• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.22-27
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• 2021

Propionic acidemia (PA) is an inherited autosomal recessive disorder, due to the deficiency of propionyl-CoA carboxylase (PCC). PCC is the enzyme which catalyzes the conversion of propionyl-CoA to D-methylmalonyl-CoA, and it is critical for the metabolism of amino acids, odd-chain fatty acids, and side chains of cholesterol. The clinical manifestations present mostly at the neonatal period with life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of a 16-year-old Korean boy with late-onset PA who presented with embolic cerebral infarction due to dilated cardiomyopathy (DCMP) with left ventricular noncompaction. And he has family history of sudden cardiac death, so we performed metabolic screening and genetic tests. Elevated levels of 3-hydroxypropionic acid, methylcitric acid and propionylglycerine were detected in urine. Plasma acylcarnitine profile showed elevated propionylcarnitine (C3). Diagnosis of PA was confirmed by genetic analysis, which revealed compound heterozygous mutations, c.[1151T>G] (p.[Phe384Cys]) and c.[1228C>T] (p.[Arg410Trp]) in PCCB gene. His heart function is in improving state and the results of biochemical analysis are stable with heart failure medication and metabolic managements. We present a case of patient without episodes of metabolic decompensation who manifests DCMP as the first symptom of PA.

• Molecules and Cells
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• v.45 no.3
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• pp.134-147
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• 2022

The anti-oxidant enzyme heme oxygenase-1 (HO-1) is known to exert anti-inflammatory effects. From a library of pyrazolo[3,4-d]pyrimidines, we identified a novel compound KKC080096 that upregulated HO-1 at the mRNA and protein levels in microglial BV-2 cells. KKC080096 exhibited anti-inflammatory effects via suppressing nitric oxide, interleukin1β (IL-1β), and iNOS production in lipopolysaccharide (LPS)-challenged cells. It inhibited the phosphorylation of IKK and MAP kinases (p38, JNK, ERK), which trigger inflammatory signaling, and whose activities are inhibited by HO-1. Further, KKC080096 upregulated anti-inflammatory marker (Arg1, YM1, CD206, IL-10, transforming growth factor-β [TGF-β]) expression. In 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridinetreated mice, KKC080096 lowered microglial activation, protected the nigral dopaminergic neurons, and nigral damage-associated motor deficits. Next, we elucidated the mechanisms by which KKC080096 upregulated HO-1. KKC080096 induced the phosphorylation of AMPK and its known upstream kinases LKB1 and CaMKKbeta, and pharmacological inhibition of AMPK activity reduced the effects of KKC080096 on HO-1 expression and LPS-induced NO generation, suggesting that KKC080096-induced HO-1 upregulation involves LKB1/AMPK and CaMKKbeta/AMPK pathway activation. Further, KKC080096 caused an increase in cellular Nrf2 level, bound to Keap1 (Nrf2 inhibitor protein) with high affinity, and blocked Keap1-Nrf2 interaction. This Nrf2 activation resulted in concurrent induction of HO-1 and other Nrf2-targeted antioxidant enzymes in BV-2 and in dopaminergic CATH.a cells. These results indicate that KKC080096 is a potential therapeutic for oxidative stress-and inflammation-related neurodegenerative disorders such as Parkinson's disease.

• Korean Journal of Environmental Agriculture
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• v.41 no.4
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• pp.335-343
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• 2022

BACKGROUND: Antibiotics used in animal husbandry for disease prevention and treatment have resulted in the rapid progression of antibiotic resistant bacteria which can be introduced into the environment through livestock feces/manure, disseminating antibiotic resistant genes (ARGs). In this study, fecal samples were collected from the livestock farms located in Jeju Island to investigate the relationship between microbial communities and ARGs. METHODS AND RESULTS: Illumina MiSeq sequencing was applied to characterize microbial communities within each fecal sample. Using quantitative PCR (qPCR), ten ARGs encoding tetracycline resistance (tetB, tetM), sulfonamide resistance (sul1, sul2), fluoroquinolone resistance (qnrD, qnrS), fluoroquinolone and aminoglycoside resistance (aac(6')-Ib), beta-lactam resistance (bla_TEM, bla_CTX-M), macrolide resistance (ermC), a class 1 integronsintegrase gene (intI1), and a class 2 integrons-integrase gene (intI2) were quantified. The results showed that Firmicutes and Bacteroidetes were dominant in human, cow, horse, and pig groups, while Firmicutes and Actinobacteria were dominant in chicken group. Among ARGs, tetM was detected with the highest number of copies, followed by sul1 and sul2. Most of the genera belonging to Firmicutes showed positive correlations with ARGs and integron genes. There were 97, 34, 31, 25, and 22 genera in chicken, cow, pig, human, and horse respectively which showed positive correlations with ARGs and integron genes. In network analysis, we identified diversity of microbial communities which correlated with ARGs and integron genes. CONCLUSION(S): In this study, antibiotic resistance patterns in human and livestock fecal samples were identified. The abundance of ARGs and integron genes detected in the samples were associated with the amount of antibiotics commonly used for human and livestocks. We found diverse microbial communities associated with antibiotics resistance genes in different hosts, suggesting that antibiotics resistance can disseminate across environments through various routes. Identifying the routes of ARG dissemination in the environment would be the first step to overcome the challenge of antibiotic resistance in the future.

• Journal of Sericultural and Entomological Science
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• v.16 no.1
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• pp.21-48
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• 1974

The studies were carried out to disclose the physical and chemical properties of sericin fraction obtained from silk cocoon shells and its characteristics of swelling and solubility. The following results were obtained. 1. The physical and chemical properties of sericin fraction. 1) In contrast to the easy water soluble sericin, the hard soluble sericin contains fewer amino acids include of polar side radical while the hard soluble amino acid sach as alanine and leucine were detected. 2) The easy soluble amino acids were found mainly on the outer part of the fibroin, but the hard soluble amino acids were located in the near parts to the fibroin. 3) The swelling and solubility of the sericin could be hardly assayed by the analysis of the amino acid composition, and could be considered to tee closely related to the compound of the sericin crystal and secondary structure. 4) The X-ray patterns of the cocoon filament were ring shape, but they disappeared by the degumming treatment. 5) The sericin of tussah silkworm (A. pernyi), showed stronger circular patterns in the meridian than the regular silkworm (Bombyx mori). 6) There was no pattern difference between Fraction A and B. 7) X-ray diffraction patterns of the Sericin 1, ll and 111 were similar except interference of 8.85A (side chain spacing). 8) The amino acids above 150 in molecular weight such as Cys. Tyr. Phe. His. and Arg. were not found quantitatively by the 60 minutes-hydrolysis (6N-HCI). 9) The X-ray Pattern of 4.6A had a tendency to disappear with hot-water, ether, and alcohol treatment. 10) The partial hydrolysis of sericin showed a cirucular interference (2A) on the meridian. 11) The sericin pellet after hydrolysis was considered to be peptides composed with specific amino acids. 12) The decomposing temperature of Sericin 111 was higher than that of Sericin I and II. 13) Thermogram of the inner portioned sericin of the cocoon shell had double endothermic peaks at 165$^{\circ}C$, and 245$^{\circ}C$, and its decomposing temperature was higher than that of other portioned sericin. 14) The infrared spectroscopic properties among sericin I, II, III and sericin extracted from each layer portion of the cocoon shell were similar. II. The characteristics of seriein swelling and solubility related with silk processing. 1) Fifteen minutes was required to dehydrate the free moisture of cocoon shells with centrifugal force controlled at 13${\times}$10$^4$ dyne/g at 3,000 R.P.M. B) It took 30 minutes for the sericin to show positive reaction with the Folin-Ciocaltue reagent at room temperature. 3) The measurable wave length of the visible radiation was 500-750m${\mu}$, and the highest absorbance was observed at the wave length of 650m${\mu}$. 4) The colorimetric analysis should be conducted at 650mu for low concentration (10$\mu\textrm{g}$/$m\ell$), and at 500m${\mu}$ for the higher concentration to obtain an exact analysis. 5) The absorbing curves of sericin and egg albumin at different wave lengths were similar, but the absorbance of the former was slightly higher than that of the latter. 6) The quantity of the sericin measured by the colorimetric analysis, turned out to be less than by the Kjeldahl method. 7) Both temperature and duration in the cocoon cooking process has much effect on the swelling and solubility of the cocoon shells, but the temperature was more influential than the duration of the treatment. 8) The factorial relation between the temperature and the duration of treatment of the cocoon cooking to check for siricin swelling and solubility showed that the treatment duration should be gradually increased to reach optimum swelling and solubility of sericin with low temperature(70$^{\circ}C$) . High temperature, however, showed more sharp increase. 9) The more increased temperature in the drying of fresh cocoons, the less the sericin swelling and solubility were obtained. 10) In a specific cooking duration, the heavier the cocoon shell is, the less the swelling and solubility were obtained. 11) It was considered that there are differences in swelling or solubility between the filaments of each cocoon layer. 12) Sericin swelling or solubility in the cocoon filament was decreased by the wax extraction.. 13) The ionic surface active agent accelerated the swelling and solubility of the sericin at the range of pH 6-7. 14) In the same conditions as above, the cation agent was absorbed into the sericin. 15) In case of the increase of Ca ang Mg in the reeling water, its pH value drifted toward the acidity. 16) A buffering action was observed between the sericin and the water hardness constituents in the reeling water. 17) The effect of calcium on the swelling and solubility of the sericin was more moderate than that of magnecium. 18) The solute of the water hardness constituents increased the electric conductivity in the reeling water.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.556-564
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• 2007

Purpose : We hypothesized that the persisting bronchial hyperresponsiveness (BHR) of adolescents with asthma remission may be controlled mainly by genetic factors, and the BHR of symptomatic asthma by airway inflammation. ${\beta}_2$-adrenoceptor gene is considered to be a candidate gene in the development of BHR. Thus, ${\beta}_2$-adrenoceptor gene polymorphism may be associated with the BHR of adolescents with asthma remission, but not with the BHR of symptomatic asthma. To evaluate this hypothesis, ${\beta}_2$-adrenoceptor gene polymorphism at 2 sites (Arg16-Gly, Gln27-Glu) were examined. Methods : Two hundred two adolescents with BHR ($PC_{20}<18\;mg/mL$) and long term remission (neither asthma-related symptoms nor medication during the previous 2 years) of their asthma (remission group), 182 adolescents with symptomatic asthma (symptomatic group), and 200 healthy adolescents (control group) were studied. Asthma phenotypes were determined using methacholine bronchial provocation test and skin prick test. Genotypes of ${\beta}_2$-adrenoceptor polymorphism were evaluated by PCR-based methods. Results : Gly/Gly allele and Gly16-Gln27 haplotype were more prevalent in the remission group than in the control group (P=0.01, P=0.02), although there was no difference between the symptomatic group and the control group. In the remission group, there was significant difference in geometric mean of $PC_{20}$ among the 3 groups subdivided by the number of Gly16-Gln27 haplotype, showing that the Gly16-Gln27 haplotype was positively associated with BHR. However, no association was found between Gly16-Gln27 haplotype and BHR in the symptomatic group. Conclusion : This study demonstrates that ${\beta}_2$-adrenoceptor polymorphism at amino acid 16 and 27 was associated with BHR persisting in adolescents with asthma remission.

• Asian-Australasian Journal of Animal Sciences
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• v.22 no.1
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• pp.99-106
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• 2009

For Exp. 1, 120 ((Yorkshire${\times}$Landrace)${\times}$Duroc) weaned pigs (7.96${\pm}$0.01 kg average initial BW, 21 days weaning) were used in a 28 d-growth assay to determine the effects of replacing soy protein concentrate (SPC) with fermented soy protein (FSP) in a starter diet (d 0 to 7) on the growth performance, apparent fecal amino acid digestibility and subsequent performance in weaned pigs. Dietary treatments included: i) FSP0 (basal diet; whey-skim milk powder-SPC based diet); ii) FSP5 (replacing SPC with 5% FSP); iii) FSP10 (replacing SPC with 10% FSP). Pigs were fed the phase I diet for 7 days, and then each group was fed a common commercial diet for 21 days to determine the effect of previous diet on subsequent performance. Average daily gain (ADG) from d 5 to 7 (linear effect, p = 0.01) and d 7 to 14 (linear effect, p<0.001) were increased as FSP level increased. The pigs fed with FSP was heavier than the pigs fed with SPC at d 5 to 7 and d 7 to 14 after weaning (p<0.05). In the entire period (d 0 to 28), there were no significant differences in weight gain and final weight between SPC and FSP diets (p>0.05). Average daily feed intake (ADFI) was higher in pigs fed with the 5% FSP diet than those fed with the other diets at d 0 to 2 post-weaning (quadratic effect, p = 0.05). Also, for the entire period of phase I (d 0 to 7), pigs consumed more 5% FSP diet compared to other treatments (quadratic effect, p = 0.03). Gain/feed (G/F) was not affected by dietary SPC or FSP in phase I and subsequent periods, but G/F from d 5 to 7 after weaning was improved linearly (p = 0.04) as dietary FSP level increased. Pigs fed with 10% FSP also improved G/F compared with those fed only SPC (p<0.05). At d 7, there were linear increments in fecal dry matter (DM) (p<0.1) and nitrogen (N) (p<0.01) digestibilities as the dietary FSP level increased. The digestibilities of fecal essential and total amino acids were increased as the FSP level increased (linear effect, p<0.1). For Exp. 2, three ((Yorkshire${\times}$Landrace)${\times}$Duroc) weaned barrows (average initial BW of 7.32 kg) were surgically fitted with a simple T-cannula approximately 15 cm prior to the ileo-cecal junction. The experimental designs were 3${\times}$3 latin squares with pigs and periods as blocking criteria. Dietary treatments and composition were the same as in Exp. 1. Apparent ileal N digestibility was increased as FSP level was increased (linear effect, p<0.05). The dietary treatments (SPC and FSP) did not affect apparent ileal DM digestibility (p>0.05). Among essential amino acids, apparent digestibility of ileal arginine (Arg), lysine (Lys), methionine (Met) and phenylalanine (Phe) were improved as the FSP level increased (linear effect, p<0.1). Also, apparent ileal total essential, non-essential and total amino acid digestibilities were increased linearly (p<0.1). In conclusion, replacing SPC with fermented soy protein appeared beneficial in growth performance, N and amino acid digestibility during the early 7 days after weaning, and an equivalent effect showed on growth performance in subsequent period of 7 to 28 days after weaning.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.11-17
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• 2017

Purpose: Phenylketonuria (PKU) results from a deficiency of phenylalanine hydroxylase (PAH). The mutation of the PAH gene results in decreased phenylalanine hydroxylase enzyme activity in hyperphenylalaninemia (HPA) patients. This study reports ten cases of patients with the benign HPA genotype c.158G>A (p.Arg53His, R53H) variant in the PAH gene and aims to evaluate the clinical significance of the R53H variant. Methods: Ten Korean patients with the HPA genotype the R53H variant were included in this study. A retrospective medical record review was conducted. We characterized the phenotypes of the patients with HPA with the R53H variant using the following system: classic PKU, moderate PKU, mild PKU, Mild HPA, and benign HPA. Results: Five patients had the R53H variant with the "Pathogenic" variants (R413P, R241C, $Y356^*$, c.442-1G>A, $Y325^*$), Two patients had the "Likely pathogenic" variants ($W187^*$, A259T), Two patients had the "Uncertain significance" variants (R53H, G344D), and One patient had the "Not provided" variant (c.1066-14C>G). Nine patients genotyped with the R53H variant were the patient with benign HPA and One patient genotyped with the R53H homozygote was within normal range of plasma phenylalanine. None of the ten patients required dietary restriction of phenylalanine or pharmacotherapy to maintain their plasma phenylalanine levels and showed no clinical symptoms of HPA. Conclusion: Ten patients with HPA genotype the R53H variant were the patient with benign HPA and showed no clinical symptoms of HPA. Thus, the R53H variant, which was previously classified as an "Uncertain significance" mutation in HPA patients, should be re-classified as "Benign."

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1068-1075
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• 2005

Purpose : The role of ghrelin, which promotes the secretion of growth hormone, was not well known until now. Recently it was found that the mutation of ghrelin gene is related to obesity and diabetes. This study is to find the screening method that can easily and effectively detect the polymorphism of Leu72Met in ghrelin gene of obesity patients and apply it to clinical usage. Methods : We compared PCR-RFLP, PCR-SSCP and ARMS methodologies for analyzing of the polymorphism of Leu72Met in ghrelin gene of obesity children, and also studied the merits and demerits of these methodologies. Results : In this study, we were able to find out the band of peculiar allele of Leu72Met in ghrelin gene using PCR-RFLP, PCR-SSCP and ARMS analyses. The polymorphism of Leu72Met in ghrelin gene determined by all above methodologies was in complete agreement. Compared to the PCR-RFLP and PCR-SSCP, ARMS analysis is simple, inexpensive and also consume less time. It is very sensitive to analyze the polymorphism and easy to understand the results of test. Conclusion : Though PCR-RFLP, PCR-SSCP and ARMS analyses were sensitive to analyze the polymorphism of Leu72Met in ghrelin gene, ARMS analysis appears to be more efficient than PCR-RFLP and PCR-SSCP. Therefore, we conclude that ARMS analysis is suitable to analyze the polymorphism of Leu72Met in ghrelin gene for large quantity of specimens.

• Korean Journal of Veterinary Research
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• v.35 no.2
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• pp.327-336
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• 1995

This study was carried out to develop the animal protein(feed for fry) that was isolated, purified and lyophilized the rumen ciliates from the healthy rumen contents which have $10^5-10^6/g$ ciliates and were discarded in abattoirs. The rumen ciliates are non-pathogenic, anaerobic and the weight of this protozoa is 2% of rumen content. The rumen protozoan and bacterial proteins both have a biological value for rats of 80-81, which is higher than the 72 of brewer's yeasts. Furthermore, the true digestibility and net protein utility of the protozoan protein are 91 and 73, much higher than those of bacterial(74 and 60) or yeast(84 and 60) proteins. The amino acids of rumen protozoa is nutritionally superior than the others. The size of rumen ciliates is $30-200{\times}20-110{\mu}m$ and so we had isolated and purified the rumen ciliates from the rumen contents by the physical methods. The purified rumen protozoa was lyophilized with freezing dryer. The results of this experiment were as follows : 1. Population dynamics of protozoan ciliates in slaughtered rumens; % of samples which small ciliates were predominated was 82.5%(52/63) and that of large ciliates was 17.5%(11/63). 1) predominant species of small ciliates were Entodinium ovinum and E nanellum. 2) predominant species of large ciliates were Epidinium ecaudatum and Diploplastron affine. 2. The lyophilized rumen ciliates which were isolated and purified from 1 kg of rumen content at the pH 6.2-6.8 was about 7.0 gram. 3. The nutrient analysis of lyophilized rqmen ciliates(LRC) was as follows: 1) Proximate analysis of the LRC and the composition of fry feed; moisture 8.05%(below 10.0), protein 35.37%(45), fat 5.39%(4.5), fiber 1.23%(below 2.5), ash 2.25%(below 15.0), Ca 0.26%(below 2.0), P 0.14%(below 1.1), energy 4,608.11(fish meal 5000 cal/g) 2) Amino acids (% in crude protein) of the LRC and the rotifer(Brachionus plicatilis); Arg 5.19%(4.50), His 2.50%(1.55), Ile 5.29%(3.45), Leu 8.11%(5.85), Lys 10.34%(6.15), Met 2.25% (0.85), Phe 5.66%(3.80), Thr 5.14% (3.45), Val 4.18%(3.90), Ala 4.13%(3.35), Asp 13.26%(8.25), Glu 16.62%(9.20), Gly 4.23%(3.10), Pro 3.25%(5.05), Ser 4.85%(3.85), Tyr 5.04%(3.05) 3) Fatty acids(% in fat) of the LRC and the rotifer(biological feed ; Brachionus plicatilis); myristic acid(C14:0) 3.27%(0.3), myristoleic acid(C14:1) 0.83%(-), palmitic acid(C16:0) 39.11% (23.5), palmitoleic acid(C16:1) 2.81%(2.0), stearic acid(C18:0) 9.36%(5.6), oleic acid(C18:1) 25.54%(3.5), linoleic acid(C18:2) 15.05%(32.9), linolenic acid(C18:3) 1.74%(9.8). Judging from the above investigated results, the analytical data of proximate analysis, amino acids, fatty acids of the purified and lyophilized rumen protozoa are reasonable for the feed of freshwater fishes(fry and fingerling). But it was disappointed of our expectation that the crude protein of lyophilized rumen ciliates contains low percentage, it was thought that because of the small ciliates(starch digester) in beef cattle rumens which were administered the concentrated feed, is much difficult to isolate and purify than the large ciliates(fiber digester).

• Korean Journal of Fisheries and Aquatic Sciences
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• v.32 no.6
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• pp.791-797
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• 1999

This study was conducted in order to evaluate nutritive values of yeasts (Kluyveromyces fragilis and Candida utilis) according to growth stages (early log phase, log phase, stationary phase and death phase) and chemical treatment of their cell wall, Proximate, amino acids, fatty acids and nucleotides composition of the yeast samples was determined. Crude protein content was high in K. fragilis ($48\~59\%$) compared to C. utilis ($26\~43\%$). Crude lipid and fiber contents of the yeasts were below than $1.6\%$ and $3.3\%$, respectively. Conposition of aspartic acid, glycine, proline, leucine, Iysine and valine of K. fragilis were higher than those of C. utilis, and glutamic acid and arginine of C. utilis were higher than those of K. fragilis. Proximate and amino acids composition was not siginificantly influenced by growth stage of the yeasts. Major fatty acids of the yeasts in all growth stages were $C_{10-18}$. $C_{16-18}$ contents were relatively high in the early log or log phase and $C_{10-12}$ contents were relatively high in the stationary or death phase. However, n-3 highly unasturated fatty acids (C$\ge$20) in the all growth stages were not observed. This result indicated that these yeast strains could not be adequate as a dietary lipid source for marine fish. Composition of nucleotides and their related compounds (ATP ADP AMP, IMP and inosine) in the early log phase yeasts were lower than those in the log, stationary and death phase yeasts.