ArticleProceedings of the National Academy of Sciences of the United States of America2007
Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
What it found
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Who cites it
23 citing papers in PubMed, 34 citations in OpenAlex.
- The Acid sphingomyelinase/ceramide System in Idiopathic Inflammatory Myopathies: a Potential Treatment Target.Inflammation · 2026Review
- Cross-talk between CFTR and sphingolipids in cystic fibrosis.FEBS open bio · 2023Review
- Bacterial phospholipases C with dual activity: phosphatidylcholinesterase and sphingomyelinase.FEBS open bio · 2021Review
- Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs.Physiological reports · 2021Article
- The Impact of Dietary Sphingolipids on Intestinal Microbiota and Gastrointestinal Immune Homeostasis.Frontiers in immunology · 2021Review
- Review
- An Innovative Lipidomic Workflow to Investigate the Lipid Profile in a Cystic Fibrosis Cell Line.Cells · 2020Article
- The bidirectional relationship between CFTR and lipids.Communications biology · 2020Review
- Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.American journal of physiology. Lung cellular and molecular physiology · 2019Article
- Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR).Scientific reports · 2017Article
- Bacterial Sphingomyelinases and Phospholipases as Virulence Factors.Microbiology and molecular biology reviews : MMBR · 2016Review
- Sphingomyelinase D inhibits store-operated Ca2+ entry in T lymphocytes by suppressing ORAI current.The Journal of general physiology · 2015Article
- Article
- Tuning voltage-gated channel activity and cellular excitability with a sphingomyelinase.The Journal of general physiology · 2013Article
- Role of pore-forming toxins in neonatal sepsis.Clinical & developmental immunology · 2013Review
- A limited 4 Å radial displacement of the S4-S5 linker is sufficient for internal gate closing in Kv channels.The Journal of biological chemistry · 2012Article
- Massive calcium-activated endocytosis without involvement of classical endocytic proteins.The Journal of general physiology · 2011Article
- Exogenous and endogenous ceramides elicit volume-sensitive chloride current in ventricular myocytes.Cardiovascular research · 2010Article
- Long acting beta2-agonist and corticosteroid restore airway glandular cell function altered by bacterial supernatant.Respiratory research · 2010Article
- Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis.American journal of respiratory cell and molecular biology · 2009Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Numerous mutations in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR, a Cl(-) channel) disrupt salt and fluid transport and lead to the formation of thick mucus in patients' airways. Obstruction by mucus predisposes CF patients to chronic infections and inflammation, which become gradually harder to control and eventually fatal. Aggressive antibiotic therapy and supportive measures have dramatically lengthened CF patients' lives. Here, we report that sphingomyelinases (SMase) from human respiratory pathogens strongly inhibit CFTR function. The hydrolysis of sphingomyelin by SMase makes it more difficult to activate CFTR by phosphorylation of its regulatory domain. By inhibiting CFTR currents, SMase-producing respiratory tract bacteria may not only aggravate pulmonary infection in some CF patients but may also elicit a condition, analogous to CFTR deficiency, in non-CF patients suffering from bacterial lung infection.
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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.