• Nutritional Sciences
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• v.1 no.1
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• pp.56-60
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• 1998

The present study was conducted to find out whether there are some differences in plasma carnitine levels among young-, middle-, and old-aged normal Korean women. Daily food intake, body fat content, plasma lipids and carnitine levels were measured in 153 samples from 44 young (20-24 years old), 49 middle-aged (30-49 years old), and 63 old (65-85 years old) normal volunteers. The differences in concentrations of nonesterified acylcarnitine and acid-soluble acylcarnitine were not statistically significant among them. However, acid insoluble acylcarnitine (AIAC) level in plasma decreased with age. Moreover, total carnitine (TCNE) level in the young group was significantally higher than in old and middle-aged groups. Body fat content in the young group was significantly lower than in old and middle-aged groups. Plasma total cholesterol increased with age and triglycerides in the old group were significantly higher than in young and middle-aged groups. These results suggest that the higher levels of AIAC and TCNE in the young group may be a reflection of their lipid metabolic state, which is different from middle-aged and old groups.

• Journal of Nutrition and Health
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• v.30 no.9
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• pp.1067-1072
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• 1997

One known effect of long chain n-3 polyunsaturated fatty acids is their ability to decrease plasma triglycerides. However, identification of the specific n-3 fatty acids and the underlying mechanisms responsible for this change remains uncertain. This present study was designed to evaluate the effects of moderate levels of dietary docosahexaenoic acid (22 :6(n-3)) on modulating plasma triglyderides. Male CD-1 mice were maintained for 15 days on identical diets containing either docosahexahexaenoic acid ethyl ester(1.5%, w/w) or linoleic acid(18 : 2(n-6)) ethyl ester (1.5%, w/w) . Plasma triglycerides were 40% lower in the docosahexaenoic acid group than in the linoleic acid group. Hepatic carnitine palmitoyltransferase activity (a key regulatory enzyme for mitocondria $\beta$-oxidation) was not significantly different between the dietary groups. However, plasma acid soluble acylcarnitine levels (which increase with increasing $\beta$-oxidation )were significantly higher in the decosahexaenoic acid group. This data suggests that plasma triglyceride levels are lower in mice fed diets containing moderate levels of docosahexaenoic acid compared to linoleic acid, but this effect on plasma triglycerides is not modulated through an augmentation of mitochondrial $\beta$-oxidation.

• Nutritional Sciences
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• v.8 no.2
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• pp.97-103
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• 2005

The goal of this study was to investigate abnotmalities in camitine metabolism present by determining blood camitine and lipid concentrations in Korean diabetic patients. The study subjects included 108 Korean diabetic patients (64 males and 44 females) who were hospitalized in Chonbuk National University Hospital and 27 subjects were also hospitalized as non-diabetic controls (10 males and 17 females). Glucose, total cholesterol, triglyceride (TG) and HDL-cholesterol in plasma were enzymatically assayed and insulin was measured by immunoradiometric assay. Nonesterified camitine (NEC), acid-soluble acylcarnitine (ASAC), and acid-insoluble acylcarnitine (AIAC) were determined by a modified radioisotopic method Glucose and insulin levels were significantly elevated in diabetic patients compared with controls. Total cholesterol was elevated in female but not male diabetic patients and triglycerides were elevated both in male and female diabetics. Plasma and urinary total carnitine (TCNE) were significantly elevated in diabetics as compared with normal controls. In male diabetics, NEC concentrations were significantly elevated above controls, but not in female subjects. Plasma NEC and TCNE concentrations were significantly increased in male diabetics, but significantly decreased in female diabetics. All urinary carnitine concentrations were significantly increased in diabetics as compared with controls. Urinary NEC concentrations were four times higher in male diabetics and three times higher in female diabetics than in controls. The ratios of serum and urinary acylcarnitine/NEC were also significantly higher in diabetics than in controls. This study suggested that there was a remarkable abnormality in lipid and carnitine metabolism in Korean diabetic patients, and the further study on carnitine metabolism and the effects of carnitine supplementation for Korean diabetic patients are needed.

• Journal of Community Nutrition
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• v.4 no.2
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• pp.78-82
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• 2002

This study compared the effect of Korean vegetarian and omnivorous diets on plasma carnitine concentrations and urinary carnitine excretion. Twenty lactoovovegetarian and twenty omnivorous female college students consented to participate in this study. Daily nutritional intake and plasma and urinary nonesterified carnitine (NEC), acid-soluble acylcarnitine (ASAC), acid-insoluble acylcarnitine (AIAC), and total carnitine (TCNE) were determined. Daily protein, fat, retinol, vitamin B$_2$and vitamin B$\_$12/ intakes were significantly lower for vegetarians, however, fiber, carbohydrate, $\beta$-carotene, folic acid and vitamin C consumptions were much higher for vegetarians than omnivores. There were no differences in plasma NEC, ASAC, AIAC and TCNE concentrations between the two groups. Urinary carnitine excretion was lower in vegetarians, but only the differences in ASAC and TCNE excretions were statistically significant. These results suggest that the lower excretion of ASAC in vegetarians may be a reflection of their lipid metabolic state and that Korean vegetarian diets may accommodate lower carnitine intakes through efficient urinary conservation of carnitine.

• Biomolecules & Therapeutics
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• v.17 no.2
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• pp.181-187
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• 2009

Carnitine is known to be involved in lipid metabolism and affects body composition as well as energy metabolism of the whole body. Improvement of obesity by L-carnitine supplement suggests that obesity can be related with the abnormality of carnitine metabolism and therefore, plasma carnitine level in normal and obesity groups was investigated. For the characterization of plasma carnitine level in obese people, 60 plasma samples collected from Korean women subjects were analyzed using LC/MS and plasma fatty acid level was also determined using GC/MS. Additionally, several clinical chemical parameters including fasting glucose, cholesterol, AST, and ALT level were measured. All the data obtained were combined and pattern recognition analysis was carried out with the dataset. Obese group showed a different metabolic pattern compared with normal group. Plasma acylcarnitine level of the obese group was found to be $11.7{\mu}g/ml$, which was higher than that of normal group ($8.0{\mu}g/ml$). Statistically significant differences in plasma fatty acid level were not observed between the two groups. Other clinical parameters for the obese group were within normal ranges but AST and ALT levels were slightly elevated compared to normal group. The obese group showed elevated plasma acylcarnitine level.

• Journal of the Korean Society of Food Science and Nutrition
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• v.28 no.3
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• pp.670-676
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• 1999

The effect of exercise and/or high fat diet on carnitine status and carnitine palmitoyltransferase I(CPT I) level were investigated in Weanling Sprague Dawley rats. The rats were fed an AIN 76 diet or a modified high fat AIN diet, supplemented with 35% corn oil, for 31 days. During the 31 day period half of the animals in each dietary group were exercised on a treadmill for 90 minutes per day. Carnitine concentrations were determined in plasma and liver and CPT I mRNA levels were measured by Northern blot analysis with CPT I cDNA probe in livers of rats. Exercise rats gained less weight than non exercised rats during the study for high fat diet group. Exercise rats had a higher plasma acid soluble acylcarnitine and acid insoluble acylcarnitine concnetrations than non exercised rats for normal diet group. Exercise or high fat diet increased liver carnitine concentration, but a mixed effect was not shown. In exercised rats, CPT I mRNA levels increased significantly relative to those of nonexercised rats. CPT I mRNA levels also increased when compared high fat fed rats with those of normal diet fed rats. These data suggest that there is a correlation between carnitine concen trations and CPT I mRNA levels and that CPT I can be regulated at the transcriptional level by exercise and/or high fat diet.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.1
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• pp.1-8
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• 2022

Long-chain fatty acid oxidation disorders (LC-FAOD) are an autosomal recessive inherited rare disease group that result in an acute metabolic crisis and chronic energy deficiency owing to the deficiency in an enzyme that converts long-chain fatty acids into energy. LC-FAOD includes carnitine palmitoyltransferase type 1 (CPT1), carnitine-acylcarnitine translocase (CACT), carnitine palmitoyltransferase type 2 (CPT2), very long-chain acyl-CoA dehydrogenase (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and trifunctional protein (TFP) deficiencies. Common symptoms of LC-FAOD are hypoketotic hypoglycemia, cardiomyopathy, and myopathy. Depending on symptom onset, the disease can be divided as neonatal period, late infancy and early childhood, adolescence, or adult onset, but symptoms can appear at any time. The neonatal screening test (NBS) can be used to identify the characteristic plasma acylcarnitine profiles for each disease and confirmed by deficient enzyme analysis or molecular testing. Before introduction of NBS, the mortality rate of LC-FAOD was very high. With NBS implementation as routine neonatal care, the mortality rate was dramatically decreased, but severe symptoms such as rhabdomyolysis recur frequently and affect the quality of life. Triheptanoin (Dojolvi^®), the first drug for pediatric and adult patients with molecularly confirmed LC-FAOD, has recently been approved by the US Food and Drug Administration in 2020. In this review, the diagnosis of LC-FAOD and treatment including triheptanoin are summarized.

• Journal of Nutrition and Health
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• v.28 no.5
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• pp.397-405
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• 1995

The present study examined the effects of swimming on some plasma parameters of lipid metabolism in the middle-aged women. The control group(C) was the women who lived without any intentional physical exercise, and the first experimental group(S I) and the second experimental group(S II) had gone swimming everyday at least one hour for 3-6 months, and more than one year, respectively. The results obtained were summarized as follows: 1) Energy and nutrient intakes of the three groups were not different, but the body fat(%) of two experimental groups(S I, S II) was significantly lower than that of control. 2) Total cholesterol and HDL-cholesterol in plasma were not significantly changed by swimming and swimming period. However, total lipids and LDL-cholesterol were significantly lower in the two experimental groups than in control. 3) The level of plasma triglycerides of SI was not different from the control, but SII showed significantly lower triglycerides. 4) Acid-soluble acylcarnitine and $\beta$-hydroxybutyrate concentration in plasma were significantly greater in SII than in control. There results suggest that regular swimming in a longer period might help to protect the risk of obesity and cardiovascular disease by modulating lipid metabolism.

• Journal of Nutrition and Health
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• v.36 no.7
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• pp.720-728
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• 2003

This study was conducted to evaluate changes in plasma concentration and urinary excretion of carnitine, as well as plasma lipid level and fatty acid composition, caused by short term supplementation of carnitine in humans. Ten healthy male subjects (21.2 $\pm$ 0.5 years old) received oral carnitine supplementation (4 g/day) as tablets for two weeks. Fasting blood and random urine samples were collected from each subject both prior to and at the end of carnitine supplemention program. Following the 2 weeks of carnitine supplementation, plasma total carnitine (TCNE) concentration increased 20% (85.1 $\pm$ 7.4 vs 67.3 $\pm$ 9.1 $\mu$ mol/1, p> 0.05), while urinary excretion of total carnitine increased ten times compared to the value measured prior to the supplementation (3051 $\pm$ 692 vs 278 $\pm$ 90.1 $\mu$ mol/g creatinine, p < 0.01). Non-esterified carnitine (NEC) comprised from 71 to 88% of TCNE in plasma, and from 32 to 40% of TCNE excreted in the urine. Two weeks of carnitine supplementation in healthy adults significantly elevated plasma level of acid soluble acylcarnitine (ASAC) which is esterified mostly with short chain fatty acids (21.6 $\pm$ 1.6 $\mu$ mol/l) compared to the value measured prior to the supplementation (6.4 $\pm$ 0.8 $\mu$ mol/l) (p < 0.05). Carnitine supplementation significantly increased plasma HDL-cholesterol level (p < 0.05), and decreased the atherogenic index (p < 0.05), but failed to cause any significant change in plasma levels of total cholesterol, triglyceride, and free fatty acids. Plasma triglyceride and phospholipid fatty acid compositions were not significaly affected as well by the oral supplementation of carnitine in subjects with normal range of blood lipid levels.

• 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.