The fundamentals and practice of docosahexaenoic acid therapy in peroxisomal disorders

Martínez, Manuela

doi:10.1097/00075197-200003000-00004

Cited by 4 publications

(4 citation statements)

References 61 publications

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“…Increased dietary intake of n-3 versus n-6 PUFA results in enhanced transcription of antioxidant enzymes (uncoupling protein 2, glutathione transferase 2τ, superoxide dismutase) and in suppressed transcription of enzymes taking part in the production of reactive oxygen and nitrogen species (RONS). Intake of n-6 PUFA increased activities of glutathione peroxidase, superoxide dismutase and catalase 187,188 . Increased severity of the metabolic syndrome has been associated with increased oxidative stress and unfavourable fatty acid metabolism.…”

Section: Oxidative Stressmentioning

confidence: 96%

“…EPA are more hydrophilic than those generated from LA. They diffuse more readily through lipoprotein envelopes and radical reaction can be terminated more rapidly on the lipoprotein surface 186,187 . Increased dietary intake of n-3 versus n-6 PUFA results in enhanced transcription of antioxidant enzymes (uncoupling protein 2, glutathione transferase 2τ, superoxide dismutase) and in suppressed transcription of enzymes taking part in the production of reactive oxygen and nitrogen species (RONS).…”

Section: Oxidative Stressmentioning

confidence: 99%

“…Isolated ethyl esters of DHA may improve clinical and biochemical parameters in children with disorders of peroxisome biogenesis (Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease) (ref. 187,249 ). Enteral supplementation with lipid emulsion containing LC n-3 PUFA and MCT had beneficial effects in patients with chylomicronemic syndrome combined with severe acute pancreatitis.…”

Section: Critically Ill Patients Cancer and Other Chronic Diseasesmentioning

confidence: 99%

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Fatty Acids as Biocompounds: Their Role in Human Metabolism, Health and Disease - A Review. Part 2: Fatty Acid Physiological Roles and Applications in Human Health and Disease

Kremmyda¹,

Tvrzická²,

Staňková³

et al. 2011

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

152

134

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Background. This is the second of two review parts aiming at describing the major physiological roles of fatty acids, as well as their applications in specific conditions related to human health.Results. The review included the current literature published in Pubmed up to March 2011. In humans, fatty acids are a principle energy substrate and structural components of cell membranes (phospholipids) and second messengers. Fatty acids are also ligands of nuclear receptors affecting gene expression. Longer-chain (LC) polyunsaturated fatty acids (PUFA), including eicosapentaenoic acid (EPA), docosahexaenoic acid (DHA), and arachidonic acid are precursors of lipid mediators such as eicosanoids (prostaglandins, leukotrienes, thromboxanes), resolvins and neuroprotectins. Lipid mediators produced by EPA and DHA (LC n-3 PUFA; mainly found in oily fish) are considered as inflammation-resolving, and thus, fish oil has been characterised as antiinflammatory. Recommendations for EPA plus DHA intake from oily fish vary between 250-450 mg/day. Dietary reference values for fat vary between nutrition bodies, but mainly agree on a low total and saturated fat intake. The existing literature supports the protective effects of LC n-3 PUFA (as opposed to n-6 PUFA and saturated fat) in maternal and offspring health, cardiovascular health, insulin sensitivity, the metabolic syndrome, cancer, critically ill patients, and immune system disorders.Conclusion. Fatty acids are involved in multiple pathways and play a major role in health. Further investigation and a nutrigenomics approach to the effects of these biocompounds on health and disease development are imperative and highlight the importance of environmental modifications on disease outcome.humans. Thus, these FA may be characterised as conditionally essential depending on EFA availability. Genetic variation in human desaturase genes affects FA metabolism, plasma lipid profiles, and risk of disease development [5][6][7] . Recommendations for minimum dietary intake of EPA plus DHA vary between 250-450 mg/day 8 , especially for pregnant women and those of reproductive age. Rich sources of LC n-3 PUFA are fish oils and the flesh of oily fish, whereas non-oily (white) fish contain them but in lower amounts.Long-chain PUFA with 18-20 carbon atoms, including EPA and AA, are precursors of eicosanoids (prostaglandins, leucotrienes and thromboxanes), which have a broad scale of regulatory, autocrine and paracrine effects. Long-chain PUFA with 20 and 22 carbon atoms are precursors of autacoids -resolvins (resolution phase interaction products), lipoxins and neuroprotectins 9 . FA are also ligands of nuclear receptors which take part in the subcellular control of metabolic pathways. Covalent modification of proteins by FA acylation enables their incorporation into membranes. Hydroxy FA are activators of some nuclear factors and are responsible for the expression of proinflammatory cytokines (interleukin (IL)-1,

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Section: Oxidative Stressmentioning

confidence: 96%

Section: Oxidative Stressmentioning

confidence: 99%

Section: Critically Ill Patients Cancer and Other Chronic Diseasesmentioning

confidence: 99%

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Fatty Acids as Biocompounds: Their Role in Human Metabolism, Health and Disease - A Review. Part 2: Fatty Acid Physiological Roles and Applications in Human Health and Disease

Kremmyda¹,

Tvrzická²,

Staňková³

et al. 2011

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

152

134

View full text Add to dashboard Cite

show abstract

“…Studies in which subcellular organelles were isolated and then recombined (Li et al 2000) and reports of the action of the specific D6-desaturase inhibitor SC-26 196 (Harmon et al 2003) strongly support the second pathway. Furthermore, patients with peroxisome disorders such as Zellweger's syndrome have lower plasma DHA concentration, which is the result of a failure to convert DPA to DHA in the absence of an active peroxisomal b-oxidation pathway (Martinez, 2000). Thus the consensus pathway for the conversion of DPA to DHA is as follows: DPA is elongated to 24 : 5n-3 which is desaturated at the D6-position by the action of D6-desaturase activity to form 24 : 6n-3.…”

Section: Recycling Of Carbon From A-linolenic Acid Into Saturated Andmentioning

confidence: 99%

Dietary α-linolenic acid and health-related outcomes: a metabolic perspective

2006

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α-Linolenic acid (αLNA; 18: 3n-3) is essential in the human diet, probably because it is the substrate for the synthesis of longer-chain, more unsaturatedn-3 fatty acids, principally EPA (20: 5n-3) and DHA (22: 6n-3), which confer important biophysical properties on cell membranes and so are required for tissue function. The extent to which this molecular transformation occurs in man is controversial. The present paper reviews the recent literature on the metabolism of αLNA in man, including the use of dietary αLNA in β-oxidation, recycling of carbon by fatty acid synthesisde novoand conversion to longer-chain PUFA. Sex differences in αLNA metabolism and the possible biological consequences are discussed. Increased consumption of EPA and DHA in fish oil has a number of well-characterised beneficial effects on health. The present paper also reviews the efficacy of increased αLNA consumption in increasing the concentrations of EPA and DHA in blood and cell lipid pools, and the extent to which such dietary interventions might be protective against CVD and inflammation. Although the effects on CVD risk factors and inflammatory markers are variable, where beneficial effects have been reported these are weaker than have been achieved from increasing consumption of EPA+DHA or linoleic acid. Overall, the limited capacity for conversion to longer-chainn-3 fatty acids, and the lack of efficacy in ameliorating CVD risk factors and inflammatory markers in man suggests that increased consumption of αLNA may be of little benefit in altering EPA+DHA status or in improving health outcomes compared with other dietary interventions.

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Mechanisms of Toxicity in Fatty Acid Oxidation Disorders

Sharer

2009

Endogenous Toxins

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The fundamentals and practice of docosahexaenoic acid therapy in peroxisomal disorders

Cited by 4 publications

References 61 publications

Fatty Acids as Biocompounds: Their Role in Human Metabolism, Health and Disease - A Review. Part 2: Fatty Acid Physiological Roles and Applications in Human Health and Disease

Fatty Acids as Biocompounds: Their Role in Human Metabolism, Health and Disease - A Review. Part 2: Fatty Acid Physiological Roles and Applications in Human Health and Disease

Dietary α-linolenic acid and health-related outcomes: a metabolic perspective

Mechanisms of Toxicity in Fatty Acid Oxidation Disorders

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