• Title/Summary/Keyword: Glucose metabolism

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The Clinical and Genetic Characteristics of Three Korean Patients with Glycogen Storage Disease Type V (McArdle Disease) (세 명의 대한민국 제 V형 당원축적근육병(McArdle 병) 환자들의 유전학적 및 임상적 특성 보고)

  • Lee, Sunghee;Kang, Eungu;Kim, Yoonmyung;Lee, Beom Hee;Kim, Gu Hwan;Yoo, Han Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.2
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    • pp.93-101
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    • 2016
  • Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

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Effects of Fermentation on the Metabolic Activities of Pine Needle Juice (발효과정이 솔잎 착즙액의 항산화, alpha-Glucosidase 및 Angiotensin Converting Enzyme 저해 활성에 미치는 영향)

  • Kim, So-Yun;Lee, Hyun-Jung;Park, Jae-Hee;Kim, Rae-Young;Cheong, Hyeonsook;Park, Eunju
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.42 no.3
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    • pp.325-334
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    • 2013
  • The objective of this study was to compare the content and metabolic activities between fresh pine needle juice (PNJ) and fermented pine needle juice (FPNJ). A variety of factors were measured, including total phenolic content (TPC), antioxidant activity [DPPH radical scavenging activity (RSA), total radical-trapping antioxidant potential (TRAP), oxygen radical absorbance capacity (ORAC), cellular antioxidant capacity (CAC)], anti-genotoxic activity, ${\alpha}$-glucosidase inhibitory activity, and angiotensin converting enzyme (ACE) inhibitory activity. The TPC was $17.3{\pm}0.2$ and $4.6{\pm}0.0$ mg GAE/g in PNJ and FPNJ, respectively. The DPPH RSA, TRAP, and ORAC values increased in a dose-dependent manner for both PNJ and FPNJ, with significantly higher activities in PNJ than FPNJ. The CAC against AAPH-induced oxidative stress in HepG2 cells was protected by both PNJ and FPNJ. Pretreatment with PNJ and FPNJ in human leukocytes produced significant reductions in $H_2O_2$-induced DNA damage at a concentration of $50{\mu}g/mL$. ${\alpha}$-Glucosidase inhibitory activity was significantly higher in FPNJ than PNJ. The ACE inhibitory activity was about 87.1% and 60.0% in 1:1 diluted PNJ and FPNJ, respectively. This study suggests that the fermentation of PNJ could enhance the regulation of blood glucose metabolism and both PNJ and FPNJ might be a new potential source of natural antioxidant, anti-diabetic, and anti-hypertensive agents applicable to food.

Hypolipidemic and Hypoinsulinemic Effects of Dietary Fiber from Agar in C57BL/6N Mice Fed a High-fat Diet (고지방사료를 섭취한 C57BL/6N 마우스에서 한천식이섬유의 혈청 지질과 인슐린 농도 저해효과)

  • Park, Jin Ju;Kim, Ji Eun;Yun, Woo Bin;Lee, Mi Lim;Choi, Jun Young;Song, Bo Ram;Kim, Dong Seob;Lee, Chung Yeoul;Lee, Hee Seob;Lim, Yong;Jung, Min Wook;Hwan, Dae Youn
    • Journal of Life Science
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    • v.27 no.8
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    • pp.937-944
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    • 2017
  • To investigate the beneficial effects of fiber derived from agar on the lipid and glucose metabolism of obese model mice, changes in the fat accumulation, lipid content, and insulin concentration were measured in C57BL/6N mice fed a high-fat diet (HFD) and treated with a beverage containing agar (BCA) for 9 weeks. The feed efficiency ratio was significantly decreased in the HFD+BCA treatment group, although the body weight and food intake were maintained a constant level. Also, the fat accumulation in the fatty tissue and liver were lower in the HFD+BCA treatment group than in the HFD+Vehicle treatment group. Furthermore, a significant decrease was detected in the levels of total cholesterol (TC) and LDL after the BCA treatment, while a constant HDL level was maintained in the same group. Moreover, the serum insulin concentration was significantly decreased in the HFD+BCALo (Low concentration) and HFD+BCAMi (Middle concentration) groups compared to the HFD+Vehicle group. Therefore, the results suggest that the long-term administration of dietary fiber from agar can improve fat accumulation in the fatty tissue and liver, the serum lipid profile, and the insulin concentration in the blood. Also, the results provide evidence that the inclusion of agar fiber in beverages results in significant hypolipidemic and hypoinsulinemic effects.

Effects of the Exercise Training on Aging Heart in Rat I. Long Term Endurance Exercise (운동훈련이 흰쥐 노화심근에 미치는 영향 I. 장기간 지구력 운동 훈련)

  • 박원학;이상선;이용덕
    • Biomedical Science Letters
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    • v.2 no.1
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    • pp.71-90
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    • 1996
  • There is considerable current interest in the effect of regular vigorous exercise and in particular endurance-running as a possible measure in improving myocardial function. Some data indicate that the aging heart may actually suffer from vigorous endurance exercise. On the contrary appropriate exercise in aged animals improves myocardial function and aerobic energy metabolism. So far there is relatively little data to indicate that endurance exercise is in fact beneficial in improving myocardial function or damaging to heart of aged animals. The present investigation aimed to study the possible effect of a long range treadmill training program on the heart in aging rats. Male rats aged 3, 10, and 20 months were divided at random into a control (sedentary) and an exercise group. The training group was exercised for 5 days a week on an automated treadmill for 20minutes at 18m/min over a period of 5 months. The exercise regimen of our experiments did not cause any significant changes in the tissues and ultrastructural as com-pared with sedentary age-matched control. Tissues and ultrastructures of myocardial cells in trained group aged 8 months are intact and well organized as well as sedentary control group. Age associated tissue and ultrastructural changes of trained group aged 15 months included : an increase in transformed mitochondria, vacuoles, lysosomes, lipid droplets and early lipofuscin. But the trained heart did not show significant difference in tissue and ultrastructural properties from those of sedentary controls. Endurance-trained group aged 25 months showed significant qualitative tissue and ultrastructural difference as compared with age-matched controls. In addition to those found in 25 months control group, focal necrosis, myofibril fraying, hypercontraction band, seperation of intercalated discs, degenerating nucleus and infiltration of collagenous fiber into myocyte were noted in trained 25 months group. The stereological examination of the mi-crographs disclosed no significant difference in the myoflbril, mitochondrion, sarcotubule and in-terstitium volume density and surface density of mitochondrial cristae and numerical density of mitochondria between trained and control group aged 8 and 15 months. In the trained 25 months group, significant increase in volume density of interstitium, lipofucsin granule were shown as compared to untrained age-matched control. On the other hand, significant decrease in mitochondrion volume density was shown. The myofibril volume density did not differ between trained and control group although trained group showed slight increase. From the data obtained a reduced mitochondria/myofibrils ratio was found in trained rat heart aged 25 months and there was no difference between trained and control rat aged 15 months. But a slight but not significant increase was found in the trained group aged 8 months as compared with same age control group. Such increase in the ratio in young animals is considered to be of great importance to cardiac pumping and adaptability. Whereas such adaptations don't seem to occur in aged heart muscle. This study proposed that repeated endurance exercise do not cause any significant qualitative and quantitative ultrastructural change of heart muscle in young(3months) and adult (10months) suggesting that the heart is able to adapt to the exercise. On the contrary, the repeated endurance exercise stress may actually induce degenerative changes in the aged heart muscle(20months).

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Imaging Neuroreceptors in the Living Human Brain

  • Wagner Jr Henry N.;Dannals Robert F.;Frost J. James;Wong Dean F.;Ravert Hayden T.;Wilson Alan A.;Links Jonathan M.;Burns H. Donald;Kuhar Michael J.;Snyder Solomon H.
    • The Korean Journal of Nuclear Medicine
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    • v.18 no.2
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    • pp.17-23
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    • 1984
  • For nearly a century it has been known that chemical activity accompanies mental activity, but only recently has it been possible to begin to examine its exact nature. Positron-emitting radioactive tracers have made it possible to study the chemistry of the human mind in health and disease, using chiefly cyclotron-produced radionuclides, carbon-11, fluorine-18 and oxygen-15. It is now well established that measurable increases in regional cerebral blood flow, glucose and oxygen metabolism accompany the mental functions of perception, cognition, emotion and motion. On May 25, 1983 the first imaging of a neuroreceptor in the human brain was accomplished with carbon-11 methyl spiperone, a ligand that binds preferentially to dopamine-2 receptors, 80% of which are located in the caudate nucleus and putamen. Quantitative imaging of serotonin-2, opiate, benzodiazapine and muscarinic cholinergic receptors has subsequently been accomplished. In studies of normal men and women, it has been found that dopamine and serotonin receptor activity decreases dramatically with age, such a decrease being more pronounced in men than in women and greater in the case of dopamine receptors than serotonin-2 receptors. Preliminary studies in patients with neuropsychiatric disorders suggests that dopamine-2 receptor activity is diminished in the caudate nucleus of patients with Huntington's disease. Positron tomography permits quantitative assay of picomolar quantities of neuro-receptors within the living human brain. Studies of patients with Parkinson's disease, Alzheimer's disease, depression, anxiety, schizophrenia, acute and chronic pain states and drug addiction are now in progress. The growth of any scientific field is based on a paradigm or set of ideas that the community of scientists accepts. The unifying principle of nuclear medicine is the tracer principle applied to the study of human disease. Nineteen hundred and sixty-three was a landmark year in which technetium-99m and the Anger camera combined to move the field from its latent stage into a second stage characterized by exponential growth within the framework of the paradigm. The third stage, characterized by gradually declining growth, began in 1973. Faced with competing advances, such as computed tomography and ultrasonography, proponents and participants in the field of nuclear medicine began to search for greener pastures or to pursue narrow sub-specialties. Research became characterized by refinements of existing techniques. In 1983 nuclear medicine experienced what could be a profound change. A new paradigm was born when it was demonstrated that, despite their extremely low chemical concentrations, in the picomolar range, it was possible to image and quantify the distribution of receptors in the human body. Thus, nuclear medicine was able to move beyond physiology into biochemistry and pharmacology. Fundamental to the science of pharmacology is the concept that many drugs and endogenous substances, such as neurotransmitters, react with specific macromolecules that mediate their pharmacologic actions. Such receptors are usually identified in the study of excised tissues, cells or cell membranes, or in autoradiographic studies in animals. The first imaging and quantification of a neuroreceptor in a living human being was performed on May 25, 1983 and reported in the September 23, 1983 issue of SCIENCE. The study involved the development and use of carbon-11 N-methyl spiperone (NMSP), a drug with a high affinity for dopamine receptors. Since then, studies of dopamine and serotonin receptors have been carried out in over 100 normal persons or patients with various neuropsychiatric disorders. Exactly one year later, the first imaging of opitate receptors in a living human being was performed [1].

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Gene Expression of Candidate Genes Involved in Fat Metabolism During In vitro Adipogenic Differentiation of Bovine Mesenchymal Stem Cell (Bovine Mesenchymal Stem Cell의 지방분화를 이용한 지방대사관련 후보 유전자의 발현분석)

  • Kim, Sung-Kon;Kim, Nam-Kuk;Yoon, Du-Hak;Kim, Tae-Hun;Yang, Boo-Keun;Lee, Hyun-Jeong
    • Journal of Animal Science and Technology
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    • v.52 no.4
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    • pp.265-270
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    • 2010
  • Adipogenesis has been one of the most intensely studied models of cellular differentiation. During adipogenesis, differential expression of many adipogenesis related genes lead to profound changes in cellular, morphological, and physiological characteristics of the differentiating cells. The aim of the present study was to examine the expression levels of adipogenic candidate genes, cAMP early repressor (ICER), nephroblastoma over-expressed protein (NOV), heat shock protein beta 1 (HSPB1) and succinate dehydrogenase (SDH), during adipogenesis of bovine mesenchymal stem cells (BMSC). The BMSC were cultured in DMEM / low glucose medium with adipogenic inducers for 6 days and the expression of various candidate genes which seemed related to adipogenesis were measured by real-time PCR. This study showed that the expression of peroxisome proliferator activated receptor ${\gamma}$(PPAR${\gamma}$) and fatty acid binding protein 4 (FABP4) genes as adipogenic indicators were increased to 3.11 and 3.11 folds on day 6 than on day 0, respectively (p<0.05). To determine whether candidate genes were related to adipogenesis, the expression levels of ICER, NOV, HSPB1, and SDH genes were measured during adipogenesis in BMSC. Our results showed that the expression level of ICER gene was significantly increased to 4.12 folds (0.01729 vs. 0.07138; p<0.05), whereas NOV, HSPB1, and SDH genes were decreased to 2.89, 3.18 and 2.36 folds, respectively, on day 6 when compared to day 0. These results suggest that these candidate genes have stimulatory or inhibitory effects on adipogenesis in BMSC, indicating that these genes may be directly or indirectly related to the adipogenic event of adipose precursor cells.

A Web-based Internet Program for Nutritional Counseling and Diet management of Patient with Diabetes Mellitus (당뇨병 환자의 웹기반 식사관리 및 영양상담 프로그램)

  • 한지숙;정지혜
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.33 no.1
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    • pp.114-122
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    • 2004
  • The purpose of this study was to develop a web-based internet program for nutritional counseling and diet management of patient with diabetes mellitus. The program consisted of four parts according to their functions and contents. The first part explained the metabolism of glucose and mechanism of insulin and insulin receptor expressed by flash 6.0, and defined the diabetes mellitus. The second part is to assess the general health status such as body weight, obesity index, basal metabolic rate and total energy requirement by the input of age, sex, height, weight and degree of activity. This part also provides tlne patient with menu lists and one day menu suitable to his weight and activity, and offers the information for food selection, snacks, convenience foods, dine-out, behavioral modification, cooking methods, food exchange lists, dietary education using buffet, and information on energy and nutrients of foods and drinks, and top 20 foods classified by nutrients. The third part is designed to investigate dietary history of patient, that is, to find out his inappropriate dietary habit and give him some suggestions for appropriate dietary behavior. This part also offers on-line counseling, follow-up management and frequently asked questions. The fourth part is evaluating their energy and nutrients intake by comparing with recommended dietary allowance for Koreans or standardized data for patient with diabetes mellitus. In this part, it is also analyzing energy and nutrients of food consumed by food group and meals, and evaluating the status of nutrient intake. These results are finally displayed as tabular forms and graphical forms on the computer screen. Therefore it is expected that the web-based internet program developed in this study will play a role in their health promotion as widely using by diabetic patients.

Persimmon Vinegar Ingestion before Endurance Exercise on Energy Substrates Utilization (지구성 운동전 감식초 섭취시 에너지기질의 이용)

  • Seo, Hyobin;Nam, Ju-Ock;Jeon, Byung-Duk;Kim, Pan-Gi;Ryu, Sungpil
    • Journal of Korean Society of Forest Science
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    • v.101 no.4
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    • pp.626-634
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    • 2012
  • The purpose of this study was to investigate the potential of persimmon vinegar as a functional beverage by analyzing the effects of persimmon vinegar ingestion on the energy substrate during endurance exercise. The healthy male adolescents (n=8) drunk persimmon vinegar ingested trial (PSV) or purified water ingested trial as the control trial (CON) 1 h prior to the exercise with the 60% of maximal oxygen uptake ($\dot{V}O_{2max}$) for 1 h. The exercise intensity was increased to the 80% of $\dot{V}O_{2max}$ and remained until exhaustion. And, the physiological variables, blood components, and amounts of energy oxidation were analyzed. There was no significant difference between trials in physiological variables, and the heart rates after exhaustion were higher in PSV compared to CON. There was no significant difference between trials in blood glucose level, while the blood lactic acids decreased significantly in PSV 30 and 60 minutes after onset of exercise. The free fatty acids concentration increased significantly in PSV from 15 minutes to 60 minutes after onset of exercise. The carbohydrate oxidation decreased significantly in PSV from 45 minutes after exercise and, on the contrary, the fatty acids oxidation increased significantly for the same period. And, fatty acids oxidation was higher in PSV compared to CON even after exhaustion. The respiratory exchange ratio was lower significantly in PSV compared to CON from 30 minutes to 60 minutes after exercise, whereas lower in CON after total exhaustion. The exercise time to exhaustion was 41% longer in PSV compared to CON. These results showed that the persimmon vinegar increase the level of lipids metabolism and decrease sense of fatigue by inhibiting carbohydrate oxidation during moderate intensity exercise, suggesting the possibility of using of persimmon vinegar as exercise functional beverage when ingested 1 h prior to the endurance exercise performance.

The Effects of Cordyceps Militaris on Lipid Metabolism, Protein Levels and Enzyme Activities in Rats Fed a High Fat Diet (동충하초 (Cordyceps Militaris)가 고지방식이를 섭취한 흰쥐의 지질대사, 단백질농도 및 효소활성에 미치는 영향)

  • 고진복
    • Journal of Nutrition and Health
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    • v.35 no.4
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    • pp.414-420
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    • 2002
  • The effects of Cordyceps militaris on weight gain, flood intakes, flood efficiency ratios, serum and hepatic lipid concentrations, serum protein levels and serum enzyme activities, were studied in adult male rats. Sprague-Dawley rats, 35 weeks old, were given four different types of diets for a succeeding period of five weeks: either a normal diet(5% com oil), a high fat diet(5% com oil + 15% lard). a CF diet (high fat diet + 3% Suiting body of cordyceps militaris), or a CM diet (high fat diet + 3% mycelium of cordyceps military). The body weight gains, hepatic weights and food efficiency ratios of the rats find (high fat) diets with CF and CM were significantly decreased compared with those find the high fat diet, but were similar to those find the normal diet. The concentrations of serum and hepatic triglycerides, and hepatic total lipids and cholesterol, of rats given CF or CM diets were significantly lower than those given the high fat diet. But the concentrations of total cholesterol, HDL-cholesterol, phopholipid and total protein in the serum of rats find the high fat, CF or CM diets were significantly higher than those find the normal diet. In the serum of the rats find the CF diet, the HDL-cholesterol/total cholesterol ratio was significantly higher but the atherogenic index was significantly lower than those fed the high fat diet. The alkaline phosphatase activity in the serum of the rats find the high fat, CF, and CM diets was more significantly decreased compared to rats on the normal dirt. No differences were noted in the weights of the pancreas, kidney and heart, the serum concentrations of glucose, hemoglobin and albumin, and the activities of GOT, GPT and ${\gamma}$-GTP, among the rats on all the experimental diets. In conclusion, the rats find the Cf or CM diets maintained normal body and hepatic weights. Despite of the high intake of fats in the CF and CM diets, the concentrations of total lipids, cholesterol and triglycerides were decreased in the liver, also, the concentration of triglycerides was decreased in the serum.

Effect of Diets with Job's Tears and Cudrania tricuspidata Leaf Mixed-powder Supplements on Body Fat and Serum Lipid Levels in Rats Fed a High-Fat Diet (율무와 꾸지뽕잎 혼합물이 고지방식이를 급여한 흰쥐의 체지방 및 혈청 지질농도에 미치는 영향)

  • Park, Ju-Hun;Lee, Kyoung-Won;Sung, Ki-Seung;Kim, Sung-Soo;Cho, Kyung-Dong;Lee, Bog-Hieu;Han, Chan-Kyu
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.41 no.7
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    • pp.943-949
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    • 2012
  • This study investigated the effects of diets with a supplement of Job's tears (JT) and Cudrania tricuspidata leaf (CTL) powder as a functional food ingredient on obesity and lipid profiles in diet-induced obese rats. Male Sprague-Dawley rats were fed a high-fat diet (D12492, 45 kcal fat) for 5 weeks during the first phase. In the second phase, which lasted 5 weeks, the rats were divided into four experimental groups. The groups were a high-fat diet group as a control (HFD), a high-fat diet with 10% JT powder supplement group (JT-10), a 10% CTL powder supplement group (CTL-10), and a 20% JT and CTL 1:1 mixed-powder supplement group (MIX-20). The adipose tissue (RFP, EFP) weights were significantly decreased in the CTL-10 and MIX-20 groups than in the HFD group (p<0.05). The fecal weight produced by the MIX-20 group was highest among all experimental groups (p<0.05), and it was significantly increased during the second phase compared to the first phase (p<0.05). The serum total cholesterol content was significantly decreased in the CTL-10 and MIX-20 groups, and the LDL-cholesterol content was significantly low in the JT-10 and MIX-20 groups than in the HFD group (p<0.05). The blood glucose levels were significantly decreased in all experimental diet supplement groups than in the HFD group (p<0.05). The findings show that a diet of mixed JT and CTL could improve lipid metabolism.