AI Article Synopsis
- The introduction of modulators, correctors, and potentiators for the CFTR molecule has significantly improved the health of most individuals with cystic fibrosis (CF).
- While these therapies impact certain fatty acids, they do not address linoleic acid (LA) deficiency, which is linked to more severe forms of CF.
- The review examines lipid abnormalities in CF patients, such as altered phospholipid and cholesterol levels, and their connection to clinical symptoms, revealing these lipid issues can manifest before birth.
Article Abstract
The care for cystic fibrosis (CF) has dramatically changed with the development of modulators, correctors, and potentiators of the CFTR molecule, which lead to improved clinical status of most people with CF (pwCF). The modulators influence phospholipids and ceramides, but not linoleic acid (LA) deficiency, associated with more severe phenotypes of CF. The LA deficiency is associated with upregulation of its transfer to arachidonic acid (AA). The AA release from membranes is increased and associated with increase of pro-inflammatory prostanoids and the characteristic inflammation is present before birth and bacterial infections. Docosahexaenoic acid is often decreased, especially in associated liver disease Some endogenously synthesized fatty acids are increased. Cholesterol and ceramide metabolisms are disturbed. The lipid abnormalities are present at birth, and before feeding in transgenic pigs and ferrets. This review focus on the lipid abnormalities and their associations to clinical symptoms in CF, based on clinical studies and experimental research.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11607544 | PMC |
| http://dx.doi.org/10.1016/j.isci.2024.111153 | DOI Listing |
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