveals a nonlinear signature that precedes progression of lung fibrosis.Clinical & translational immunology · 2024Article
- Identification of TIMP1-induced dysregulation of epithelial-mesenchymal transition as a key pathway in inflammatory bowel disease and small intestinal neuroendocrine tumors shared pathogenesis.Frontiers in genetics · 2024Article
- Airway ciliated cells in adult lung homeostasis and COPD.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Article
- Aberrant Multiciliogenesis in Pulmonary Fibrosis: Bystander or Driver of Disease Progression?American journal of respiratory cell and molecular biology · 2022Article
- Aberrant Multiciliogenesis in Idiopathic Pulmonary Fibrosis.American journal of respiratory cell and molecular biology · 2022Article
- A cell-based GEF assay reveals new substrates for DENN domains and a role for DENND2B in primary ciliogenesis.Science advances · 2022Article
- Myocardin-related transcription factor and serum response factor regulate cilium turnover by both transcriptional and local mechanisms.iScience · 2021Article
- Kinesin family member 3A inhibits the carcinogenesis of non-small cell lung cancer and prolongs survival.Oncology letters · 2020Article
- Silica Perturbs Primary Cilia and Causes Myofibroblast Differentiation during Silicosis by Reduction of the KIF3A-Repressor GLI3 Complex.Theranostics · 2020Article
- Fibroblast Primary Cilia Are Required for Cardiac Fibrosis.Circulation · 2019Article
- Is idiopathic pulmonary fibrosis a cancer-like disease? Transcriptome analysis to fuel the debate.ERJ open research · 2019Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue. Then, we investigated the distribution of primary cilia in human IPF tissue samples using immunofluorescence. Tissues from six IPF cases showed an increase in the number of primary cilia in alveolar cells and fibroblasts. In addition, we observed an increase in ciliogenesis related genes such as IFT20 and IFT88 in IPF. Since major components of the SHH signaling pathway are known to be localized in primary cilia, we quantified the mRNA expression of the SHH signaling components using qRT-PCR in both IPF and control lung. mRNA levels of
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.