scholarly journals Determinants of Plasma Docosahexaenoic Acid Levels and Their Relationship to Neurological and Cognitive Functions in PKU Patients: A Double Blind Randomized Supplementation Study

Nutrients ◽  
2018 ◽  
Vol 10 (12) ◽  
pp. 1944 ◽  
Author(s):  
Hans Demmelmair ◽  
Anita MacDonald ◽  
Urania Kotzaeridou ◽  
Peter Burgard ◽  
Domingo Gonzalez-Lamuno ◽  
...  

Children with phenylketonuria (PKU) follow a protein restricted diet with negligible amounts of docosahexaenoic acid (DHA). Low DHA intakes might explain subtle neurological deficits in PKU. We studied whether a DHA supply modified plasma DHA and neurological and intellectual functioning in PKU. In a double-blind multicentric trial, 109 PKU patients were randomized to DHA doses from 0 to 7 mg/kg&day for six months. Before and after supplementation, we determined plasma fatty acid concentrations, latencies of visually evoked potentials, fine and gross motor behavior, and IQ. Fatty acid desaturase genotypes were also determined. DHA supplementation increased plasma glycerophospholipid DHA proportional to dose by 0.4% DHA per 1 mg intake/kg bodyweight. Functional outcomes were not associated with DHA status before and after intervention and remained unchanged by supplementation. Genotypes were associated with plasma arachidonic acid levels and, if considered together with the levels of the precursor alpha-linolenic acid, also with DHA. Functional outcomes and supplementation effects were not significantly associated with genotype. DHA intakes up to 7 mg/kg did not improve neurological functions in PKU children. Nervous tissues may be less prone to low DHA levels after infancy, or higher doses might be required to impact neurological functions. In situations of minimal dietary DHA, endogenous synthesis of DHA from alpha-linolenic acid could relevantly contribute to DHA status.

2008 ◽  
Vol 49 (9) ◽  
pp. 1963-1980 ◽  
Author(s):  
James C. DeMar ◽  
Carmine DiMartino ◽  
Adam W. Baca ◽  
William Lefkowitz ◽  
Norman Salem

Microbiology ◽  
2004 ◽  
Vol 150 (6) ◽  
pp. 1983-1990 ◽  
Author(s):  
Takahiro Oura ◽  
Susumu Kajiwara

Fungi, like plants, are capable of producing the 18-carbon polyunsaturated fatty acids linoleic acid and α-linolenic acid. These fatty acids are synthesized by catalytic reactions of Δ12 and ω3 fatty acid desaturases. This paper describes the first cloning and functional characterization of a yeast ω3 fatty acid desaturase gene. The deduced protein encoded by the Saccharomyces kluyveri FAD3 gene (Sk-FAD3) consists of 419 amino acids, and shows 30–60 % identity with Δ12 fatty acid desaturases of several eukaryotic organisms and 29–31 % identity with ω3 fatty acid desaturases of animals and plants. During Sk-FAD3 expression in Saccharomyces cerevisiae, α-linolenic acid accumulated only when linoleic acid was added to the culture medium. The disruption of Sk-FAD3 led to the disappearance of α-linolenic acid in S. kluyveri. These findings suggest that Sk-FAD3 is the only ω3 fatty acid desaturase gene in this yeast. Furthermore, transcriptional expression of Sk-FAD3 appears to be regulated by low-temperature stress in a manner different from the other fatty acid desaturase genes in S. kluyveri.


2017 ◽  
Vol 66 (2) ◽  
pp. 181-185 ◽  
Author(s):  
Yasutoshi Ando ◽  
Shinichiro Saito ◽  
Nami Yamanaka ◽  
Chizuka Suzuki ◽  
Takahiro Ono ◽  
...  

2006 ◽  
Vol 131 (2) ◽  
pp. 284-289 ◽  
Author(s):  
Mauricio A. Cañoles ◽  
Randolph M. Beaudry ◽  
Chuanyou Li ◽  
Gregg Howe

Six-carbon aldehydes and alcohols formed by tomato (Lycopersicon esculentum Mill.) leaf and fruit tissue following disruption are believed to be derived from the degradation of lipids and free fatty acids. Collectively, these C-6 volatiles comprise some of the most important aroma impact compounds. If fatty acids are the primary source of tomato volatiles, then an alteration in the fatty acid composition such as that caused by a mutation in the chloroplastic omega-3-fatty acid desaturase (ω-3 FAD), referred to as LeFAD7, found in the mutant line of `Castlemart' termed Lefad7, would be reflected in the volatile profile of disrupted leaf and fruit tissue. Leaves and fruit of the Lefad7 mutant had ≈10% to 15% of the linolenic acid (18:3) levels and about 1.5- to 3-fold higher linoleic acid (18:2) levels found in the parent line. Production of unsaturated C-6 aldehydes Z-3-hexenal, Z-3-hexenol, and E-2-hexenal and the alcohol Z-3-hexenol derived from 18:3 was markedly reduced in disrupted leaf and fruit tissue of the Lefad7 mutant line. Conversely, the production of the saturated C-6 aldehyde hexanal and its alcohol, hexanol, were markedly higher in the mutant line. The shift in the volatile profile brought about by the loss of chloroplastic FAD activity in the Lefad7 line was detected by sensory panels at high significance levels (P < 0.0005) and detrimentally affected fruit sensory quality. The ratios and amounts of C-6 saturated and unsaturated aldehydes and alcohols produced by tomato were dependent on substrate levels, suggesting that practices that alter the content of linoleic and linolenic acids or change their ratios can influence tomato flavor.


Crop Science ◽  
2005 ◽  
Vol 45 (5) ◽  
pp. 1830-1836 ◽  
Author(s):  
Kristin Bilyeu ◽  
Lavanya Palavalli ◽  
David Sleper ◽  
Paul Beuselinck

2020 ◽  
Vol 111 (5) ◽  
pp. 1068-1078 ◽  
Author(s):  
Susan Sergeant ◽  
Brian Hallmark ◽  
Rasika A Mathias ◽  
Tammy L Mustin ◽  
Priscilla Ivester ◽  
...  

ABSTRACT Background Unexplained heterogeneity in clinical trials has resulted in questions regarding the effectiveness of ɣ-linolenic acid (GLA)-containing botanical oil supplements. This heterogeneity may be explained by genetic variation within the fatty acid desaturase (FADS) gene cluster that is associated with circulating and tissue concentrations of arachidonic acid (ARA) and dihomo-ɣ-linolenic acid (DGLA), both of which may be synthesized from GLA and result in proinflammatory and anti-inflammatory metabolites, respectively. Objectives The objective of this study was to prospectively compare the capacity of a non-Hispanic white cohort, stratified by FADS genotype at the key single-nucleotide polymorphism (SNP) rs174537, to metabolize 18-carbon omega-6 (n-6) PUFAs in borage oil (BO) and soybean oil (SO) to GLA, DGLA, and ARA. Methods Healthy adults (n = 64) participated in a randomized, double-blind, crossover intervention. Individuals received encapsulated BO (Borago officinalis L.; 37% LA and 23% GLA) or SO [Glycine max (L.) Merr.; 50% LA and 0% GLA] for 4 wk, followed by an 8-wk washout period, before consuming the opposite oil for 4 wk. Serum lipids and markers of inflammation (C-reactive protein) were assessed for both oil types at baseline and during weeks 2 and 4 of the intervention. Results SO supplementation failed to alter circulating concentrations of any n-6 long-chain PUFAs. In contrast, a modest daily dose of BO elevated serum concentrations of GLA and DGLA in an rs174537 genotype–dependent manner. In particular, DGLA increased by 57% (95% CI: 0.38, 0.79) in GG genotype individuals, but by 141% (95% CI: 1.03, 2.85) in TT individuals. For ARA, baseline concentrations varied substantially by genotype and increased modestly with BO supplementation, suggesting a key role for FADS variation in the balance of DGLA and ARA. Conclusions The results of this study clearly suggest that personalized and population-based approaches considering FADS genetic variation may be necessary to optimize the design of future clinical studies with GLA-containing oils. This trial was registered at clinicaltrials.gov as NCT02337231.


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