Fatty acid pattern of platelet phospholipids in cystic fibrosis

Rogiers, Vera; Vercruysse, Antoine; Dab, I.; Crokaert, R; Vis, Henri-Louis

doi:10.1007/bf00540261

Cited by 8 publications

(6 citation statements)

References 6 publications

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“…Docosahexaenoic acid deficiency in CF subjects has been previously reported by studying the fatty acid composition of serum [4] and red blood cell PLs [40], but, as far as we know, the localization of this deficiency in particular molecular species is reported in this paper for the first time. This result might be important because pPE species are known to have an antioxidative action involved in scavenging radicals at the vinyl ether linkage, and to protect membrane against peroxidation [41][42][43].…”

Section: Discussionmentioning

confidence: 68%

“…On the contrary, statistical differences found between normal and CF patients with P ins , in the PC species corresponding to m/z 820.4 and m/z 792,4, account for 3,8%, and the sum of targeted species constitute 12% of this class in normal subjects. Therefore, these data might be considered reliable and fit with the ones from literature obtained in other blood cells by means of GC analysis of fatty acids [40].…”

Section: Discussionmentioning

confidence: 86%

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Normal phase liquid chromatography–electrospray ionization tandem mass spectrometry analysis of phospholipid molecular species in blood mononuclear cells: application to cystic fibrosis

Malavolta

Boselli

Frega

2004

Journal of Chromatography B

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Section: Discussionmentioning

confidence: 68%

Section: Discussionmentioning

confidence: 86%

Normal phase liquid chromatography–electrospray ionization tandem mass spectrometry analysis of phospholipid molecular species in blood mononuclear cells: application to cystic fibrosis

Malavolta

Boselli

Frega

2004

Journal of Chromatography B

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“…Interestingly, parallel to this decrease in PUFA, increases in the levels of SFA, and more specifically Palmitate (16:0) and Stearate (18:0) have also been reported in CF individuals, particularly in circulating lipids, namely NEFA and Cholesteryl esters [36,37] and in PL extracted from lung samples, i. e. bronchial secretions [20] and freshly dissociated bronchial epithelial cells [17,18]. Altogether, these cumulative processes result in an overall FA imbalance towards more saturated species.…”

Section: Constant Dysregulation Of Fatty Acid Profile In Cfmentioning

confidence: 82%

Glued in lipids: Lipointoxication in cystic fibrosis

Vandebrouck

Ferreira

2020

EBioMedicine

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Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane regulator (CFTR) gene, which encodes a chloride channel located at the apical surface of epithelial cells. Unsaturated Fatty Acid (UFA) deficiency has been a persistent observation in tissues from patients with CF. However, the impacts of such deficiencies on the etiology of the disease have been the object of intense debates. The aim of the present review is first to highlight the general consensus on fatty acid dysregulations that emerges from, sometimes apparently contradictory, studies. In a second step, a unifying mechanism for the potential impacts of these fatty acid dysregulations in CF cells, based on alterations of membrane biophysical properties (known as lipointoxication), is proposed. Finally, the contribution of lipointoxication to the progression of the CF disease and how it could affect the efficacy of current treatments is also discussed.

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“…The old report about LA deficiency in CF has been confirmed in different blood compartments [51][52][53] and in various tissues [54][55][56][57]. Many studies of supplementation with LA have been performed, but were often of relatively short duration and, lacking in stable study conditions, not resulting in clear outcomes and motivating general recommendations.…”

Section: Fatty Acid Abnormalities In Cfmentioning

confidence: 99%

Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations

Strandvik

2022

Nutrients

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Nutrition is important in cystic fibrosis (CF) because the disease is associated with a higher energy consumption, special nutritional deficiencies, and malabsorption mainly related to pancreatic insufficiency. The clinical course with deterioration of lung function has been shown to relate to nutrition. Despite general recommendation of high energy intake, the clinical deterioration is difficult to restrain suggesting that special needs have not been identified and specified. It is well-known that the CF phenotype is associated with lipid abnormalities, especially in the essential or conditionally essential fatty acids. This review will concentrate on the qualitative aspects of fat metabolism, which has mainly been neglected in dietary fat recommendations focusing on fat quantity. For more than 60 years it has been known and confirmed that the patients have a deficiency of linoleic acid, an n-6 essential fatty acid of importance for membrane structure and function. The ratio between arachidonic acid and docosahexaenoic acid, conditionally essential fatty acids of the n-6 and n-3 series, respectively, is often increased. The recently discovered relations between the CFTR modulators and lipid metabolism raise new interests in this field and together with new technology provide possibilities to specify further specify personalized therapy.

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Fatty acid pattern of platelet phospholipids in cystic fibrosis

Cited by 8 publications

References 6 publications

Normal phase liquid chromatography–electrospray ionization tandem mass spectrometry analysis of phospholipid molecular species in blood mononuclear cells: application to cystic fibrosis

Normal phase liquid chromatography–electrospray ionization tandem mass spectrometry analysis of phospholipid molecular species in blood mononuclear cells: application to cystic fibrosis

Glued in lipids: Lipointoxication in cystic fibrosis

Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations

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