ArticleCell reports2014
Folliculin controls lung alveolar enlargement and epithelial cell survival through E-cadherin, LKB1, and AMPK.
Article in Cell reports, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 61 papers.
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Who cites it
61 citing papers in PubMed, 101 citations in OpenAlex.
- A novel pathogenic variant in folliculin identified in a Chinese family with BHD syndrome.BMC medical genomics · 2026Article
- Hyperactivation of mTORC1 signaling mediates folliculin deficiency-induced pulmonary cyst formation in Birt-Hogg-Dubé syndrome.The Journal of clinical investigation · 2026Article
- Imaging features of lung cancer with cystic airspaces: clinical utility, challenges, and perspectives.Frontiers in oncology · 2026Review
- Wall disruption and cystic dilatation of interlobular septa in Birt-Hogg-Dubé syndrome.Respiratory medicine case reports · 2026Article
- Free-floating patient-derived organotypic tumor spheroids (PDOTS) from non-small cell lung cancer (NSCLC) tumors: a versatile tool for personalized testing of chemotherapeutic drugs.Research in pharmaceutical sciences · 2025Article
- Evolution of cystic lung disease in Birt-Hogg-Dubé syndrome: assessment of older patients.BMJ open respiratory research · 2025Article
- Features of Lung Cyst in Birt-Hogg-Dubé Syndrome from Patients with Multiple Lung Cysts.Tuberculosis and respiratory diseases · 2025Article
- Lung cancer associated with cystic airspaces: A contemporary review.Tuberkuloz ve toraks · 2024Review
- Repurposing Nitazoxanide for Potential Treatment of Rare Disease Lymphangioleiomyomatosis.Biomolecules · 2024Article
- Hyperactive mTORC1 in lung mesenchyme induces endothelial cell dysfunction and pulmonary vascular remodeling.The Journal of clinical investigation · 2023Article
- Outstanding Characteristics of Birt-Hogg-Dube Syndrome in Korea.Diagnostics (Basel, Switzerland) · 2023Article
- Article
- PRDM10 directs FLCN expression in a novel disorder overlapping with Birt-Hogg-Dubé syndrome and familial lipomatosis.Human molecular genetics · 2023Article
- Birt-Hogg-Dubé syndrome with c.1579_1580insA variant in a Chinese family: a case report.Frontiers in medicine · 2023Article
- Review
- Genetic insight into Birt-Hogg-Dubé syndrome in Indian patients reveals novel mutations at FLCN.Orphanet journal of rare diseases · 2022Article
- Serum Folliculin Is Related to Lower Pulmonary Function in Patients With Asthma.Allergy, asthma & immunology research · 2021Article
- Novel folliculin gene mutations in Polish patients with Birt-Hogg-Dubé syndrome.Orphanet journal of rare diseases · 2021Article
- Folliculin haploinsufficiency causes cellular dysfunction of pleural mesothelial cells.Scientific reports · 2021Article
- Folliculin: A Regulator of Transcription Through AMPK and mTOR Signaling Pathways.Frontiers in cell and developmental biology · 2021Review
1 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
15 authors at 6 institutions in 1 country.
Funding
Abstract
Spontaneous pneumothoraces due to lung cyst rupture afflict patients with the rare disease Birt-Hogg-Dubé (BHD) syndrome, which is caused by mutations of the tumor suppressor gene folliculin (FLCN). The underlying mechanism of the lung manifestations in BHD is unclear. We show that BHD lungs exhibit increased alveolar epithelial cell apoptosis and that Flcn deletion in mouse lung epithelium leads to cell apoptosis, alveolar enlargement, and an impairment of both epithelial barrier and overall lung function. We find that Flcn-null epithelial cell apoptosis is the result of impaired AMPK activation and increased cleaved caspase-3. AMPK activator LKB1 and E-cadherin are downregulated by Flcn loss and restored by its expression. Correspondingly, Flcn-null cell survival is rescued by the AMPK activator AICAR or constitutively active AMPK. AICAR also improves lung condition of Flcn(f/f):SP-C-Cre mice. Our data suggest that lung cysts in BHD may result from an underlying defect in alveolar epithelial cell survival, attributable to FLCN regulation of the E-cadherin-LKB1-AMPK axis.
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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.