Biochemistry of very-long-chain and long-chain ceramides in cystic fibrosis and other diseases: The importance of side chain

Publication date: Available online 21 August 2019Source: Progress in Lipid ResearchAuthor(s): Dušan Garić, Juan B. De Sanctis, Juhi Shah, Daciana Catalina Dumut, Danuta RadziochAbstractCeramides, the principal building blocks of all sphingolipids, have attracted the attention of many scientists around the world interested in developing treatments for cystic fibrosis, the most common genetic disease of Caucasians. Many years of fruitful research in this field have produced some fundamentally important, yet controversial results.Here, we aimed to summarize the current knowledge on the role of long- and very-long- chain ceramides, the most abundant species of ceramides in animal cells, in cystic fibrosis and other diseases. We also aim to explain the importance of the length of their side chain in the context of stability of transmembrane proteins through a concise synthesis of their biophysical chemistry, cell biology, and physiology. This review also addresses several remaining riddles in this field.Finally, we discuss the technical challenges associated with the analysis and quantification of ceramides. We provide the evaluation of the antibodies used for ceramide quantification and we demonstrate their lack of specificity. Results and discussion presented here will be of interest to anyone studying these enigmatic lipids.
Source: Progress in Lipid Research - Category: Lipidology Source Type: research

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Source: Expert Review of Respiratory Medicine - Category: Respiratory Medicine Tags: Expert Rev Respir Med Source Type: research
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Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Letters Source Type: research
Cystic Fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, resulting in defective CFTR-mediated chloride and bicarbonate transport, with dysregulation of epithelial sodium channels (ENaC). These changes alter fluid and electrolyte homeostasis and result in an exaggerated proinflammatory response driven, in part, by infection. We tested the hypothesis that NLRP3-inflammasome activation and ENaC upregulation drives exaggerated innate-immune responses in this multisystem disease. We identify an enhanced proinflammatory signature, as evidenced by incr...
Source: eLife - Category: Biomedical Science Tags: Human Biology and Medicine Immunology and Inflammation Source Type: research
Source: BMJ News - Category: General Medicine Source Type: research
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Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
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Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
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Source: Respiratory Medicine - Category: Respiratory Medicine Authors: Tags: Short communication Source Type: research
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Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
Condition:   Bronchiectasis Intervention:   Drug: Roflumilast Sponsor:   Assiut University Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
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Source: the Mail online | Health - Category: Consumer Health News Source Type: news
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