ArticleAmerican journal of physiology. Lung cellular and molecular physiology2008
cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells.
Article in American journal of physiology. Lung cellular and molecular physiology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
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Who cites it
30 citing papers in PubMed, 41 citations in OpenAlex.
- Cystic Fibrosis-related neurodegenerative disease associated with tauopathy and cognitive decline in aged CF mice.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes.American journal of physiology. Lung cellular and molecular physiology · 2022Article
- Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis.Biomolecules · 2021Article
- Resveratrol restores intracellular transport in cystic fibrosis epithelial cells.American journal of physiology. Lung cellular and molecular physiology · 2020Article
- HDAC6 depletion improves cystic fibrosis mouse airway responses to bacterial challenge.Scientific reports · 2019Article
- Acetyl-CoA carboxylase inhibition regulates microtubule dynamics and intracellular transport in cystic fibrosis epithelial cells.American journal of physiology. Lung cellular and molecular physiology · 2019Article
- Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.The Journal of clinical investigation · 2019Article
- Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Therapeutic Potential of Targeting ß-Arrestin.Frontiers in pharmacology · 2019Review
- CFTR Deletion Confers Mitochondrial Dysfunction and Disrupts Lipid Homeostasis in Intestinal Epithelial Cells.Nutrients · 2018Article
- Neutrophil Membrane Cholesterol Content is a Key Factor in Cystic Fibrosis Lung Disease.EBioMedicine · 2017Article
- Cell Plasma Membrane Cholesterol as a Diagnostic.Current opinion in electrochemistry · 2017Article
- Ibuprofen regulation of microtubule dynamics in cystic fibrosis epithelial cells.American journal of physiology. Lung cellular and molecular physiology · 2016Article
- Multifaceted role of β-arrestins in inflammation and disease.Genes and immunity · 2015Review
- Role of Exchange Protein Activated by cAMP 1 in Regulating Rates of Microtubule Formation in Cystic Fibrosis Epithelial Cells.American journal of respiratory cell and molecular biology · 2015Article
- The emerging roles of β-arrestins in fibrotic diseases.Acta pharmacologica Sinica · 2015Review
- FOXO1 content is reduced in cystic fibrosis and increases with IGF-I treatment.International journal of molecular sciences · 2014Article
- The stimulation of adenosine A2A receptors ameliorates the pathological phenotype of fibroblasts from Niemann-Pick type C patients.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- β-Arrestins in the immune system.Progress in molecular biology and translational science · 2013Review
- Increased Expression of RhoA in Epithelium and Smooth Muscle of Obese Mouse Models: Implications for Isoprenoid Control of Airway Smooth Muscle and Fibroblasts.Journal of allergy · 2013Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
The goal of this study was to identify a mechanism regulating cholesterol accumulation in cystic fibrosis (CF) cells. Both CFTR activation and expression are regulated by the cAMP pathway, and it is hypothesized that a feedback response involving this pathway may be involved in the phenotype of cholesterol accumulation. To examine the role of the cAMP pathway in cholesterol accumulation, we treated two CF model cell lines with the Rp diastereomer of adenosine 3',5'-cyclic monophosphorothioate (Rp-cAMPS) and visualized by filipin staining. Rp-cAMPS treatment eliminated cholesterol accumulation in CF cells, whereas 8-bromo-cAMP treatment led to cholesterol accumulation in wild-type cells. To confirm these findings in an independent model system, we also examined the role of cAMP in modulating cholesterol accumulation in Niemann-Pick type C (NPC) fibroblasts. Expression of the protein related to NPC, NPC1, is also directly regulated by cAMP; therefore, it is postulated that NPC cells exhibit the same cAMP-mediated control of cholesterol accumulation. Cholesterol accumulation in NPC cells also was reduced by the presence of Rp-cAMPS. Expression of beta-arrestin-2 (betaarr2), a marker of cellular response to cAMP signaling, was significantly elevated in CF model cells, Cftr(-/-) MNE, primary tissue obtained by nasal scrapes from CF subjects, and in NPC fibroblasts compared with respective controls.
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