• Animal Bioscience
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• v.36 no.9
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• pp.1414-1425
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• 2023

Objective: Long chain n-3 polyunsaturated fatty acids (PUFA) exert positive effects on human health. The long chain n-3 PUFA of pork can be increased by adding fish oil to the diet. Due to the cost and availability of fish oil an alternative source must be found. Methods: This study evaluated the effect of five dietary oils on meat quality, fatty acid composition and lipid stability. The five diets contained 1% palm oil (Control), 1% soya oil, 1% linseed oil, 1% fish oil, and 1% Echium oil, respectively. The trial consisted of 60 gilts, randomly allocated to five groups. Results: All color parameters, extractable fat content, fat free dry matter, and moisture content of the m. longissimus muscle were unaffected by dietary treatment. Consumers and a trained sensory panel could not detect a difference between the control samples and the Echium oil sample during sensory analysis. Samples containing higher levels of PUFA (soya, linseed, fish, and Echium oil) had higher levels of primary and secondary lipid oxidation products after refrigerated and frozen storage. However, these values were still well below the threshold value where off flavors can be detected. The Echium oil treatment had significantly higher levels of long chain PUFA than the linseed oil treatment, but it was still significantly lower than that of the fish oil treatment. Conclusion: Echium oil supplementation did not increase the levels of n-3 to the same extent as fish oil did. The result did however suggest that Echium oil can be used in pig diets to improve muscle long chain n-3 fatty acid content without any adverse effects on meat quality when compared to linseed, soya, and palm oil.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1083-1089
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• 2002

Purpose : The prevalence of obese children has recently increased. Obesity is known to be associated with complications such as hypertension, fatty liver, hyperlipidemia, and insulin resistance. L-carnitine is an essential cofactor for the transport of long chain fatty acids into mitochondria for ${\beta}$-oxidation. The purpose of this study is to measure serum free fatty acid and carnitine levels, and evaluate the role of L-carnitine as a therapeutic drug in obese children with fatty liver. Methods : Nine obese children, ranging from seven to 18 years of age, and 10 normal children were examined. Serum lipid(total cholesterol, triglyceride, HDL-cholesterol, and LDL-cholesterol) and fatty acid levels were analyzed. Serum total, free, and acyl carnitine levels were performed also by a new enzymatic cycling technique. Results : Long chain fatty acids(myristic acid, palmitoleic acid, palmitic acid, linoleic acid, oleic acid, and stearic acid)were significantly increased in obese children compared to the control group. Total, and acyl carnitine levels were significantly increased in obese children compared to the control group. Conclusion : Serum free fatty acid and carnitine levels were significantly increased in obese children with fatty liver compared to the normal control. This may suggest that L-carnitine can be used as antilipidemic agent to decrease fatty acid and lipid levels for obese children. Prospective studies will investigate serum fatty acid and carnitine levels after treatment of L-carnitine in obese children in the future.

• Transactions of the Korean Society of Automotive Engineers
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• v.15 no.6
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• pp.81-86
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• 2007

Biodiesel fuel that consists of saturated and unsaturated long-chain fatty acid alkyl esters is an alternative diesel fuel produced from vegetable oils or animal fats. However, air causes autoxidation of biodiesel fuel during storage, which can reduce fuel quality by adversely affecting its properties, such as the kinematic viscosity and acid value. One approach for improving the resistance of fatty derivatives to autoxidation is to mix them with antioxidants. This study investigated the effectiveness of five such antioxidants in mixtures with biodiesel fuels produced by three biodiesel manufacturers : tert-butylhydroquinone (TBHQ), butylated hydroxyanisole (BHA), butylated hydroxytoluene (BHT), propyl gallate (PrG) and $\alpha$-tocopherol. Oxidation stability was determined using Rancimat equipment. The results show that TBHQ, BHA, and BHT were the most effective and $\alpha$-tocopherol was the least effective at increasing the oxidation stability of biodiesel. This study recommends that TBHQ and PrG be used for safeguarding biodiesel fuel from the effects of autoxidation during storage.

• Molecules and Cells
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• v.44 no.9
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• pp.637-646
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• 2021

Free fatty acids are converted to acyl-CoA by long-chain acyl-CoA synthetases (ACSLs) before entering into metabolic pathways for lipid biosynthesis or degradation. ACSL family members have highly conserved amino acid sequences except for their N-terminal regions. Several reports have shown that ACSL1, among the ACSLs, is located in mitochondria and mainly leads fatty acids to the β-oxidation pathway in various cell types. In this study, we investigated how ACSL1 was localized in mitochondria and whether ACSL1 overexpression affected fatty acid oxidation (FAO) rates in C2C12 myotubes. We generated an ACSL1 mutant in which the N-terminal 100 amino acids were deleted and compared its localization and function with those of the ACSL1 wild type. We found that ACSL1 adjoined the outer membrane of mitochondria through interaction of its N-terminal region with carnitine palmitoyltransferase-1b (CPT1b) in C2C12 myotubes. In addition, overexpressed ACSL1, but not the ACSL1 mutant, increased FAO, and ameliorated palmitate-induced insulin resistance in C2C12 myotubes. These results suggested that targeting of ACSL1 to mitochondria is essential in increasing FAO in myotubes, which can reduce insulin resistance in obesity and related metabolic disorders.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.19 no.1
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• pp.20-25
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• 2019

Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessively inherited metabolic disorder of mitochondrial long-chain fatty acid oxidation. The clinical features of VLCAD deficiency is classified by three clinical forms according to the severity. Here, we report a case of later-onset episodic myopathic form of VLCAD deficiency whose diagnosis was confirmed by plasma acylcarnitine analysis and" multigene panel multigene panel sequencing. A 34-year old female patient visited genetics clinic for genetic evaluation for history of recurrent myopathy with intermittent rhabdomyolysis. She suffered first episode of rhabdomyolysis with acute renal failure requiring hemodialysis at twelve years old. After then, she suffered several times of recurrent rhabdomyolysis provoked by prolonged exercise or fasting. Physical and neurologic exam was normal. Serum AST/ALT and creatinine kinase (CK) levels were mildly elevated. However, according to her previous medical records, her AST/ALT, CK were highly elevated when she had rhabdomyolysis. In suspicion of fatty acid oxidation disorder, multigene panel sequencing and plasma acylcarnitine analysis were performed in non-fasting, asymptomatic condition for the differential diagnosis. Plasma acylcarnitine analysis revealed elevated levels of C14:1 ($1.453{\mu}mol/L$; reference, 0.044-0.285), and C14:2 ($0.323{\mu}mol/L$; 0.032-0.301) and upper normal level of C14 ($0.841{\mu}mol/L$; 0.065 -0.920). Two heterozygous mutation in ACADVL were detected by multigene panel sequencing and confirmed by Sanger sequencing: c.[1202G>A(;) 1349G>A] (p.[(Ser 401Asn)(;)(Arg450His)]). Diagnosis of VLCAD deficiency was confirmed and frequent meal with low-fat diet was educated for preventing acute metabolic derangement. Fatty acid oxidation disorders have diagnostic challenges due to their intermittent clinical and laboratorial presentations, especially in milder late-onset forms. We suggest that multigene panel sequencing could be a useful diagnostic tool for the genetically and clinically heterogeneous fatty acid oxidation disorders.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.1
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• pp.21-27
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• 2022

Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency (VLCADD) leads to a defective 𝛽-oxidation, specifically during prolonged fasting, infection, or exercise. Patients with VLCADD usually suffer from cardiomyopathy, hypoketotic hypoglycemia, hepatic dysfunction, exercise intolerance, muscle pain, and rhabdomyolysis, and sometimes succumb to sudden death. VLCADD is generally classified into three phenotypes: severe early-onset cardiac and multiorgan failure, hypoketotic hypoglycemia, and later-onset episodic myopathy. Diagnostic evaluation comprises acylcarnitine analysis, genetic analysis, and VLCAD activity assay. In the acylcarnitine analysis, the key metabolites are C14:1, C14:2, C14, and C12:1. A C14:1 level >1 mmol/L strongly suggests VLCADD. Various treatment recommendations are available for this condition. Dietary management includes decreasing fat content, increasing medium-chain triglyceride levels, and decreasing fasting periods. Supplementation with L-carnitine is controversial. Triheptanoin (a seven-carbon fatty acid triglyceride) treatment demonstrates improvement of cardiac functions. Bezafibrate may improve the quality of life of patients with VLCAD.

• Toxicological Research
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• v.14 no.3
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• pp.285-291
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• 1998

This study was designed to examine the effects of polyundaturated fatty acid(PUFA) from different sourecs on hepatic lipogenic enzyme and peroxisomal ${\beta}$-oxidation in murine hepatocarcinogenesis initiated by diethylnitrodamine (DEN). Male Sprague-Dawley rats were fed one of three diets containing 10%(w/w)fat; fish oil-corn oil blended(FO), corn oil-beef tallow-fish oil blended(CF), or corn oil-beef tallow-perilla oil blended (CP), from the gestation period. At 10 weeks, animals were received a single inraperitoneal injection of DEN (200mg/kg body weight), were subjected to two-thirds partial hepatectomy 3 weeks later and were sacrificed 8 weeks after DEN initiation. The areas of placental glutathione S-transferase (GST-P) positive foci were significantly smaller in rats fed fish oil containing diets (FO and CF) than those fed CP diet. Fish oil feeding significantly decreased th activities of lipogenic enzyme. Rats fed fish oil containing diets (FO, CF) exhibited the lower fatty acid synthase (FAS) activity than those fed CP diet and FAS activity was positively correlated with areas of GSP-P positivie foci. Glucose-6-phophate dehydrogenase activity was the lowest and peroxisomal ${\beta}$-oxidation was stimulated in rats fed FO diet compared to other groups. It was also found that serum cholesterol was decreased in FO group. Therefore, the preventive effect against hepatocarcinogenesis and hypolipidemic effect of fish oil can be explained partly by suppression of the hepatic lipogenesis and by increase of peroxisomal ${\beta}$-oxidation.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1075-1082
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• 2002

Purpose : Obesity is known to be associated with hypertension, dyslipidemia, and fatty liver and is thought to be associated with increased levels of free fatty acids. One of the strategies for decreasing free fatty acid levels is stimulation of hepatic lipid oxidation with L-carnitine. Carnitine is an essential cofactor for transport of long-chain fatty acid into mitochondria for oxidation. This study was designed to evaluate the changes of serum fatty acids and carnitine levels after exogenous injection of L-carnitine. Methods : Sprague Dawley rats were divided into two groups. Group A was control. Group B was given intraperitoneal injection with L-carnitine(200 mg/kg) daily for two weeks. Serum lipid (total cholesterol, triglyceride, HDL-cholesterol, LDL-cholesterol) and fatty acid levels were analyzed on the first day of the first and second weeks after injection of L-carnitine. Total, free, and acyl carnitine levels also were performed by a enzymatic cycling techniques at the same day intervals. Results : There was no significant difference between the two groups in total cholesterol, HDL-cholesterol, LDL-cholesterol levels before and after the administration of L-carnitine. But triglyceride levels were significantly decreased at the first week in group B compared with group A. Among free fatty acids, linoleic acid showed significant decrement(A group : $131.3{\pm}31.3mg/dL$ vs B group : $90.0{\pm}7.0mg/dL$) at the first week. Total, free, and acyl carnitine levels showed significant increments at all days intervals, but only free carnitine showed significant increments according to cumulative doses of carnitine. Conclusion : Plasma linoleic acid, a long-chain fatty acid, showed significant decrement after administration of L-carnitine in the first week. This may suggest that L-carnitine can be used as an antilipidemic agent for obese patients. A prospective study will investigate obese children in the future.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.49-53
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• 2012

X-linked adrenoleukodystrophy (ALD) is a rare inherited metabolic disease which results in impaired peroxisomal ${\beta}$-oxidation and the accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex, the myelin of the central nervous system, and the testes. X-linked ALD is caused by mutations in the ABCD1 gene encoding an ATP-binding cassette transporter superfamily located in the peroxisomal membrane. This disease is characterized by a variety of phenotypes. The classic childhood cerebral ALD is a rapidly progressive demyelinating condition affecting the cerebral white matter before the age of 10 years in boys. We report the case of a 8-year-old with childhood cerebral X-linked ALD who developed inattention, hyperactivity, motor incoordination and hemiparesis. We diagnosed ALD with elevated plasma very long chain fatty acid level and diffuse high signal intensity lesions in both parieto-occipital white matter and cerebellar white matter in brain MRI. We identified a novel c.983delT (p.Met329CysfsX7) mutation of the ABCD1 gene. There is no correlation between X-ALD phenotype and mutations in the ABCD1 gene. Further studies for searching additional non-genetic factor which determine the phenotypic variation will be needed.

• Food Science and Biotechnology
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• v.18 no.1
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• pp.79-83
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• 2009

Acidolysis of olive oil with omega-3 (n-3) polyunsaturated fatty acids (PUFAs) was carried out to produce a structured lipid. Novozym $435^{(R)}$ from Candida antarctica was used as the biocatalyst. Response surface methodology (RSM) was used to determine optimum conditions for lipase-catalyzed enrichment of olive oil. Three factors, 5 levels, central composite design was used. The effects of incubation time, temperature, and substrate mole ratio on incorporation ratio (n-3 fatty acids/total fatty acids, %) were investigated. From the evaluation of response surface graphs, the optimal conditions for incorporation of long chain n-3 PUFAs into olive oil were $40-60^{\circ}C$ for temperature, 30-45 hr for reaction time, and 3:1-5:1 (n-3 fatty acids/olive oil) for substrate mole ratio. Experiments conducted under optimized conditions predicted by the model equation obtained from RSM yielded structured lipids with 50.8% n-3 PUFAs. This value agreed well with that predicted by the model. Oxidative stability tests showed that the product was more susceptible to oxidation than unmodified olive oil. Antioxidant addition improved the oxidative stability of the product.