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Brain Pathology (Zurich, Switzerland) Jul 2010Mutations in the ABCD1 gene cause the clinical spectrum of the neurometabolic disorder X-linked adrenoleukodystrophy/adrenomyeloneuropathy (X-ALD/AMN). Currently, the... (Review)
Review
Mutations in the ABCD1 gene cause the clinical spectrum of the neurometabolic disorder X-linked adrenoleukodystrophy/adrenomyeloneuropathy (X-ALD/AMN). Currently, the most efficient therapeutic opportunity for patients with the cerebral form of X-ALD is hematopoietic stem cell transplantation and possibly gene therapy of autologous hematopoietic stem cells. Both treatments, however, are only accessible to a subset of X-ALD patients, mainly because of the lack of markers that can predict the onset of cerebral demyelination. Moreover, for female or male X-ALD patients with AMN, currently only unsatisfying therapeutic opportunities are available. Thus, this review focuses on current and urgently needed future pharmacological therapies. The treatment of adrenal and gonadal insufficiency is well established, whereas applications of immunomodulatory and immunosuppressive drugs have failed to prevent progression of cerebral neuroinflammation. The use of Lorenzo's oil and the inefficacy of lovastatin to normalize very-long-chain fatty acids in clinical trials as well as currently experimental and therefore possible future therapeutic strategies are reviewed. The latter include pharmacological gene therapy mediated by targeted upregulation of ABCD2, the closest homolog of ABCD1, antioxidative drug treatment, small molecule histone deacetylase inhibitors such as butyrates and valproic acid, and other neuroprotective attempts.
Topics: Adrenoleukodystrophy; Drug Combinations; Erucic Acids; Fatty Acids; Hematopoietic Stem Cell Transplantation; Humans; Triolein
PubMed: 20626746
DOI: 10.1111/j.1750-3639.2010.00393.x -
Iranian Journal of Biotechnology Jan 2022is one of the most important oilseeds that has a proportionate profile of essential unsaturated fatty acids that are suitable for human nutrition. In this regard, we...
BACKGROUND
is one of the most important oilseeds that has a proportionate profile of essential unsaturated fatty acids that are suitable for human nutrition. In this regard, we can mention a high percentage and a reasonable ratio of omega 3 and omega 6.
OBJECTIVES
In the current study, the created variation of second-generation mutant (M2) camelina lines in terms of fatty acid profiles and ISSR molecular markers in was evaluated.
MATERIALS AND METHODS
For this purpose, while producing the first-generation of mutant plants (M1), 200 M2 seeds with 0.1% and 0.5% ethyl methanesulfonate (EMS) mutations were treated in two replications for 8 and 16 hours based on a completely randomized design.
RESULTS
The results of mean comparisons showed that there was no significant difference between treatments in terms of fatty acids of palmitic acid, stearic acid, linoleic acid, eicosadienoic acid, oleic acid and erucic acid. The cluster analysis revealed that all the treatments used with five replications were divided into eight groups. It was found that all replications of the treatment with a concentration of 0.1% and a time of 16 hours (C1T2) were in the second group with the lowest palmitic acid was present among other treatments. Therefore, C1T2 treatment is recommended as the best treatment to reduce palmitic acid. Examination of the information content of ISSR molecular markers also showed that markers 2, 5, and 6 were the best informative markers in the detection of camelina fatty acid profiles.
CONCLUSION
A significant variation has been created in the fatty acids profile and it can be applied in future breeding programs depending on the intended purpose.
PubMed: 35891951
DOI: 10.30498/ijb.2021.263748.2948 -
Brazilian Journal of Biology = Revista... 2021The aim of the present study was to determine and compare the fatty acids, lipid soluble vitamins, sterols, phenolics, and antioxidant capacities of three endemic Salvia...
The aim of the present study was to determine and compare the fatty acids, lipid soluble vitamins, sterols, phenolics, and antioxidant capacities of three endemic Salvia L. taxa (S. euphratica var. Montbret & Aucher ex Bentham leiocalycina (Rech. Fil.) Hedge, S. euphratica var. Montbret & Aucher ex Bentham euphratica (Rech. Fil.) Hedge and S. pseudoeuphratica Rech.Fil.) and to evaluate these results systematically. The fatty acid compositions were determined by using gas chromatography, while the lipid soluble vitamins, sterols, and phenolics were determined by using HPLC. Also, the antioxidant capacities of three Salvia taxa were measured in vitro. Palmitic acid was found as major saturated fatty acid while oleic acid, linoleic acid, α-linolenic acid, and erucic acid were found as major unsaturated fatty acids in the present study. It was found that S. euphratica var. euphratica had lower palmitic acid (8.94 ± 0.71%), total saturated fatty acid (19.16 ± 0.15%), and higher unsaturated fatty acid content (82.08 ± 0.52%) than other studied taxa. Furthermore, it was shown that S. euphratica var. euphratica had different 18.3/18:2 (0.36) unsaturated/saturated fatty acid (4.28) ratios. However, this study demonstrated that Salvia taxa had low lipid soluble vitamins, sterol contents. On the other hand, it was shown that Salvia taxa had similar catechin (509.2 ± 4.21 µg/g and 552.2 ± 9.21 µg/g) and vanillic acid amounts (351.2 ± 2.17 µg/g and 396.8 ± 4.1 µg/g) in this study. And also, it was found that Salvia taxa had high rosmarinic acid content while S. euphratica var. leiocalycina had the highest rosmarinic acid content (1480 ± 7.57 µg/g). On the other hand, it was shown that the two ferulic acid contents of S. euphratica varieties were higher (1175 ±/5.21 µgmg-1740.2 ± 4.82 µg/mg) than the ferulic acid content of S. pseudoeuphratica of which was the lowest (19.2 ± 0.97 µg/mg). The present results suggested that the biochemical results guided the morphological studies, and Salvia taxa have a potent antioxidant capacity.
Topics: Antioxidants; Fatty Acids; Plant Extracts; Salvia; Turkey
PubMed: 34190799
DOI: 10.1590/1519-6984.239539 -
TheScientificWorldJournal 2012Some Saudi herbs and spices were analyzed. The results indicated that mustard, black cumin, and cress seeds contain high amount of fat 38.45%, 31.95% and 23.19%,...
Some Saudi herbs and spices were analyzed. The results indicated that mustard, black cumin, and cress seeds contain high amount of fat 38.45%, 31.95% and 23.19%, respectively, as compared to clove (16.63%), black pepper (5.34%) and fenugreek (4.51%) seeds. Cress, mustard, black cumin and black pepper contain higher protein contents ranging from 26.61 to 25.45%, as compared to fenugreek (12.91%) and clove (6.9%). Crude fiber and ash content ranged from 6.36 to 23.6% and from 3.57 to 7.1%, respectively. All seeds contain high levels of potassium (ranging from 383 to 823 mg/100 g), followed by calcium (ranging from 75 to 270 mg/100 g), Magnesium (ranged from 42 to 102 mg/100 g) and iron (ranged from 20.5 to 65 mg/100 g). However, zinc, manganese and copper were found at low levels. The major fatty acids in cress and mustard were linolenic acid (48.43%) and erucic acid (29.81%), respectively. The lenoleic acid was the major fatty acid in black cumin, fenugreek, black pepper and clove oils being 68.07%, 34.85%, 33.03% and 44.73%, respectively. Total unsaturated fatty acids were 83.24, 95.62, 86.46, 92.99, 81.34 and 87.82% for cress, mustard, black cumin, fenugreek, black pepper and clove, respectively. The differences in the results obtained are due to environmental factors, production areas, cultivars used to produce seeds and also due to the different methods used to prepare these local spices.
Topics: Dianthus; Environment; Fatty Acids; Lepidium; Nigella sativa; Nutritive Value; Piper nigrum; Saudi Arabia; Sinapis; Spices; Trigonella
PubMed: 23319888
DOI: 10.1100/2012/859892 -
Frontiers in Nutrition 2023A previous double-blind, randomized clinical trial of 42 healthy individuals conducted with N6.2 found that the probiotic's mechanistic tryptophan pathway was...
A previous double-blind, randomized clinical trial of 42 healthy individuals conducted with N6.2 found that the probiotic's mechanistic tryptophan pathway was significantly modified when the data was stratified based on the individuals' lactic acid bacteria (LAB) stool content. These results suggest that confounding factors such as dietary intake which impact stool LAB content may affect the response to the probiotic treatment. Using dietary intake, serum metabolite, and stool LAB colony forming unit (CFU) data from a previous clinical trial, the relationships between diet, metabolic response, and fecal LAB were assessed. The diets of subject groups with high vs. low CFUs of LAB/g of wet stool differed in their intakes of monounsaturated fatty acids, vegetables, proteins, and dairy. Individuals with high LAB consumed greater amounts of cheese, fermented meats, soy, nuts and seeds, alcoholic beverages, and oils whereas individuals with low LAB consumed higher amounts of tomatoes, starchy vegetables, and poultry. Several dietary variables correlated with LAB counts; positive correlations were determined for nuts and seeds, fish high in N-3 fatty acids, soy, and processed meats, and negative correlations to consumption of vegetables including tomatoes. Using machine learning, predictors of LAB count included cheese, nuts and seeds, fish high in N-3 fatty acids, and erucic acid. Erucic acid alone accurately predicted LAB categorization, and was shown to be utilized as a sole fatty acid source by several species regardless of their mode of fermentation. Several metabolites were significantly upregulated in each group based on LAB titers, notably polypropylene glycol, caproic acid, pyrazine, and chondroitin sulfate; however, none were correlated with the dietary intake variables. These findings suggest that dietary variables may drive the presence of LAB in the human gastrointestinal tract and potentially impact response to probiotic interventions.
PubMed: 37153913
DOI: 10.3389/fnut.2023.1118679 -
PloS One 2016This study investigated the relationship between erythrocyte membrane fatty acid (FA) levels and the severity of symptoms of individuals at ultra-high risk (UHR) for... (Randomized Controlled Trial)
Randomized Controlled Trial
This study investigated the relationship between erythrocyte membrane fatty acid (FA) levels and the severity of symptoms of individuals at ultra-high risk (UHR) for psychosis. Subjects of the present study consisted of 80 neuroleptic-naïve UHR patients. Partial correlation coefficients were calculated between baseline erythrocyte membrane FA levels, measured by gas chromatography, and scores on the Positive and Negative Syndrome Scale (PANSS), Global Assessment of Functioning Scale, and Montgomery-Asberg Depression Rating Scale (MADRS) after controlling for age, sex, smoking and cannabis use. Subjects were divided into three groups according to the predominance of positive or negative symptoms based on PANSS subscale scores; membrane FA levels in the three groups were then compared. More severe negative symptoms measured by PANSS were negatively correlated with two saturated FAs (myristic and margaric acids), one ω-9 monounsaturated FA (MUFA; nervonic acid), and one ω-3 polyunsaturated FA (PUFA; docosapentaenoic acid), and were positively correlated with two ω-9 MUFAs (eicosenoic and erucic acids) and two ω-6 PUFAs (γ-linolenic and docosadienoic acids). More severe positive symptoms measured by PANSS were correlated only with nervonic acid. No associations were observed between FAs and MADRS scores. In subjects with predominant negative symptoms, the sum of the ω-9 MUFAs and the ω-6:ω-3 FA ratio were both significantly higher than in those with predominant positive symptoms, whereas the sum of ω-3 PUFAs was significantly lower. In conclusion, abnormalities in FA metabolism may contribute to the neurobiology of psychopathology in UHR individuals. In particular, membrane FA alterations may play a role in negative symptoms, which are primary psychopathological manifestations of schizophrenia-related disability.
Topics: Adolescent; Adult; Austria; Erythrocyte Membrane; Erythrocytes; Fatty Acids; Fatty Acids, Omega-3; Female; Humans; Male; Psychopathology; Psychotic Disorders; Young Adult
PubMed: 26963912
DOI: 10.1371/journal.pone.0151417 -
Journal of Neurology, Neurosurgery, and... Jul 1997X linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal metabolism, biochemically characterised by accumulation of saturated very long chain fatty... (Review)
Review
X linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal metabolism, biochemically characterised by accumulation of saturated very long chain fatty acids. Accumulation of these fatty acids is associated with cerebral demyelination, peripheral nerve abnormalities, and adrenocortical and testicular insufficiency. The lowest estimated birth incidence is one per 100,000. At least six phenotypes can be distinguished, of which the two most frequent are childhood cerebral ALD and adrenomyeloneuropathy. The X-ALD gene has been identified, but thus far no relation between genotype and phenotype has been found. Diagnosis is relatively easy and can be confirmed reliably, and prenatal testing is possible in affected families. Several therapeutic options, some with promising perspectives, are available. Neurologists and other physicians seem not to be familiar with the many facets of X-ALD. In this review, the clinical presentation, the relative frequencies of the different phenotypes, and the diagnostic and therapeutic options are presented.
Topics: Adolescent; Adult; Age of Onset; Aged; Child; Dietary Fats, Unsaturated; Drug Combinations; Erucic Acids; Fatty Acids; Female; Genotype; Humans; Male; Microbodies; Peroxisomal Disorders; Phenotype; Prenatal Diagnosis; Triolein; X Chromosome
PubMed: 9221959
DOI: 10.1136/jnnp.63.1.4 -
The Journal of Biological Chemistry Feb 2022Although diabetes normally causes an elevation of cholesterol biosynthesis and induces hypercholesterolemia in animals and human, the mechanism linking diabetes to the...
Although diabetes normally causes an elevation of cholesterol biosynthesis and induces hypercholesterolemia in animals and human, the mechanism linking diabetes to the dysregulation of cholesterol biosynthesis in the liver is not fully understood. As liver peroxisomal β-oxidation is induced in the diabetic state and peroxisomal oxidation of fatty acids generates free acetate, we hypothesized that peroxisomal β-oxidation might play a role in liver cholesterol biosynthesis in diabetes. Here, we used erucic acid, a specific substrate for peroxisomal β-oxidation, and 10,12-tricosadiynoic acid, a specific inhibitor for peroxisomal β-oxidation, to specifically induce and suppress peroxisomal β-oxidation. Our results suggested that induction of peroxisomal β-oxidation increased liver cholesterol biosynthesis in streptozotocin-induced diabetic mice. We found that excessive oxidation of fatty acids by peroxisomes generated considerable free acetate in the liver, which was used as a precursor for cholesterol biosynthesis. In addition, we show that specific inhibition of peroxisomal β-oxidation decreased cholesterol biosynthesis by reducing acetate formation in the liver in diabetic mice, demonstrating a crosstalk between peroxisomal β-oxidation and cholesterol biosynthesis. Based on these results, we propose that induction of peroxisomal β-oxidation serves as a mechanism for a fatty acid-induced upregulation in cholesterol biosynthesis and also plays a role in diabetes-induced hypercholesterolemia.
Topics: Animals; Cholesterol; Diabetes Mellitus, Experimental; Fatty Acids; Hypercholesterolemia; Liver; Mice; Microbodies; Oxidation-Reduction; Peroxisomes
PubMed: 35007532
DOI: 10.1016/j.jbc.2022.101572 -
Molecules (Basel, Switzerland) Feb 2023Erucic acid (EA) is monounsaturated fatty acid (22:1 n-9), synthesized in the seeds of many plants from the family, with , , or considered as its richest source. As... (Review)
Review
Erucic acid (EA) is monounsaturated fatty acid (22:1 n-9), synthesized in the seeds of many plants from the family, with , , or considered as its richest source. As the compound has been blamed for the poisoning effect in Toxic Oil Syndrome, and some data indicated its cardiotoxicity to rats, EA has been for decades classified as toxic substance, the use of which should be avoided. However, the cardiac adverse effects of EA have not been confirmed in humans, and the experiments in animal models had many limitations. Thus, the aim of this review was to present the results of the so far published studies on both toxic, and pharmacological properties of EA, trying to answer the question on its future medicinal use. Despite the ambiguous and relatively small data on toxic and beneficial effects of EA it seems that the compound is worth investigating. Further research should be particularly directed at the verification EA toxicity, more in-depth studies on its neuroprotective and cytotoxic properties, but also its use in combination with other drugs, as well as its role as a drug carrier.
Topics: Humans; Rats; Animals; Erucic Acids; Fatty Acids, Monounsaturated; Brassica napus; Seeds
PubMed: 36838911
DOI: 10.3390/molecules28041924 -
Nutrients Jan 2023Depression represents one of the major causes of disability worldwide, with an important socioeconomic cost. Although many risk factors have been considered in its...
BACKGROUND
Depression represents one of the major causes of disability worldwide, with an important socioeconomic cost. Although many risk factors have been considered in its pathogenesis, nutrition seems to play a determinant role in its prevention. With regard to individual macronutrients, dietary fats and especially n-3 polyunsaturated fatty acids (n-3 PUFA) are the most studied. However, previous data about other dietary fatty acids, such as n-6 PUFA, are conflicting, and little is known about saturated fatty acids (SFA), especially when considering carbon chain length. Thus, we investigated whether single types and subtypes of dietary fats are related to depressive symptoms in Italian individuals living in the Mediterranean area.
METHODS
Dietary and socio-demographic data of 1572 individuals were analyzed. Food frequency questionnaires (FFQs) were used to determine the consumption of total dietary fat and each specific class of dietary fat, such as SFA, monounsaturated fatty acid (MUFA), and PUFA. The intake of fatty acids was also assessed according to the carbon-chain length of each single class. The Center for Epidemiologic Studies Depression Scale (CES-D) was used as a screening tool for depressive symptoms.
RESULTS
After adjustment for potential confounding factors, a significant inverse association between low/moderate levels of PUFA intake and depressive symptoms (Q2 vs. Q1, odds ratio (OR) = 0.60, 95% CI: 0.44, 0.84) was found. On the other hand, moderate saturated fat consumption was associated with depressive symptoms (Q3 vs. Q1, OR = 1.44, 95% CI: 1.02, 2.04). However, when considering carbon chain length, individuals with a lower to moderate intake of short-chain saturated fatty acids (SCSFA) and medium-chain saturated fatty acids (MCSFA) were less likely to have depressive symptoms (Q3 vs. Q1, OR = 0.48, 95% CI: 0.31, 0.75), while moderate intake of arachidic acid (C20:0) was directly associated with depressive symptoms (Q3 vs. Q1, OR = 1.87, 95% CI: 1.26, 2.77). Among single MUFAs, higher myristoleic acid (C14:1) intake was directly associated with depressive symptoms (Q4 vs. Q1, OR = 1.71, 95% CI: 1.12, 2.61), while moderate intake of erucic acid (C22:1) was associated with lower odds of having depressive symptoms (Q3 vs. Q1, OR = 0.54, 95% CI: 0.33, 0.86). When considering individual PUFAs, individuals with moderate and higher intakes of arachidonic acid (C20:4) were less likely to have depressive symptoms (OR = 0.64, 95% CI: 0.45, 0.91; OR = 0.59, 95% CI: 0.38, 0.91, respectively). Similarly, higher eicosapentaenoic acid (C20:5) intake was inversely associated with depressive symptoms (Q4 vs. Q1, OR = 0.35, 95% CI: 0.12, 0.98), while a significant association for docosahexaenoic acid (C22:6) was retrieved only for low intakes (Q2 vs. Q1, OR = 0.33, 95% CI: 0.12, 0.88).
CONCLUSIONS
Dietary fat intake may be associated with depressive symptoms, underlying the importance of distinguishing between different fat types. This study confirms the pivotal role of PUFAs and reopens the debate on the role of saturated fatty acids, suggesting plausible effects of moderate intakes of short-chain fatty acids.
Topics: Adult; Humans; Dietary Fats; Depression; Fatty Acids, Unsaturated; Fatty Acids; Diet; Fatty Acids, Volatile
PubMed: 36771380
DOI: 10.3390/nu15030675