• Journal of The Korean Society of Inherited Metabolic disease
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• v.1 no.1
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• pp.5-12
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• 2001

아시아 및 한국에서 처음 발견된 3명의 소아 Ethylmalonic encephalopathy (EE) 환자들은 riboflavin을 이용한 치료법에 점진적인 호전을 보인 것으로 나타났다. Ethylmalonic aciduria가 3가지 효소-2-methyl-branched-chain acyl CoA dehydrogenase (2MBCAD), iso-valeryl CoA dehydrogenase (IVCAD), 그리고 short-chain acyl CoA dehydrogenase (SCAD) - 들의 부분적인 결함으로 생긴다는 가설하에 dehydrogenase (탈수효소)의 보조 효소인 riboflavin이 부분적으로 나마 치료 과정에서 어떤 역할을 수행하는지의 여부를 살펴보았다. 다음의 세 증례에서 riboflavin 혹은 coenzyme Q10을 병행하여 치료한 결과 인지 행동(cognitive behavior)과 만성 점액성 설사(chronic mucoid diarrhea)가 호전된 것으로 관찰되었다. 또한, 치료 후 운동 기능도 일부 호전되었다. EE의 질병 발생 기전이 현재까지 완전히 밝혀지지는 않았지만, 세 명의 EE 환자들의 치료경과를 관찰하여 위의 가설을 뒷받침 할 수 있었다.

• Korean Journal of Optics and Photonics
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• v.2 no.4
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• pp.227-232
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• 1991

The possibility for the high intensity and energy possessing a short pulse in the HF chemical laser system which contained fluoride molecules (RF) was demonstrated theoretically through the numerical model simulation. The calculation was accomplished by assuming that the thermal branched chain mechanism of RF was occurred in the initiation step of $H_2+F_2$ chain reaction. Variations of the major chemicals and the temperature in the system were calculated as a function of time. An analysis was also performed to evaluate output pulse profile through parametric studies.

• The Korean Journal of Food And Nutrition
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• v.28 no.5
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• pp.813-820
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• 2015

This study was performed to develop patties with quality characteristics using mealworm powder, followed by assessments of general compositions and the branched-chain amino acid (BCAA) levels of the patties. An analysis of the chromaticity of the patties shows that the lightness and yellowness were decreased, whereas the redness was increased, as the amount of mealworm powder was increased. According to the sensory evaluation, the mealworm patty that contain 20% of mealworm powder (M20) showed an overall high preference level. In terms of the mechanical properties, the hardness, gumminess, and chewiness were significantly increased, whereas the springiness and cohesivensee were decreased, as the amount of mealworm powder was increased. The general composition of the M20 consists of 41.84% (moisture), 8.78% (carbohydrates), 34.42% (crude protein), 13.15% (crude fats), and 1.81% (crude ash). The BCAA contents (leucine, isoleucine, and valine) significantly increased in correspondence with the increases of the as mealworm-powder amount regarding the M20, the BCAA composition consists of the following: leucine (2,906.25 mg/100 g), isoleucine (1,459.09 mg/100 g), and valine (1,813.18 mg/100g). The conclusion of this study suggests that mealworm is a potential food material that could possibly replace meat.

• Journal of Microbiology and Biotechnology
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• v.9 no.6
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• pp.695-703
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• 1999

We have studied the stereospecificities of various pyridoxal 5'-phosphate (PLP) dependent enzymes for the hydrogen transfer between the C-4' of a bound coenzyme and the C-2 of a substrate in the transamination catalyzed by the enzymes. Stereospecificities reflect the structures of enzyme active-sites, in particular the geometrical relationship between the coenzyme-substrate Schiff base and the active site base participating in an $\alpha$-hydrogen abstraction. The PLP enzymes studied so far catalyze only a si-face specific (pro-S) hydrogen transfer. This stereochemical finding suggests that the PLP enzymes have the same topological active-site structures, and that the PLP enzymes have evolved divergently from a common ancestral protein. However, we found that o-amino acid aminotransferase, branched chain L-amino acid aminotransferase, and 4-amino-4-deoxychorismate lyase, which have significant sequence homology with one another, catalyze a re-face specific (pro-R) hydrogen transfer. We also showed that PLP-dependent amino acid racemases, which have no sequence homology with any aminotransferases, catalyze a non-stereospecific hydrogen transfer: the hydrogen transfer occurs on both faces of the planar intermediate. Crystallographical studies have shown that the catalytic base is situated on the re-face of the C-4' of the bound coenzyme in o-amino acid aminotransferase and branched chain L-amino acid aminotransferase, whereas the catalytic base is situated on the si-face in other aminotransferases (such as L-aspartate aminotransferase) catalyzing the si-face hydrogen transfer. Thus, we have clarified the stereospecificities of PLP enzymes in relation with the primary structures and three-dimensional structures of the enzymes. The characteristic stereospecificities of these enzymes for the hydrogen transfer suggest the convergent evolution of PLP enzymes.

• Korean Journal of Microbiology
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• v.31 no.4
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• pp.261-266
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• 1993

A new methyltrophic bacterium which utilizes methanol as a sole source of carbon and energy was isolated from soil. It was Gram-negative, nonmotile, nonspore-forming rod, and strictly aerobic bacterium. Catalase and oxidase activities were present. Nitrate was reduced to nitrite. Vitamins and other growth factors were not required. Generation time was 1.6 hr under the optimal condition. The isolate assimilated methanol via the ribulose mono-phosphate pathway (Enter-Doudoroff varient) and did not have .alpha.-ketoglutarate dehydrogenase. It assimilated ammonia through glutamate dehydrogenase. The guanine plus cytosine content of the DNA was 61.0 mol%. The celular fatty acid composition was primarily straight-chain saturated $C^{16 : 0}$ acids (palmitic acids) and unsaturated $C_{16 :1}$ acid (palmitoleic acids), and the isolate also contained two unidentified $C_{17}$ branched fatty acids. The major ubiquinone was Q-8, and Q-6 and Q-7 were present as minor components. Phosphatidylethanolamine and phosphatidylglycerol were predominantly present, and diphosphatidyglycerol was also detected. Based on the physiological and biochemical properties, the isolate was assigned to a novel species of the genus Methylobacillus, Methylobacillus methanolovorus sp. nov.

• Animal Bioscience
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• v.34 no.10
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• pp.1600-1606
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• 2021

Objective: This study was conducted to enhance our understanding of nitrogen (N) metabolism and mammary amino acid (AA) utilization in lactating cows with divergent phenotypes of residual feed intake (RFI). Methods: Fifty-three multiparous mid-lactation Holstein dairy cows were selected for RFI measurements over a 50-d experimental period. The 26 cows with the most extreme RFI values were classified into the high RFI (n = 13) and low RFI (n = 13) groups, respectively, for analysis of N metabolism and AA utilization. Results: Compared with the high RFI cows, the low RFI animals had lower dry matter intake (p<0.01) with no difference observed in milk yield between the two groups (p>0.10). However, higher ratios of milk yield to dry matter intake (p<0.01) were found in the low RFI cows than in the high RFI cows. The low RFI cows had significant greater ratios of milk protein to metabolizable protein (p = 0.02) and milk protein to crude protein intake than the high RFI cows (p = 0.01). The arterial concentration and mammary uptake of essential AA (p<0.10), branched-chain AA (p<0.10), and total AA (p<0.10) tended to be lower in the low RFI cows. Additionally, the low RFI cows tended to have a lower ratio of AA uptake to milk output for essential AA (p = 0.08), branched-chain AA (p = 0.07) and total AA (p = 0.09) than the high RFI cows. Conclusion: In summary, both utilization of metabolizable protein for milk protein and mammary AA utilization are more efficient in cows with lower RFI than in the high RFI cows. Our results provide new insight into the protein metabolic processes (related to N and AA) involved in feed efficiency.

• Animal Bioscience
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• v.36 no.2
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• pp.307-314
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• 2023

Objective: Chicken essence and branched chain amino acid (BCAA) supplementation has been recognized to significantly relieve fatigue. To obtain chicken essence with high amounts of BCAA, spent hens herein was used to prepare dripped chicken essence (SCE) and compared with commercial dripped chicken essence (CCE) for in vivo anti-fatigue effect. Methods: To determine the effect on anti-fatigue by dripped chicken essence, the exhaustive swimming was performed. Thirty-two 7-week ICR mice were divided into four groups, which included the control group (CG), CCE, SCE-1X and SCE-2X. The mice were given daily oral administration (0.012 mL/g body weight/d). The fatigue index analysis was conducted weekly. Results: The results showed that SCE had a higher BCAA level as expected, and mice treated with dripped chicken essence (CCE and SCE) could significantly improve exercise performance. The lower blood lactate level, blood urea nitrogen level and creatine phosphokinase activity were found in the supplement of SCE group compared with the CCE group, which suggested that the SCE possessed strong anti-fatigue ability. This could possibly be due to the higher content of BCAA. Conclusion: In this study, SCE promoted recovery from physical fatigue in mice and elevated endurance ability. Among them, the double dose (SCE-2X) showed the strongest anti-fatigue ability. Taken together, spent chickens could be a good source of chicken essence to improve the effect of anti-fatigue.

• Korean Journal of Exercise Nutrition
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• v.24 no.4
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• pp.28-33
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• 2020

[Purpose] This study aimed to investigate the effects of branched-chain amino acid (BCAA) supplement on delayed onset muscle soreness (DOMS) by analyzing the maximum muscle strength and indicators of muscle damage. [Methods] Twelve men with majors in physical education were assigned to the BCAA group and placebo group in a double-blinded design, and repeated measurements were conducted. DOMS was induced with an isokinetic exercise. Following BCAA administration, the changes in the knee extension peak torque, flexion peak torque, aspartate aminotransferase (AST), creatine kinase (CK), and lactate dehydrogenase (LDH) concentrations were analyzed. The maximum knee muscle strength was measured at the baseline (pre-D0) following BCAA administration for 5 days before exercise (-D5, -4D, -3D, -2D, -1D). In contrast, the post-treatment measurements (D3) were recorded after BCAA administration for 3 days (post-D0, D1, D2). Blood samples were obtained before (pre-D0), immediately after (post-D0), 24 h (D1), 48 h (D2), and 72 h (D3) after the exercise to analyze the indicators of muscle strength. BCAA was administered twice daily for 8 days (5 days and 3 days before inducing DOMS and during the experimental period, respectively). [Results] There was no difference in the flexion peak torque between the groups. However, the BCAA group showed a significantly higher extension peak torque at D3 (second isokinetic exercise), compared to the placebo group (p<.05). There was no difference in AST changes between the groups. Nonetheless, the CK and LDH were significantly reduced in the BCAA group, compared to the placebo group. There was no correlation between the extension peak torque and flexion peak torque. However, the CK and LDH increased proportionately in DOMS. Moreover, their concentrations significantly increased with a decreasing peak torque (p<.01). [Conclusion] An exercise-induced DOMS results in a decrease in the peak torque and a proportional increase in the CK and LDH concentrations. Moreover, the administration of BCAA inhibits the reduction of the extension peak torque and elevation of CK and LDH concentrations. Therefore, BCAA might be administered as a supplement to maintain the muscle strength and prevent muscle damage during vigorous exercises that may induce DOMS in sports settings.

• Journal of Microbiology and Biotechnology
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• v.33 no.6
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• pp.724-735
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• 2023

NdgR, a global regulator in soil-dwelling and antibiotic-producing Streptomyces, is known to regulate branched-chain amino acid metabolism by binding to the upstream region of synthetic genes. However, its numerous and complex roles are not yet fully understood. To more fully reveal the function of NdgR, phospholipid fatty acid (PLFA) analysis with gas chromatography-mass spectrometry (GC-MS) was used to assess the effects of an ndgR deletion mutant of Streptomyces coelicolor. The deletion of ndgR was found to decrease the levels of isoleucine- and leucine-related fatty acids but increase those of valine-related fatty acids. Furthermore, the defects in leucine and isoleucine metabolism caused by the deletion impaired the growth of Streptomyces at low temperatures. Supplementation of leucine and isoleucine, however, could complement this defect under cold shock condition. NdgR was thus shown to be involved in the control of branched-chain amino acids and consequently affected the membrane fatty acid composition in Streptomyces. While isoleucine and valine could be synthesized by the same enzymes (IlvB/N, IlvC, IlvD, and IlvE), ndgR deletion did not affect them in the same way. This suggests that NdgR is involved in the upper isoleucine and valine pathways, or that its control over them differs in some respect.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.31-38
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• 2023

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency in branched chain α-keto acid dehydrogenase (BCKAD). Between 1997, when Korea's MSUD case was first reported, and 2023, 14 cases were reported in the literature. 29% of the cases experienced developmental delay, and 29% expired. The prevalence of MSUD in Korea was estimated to be 1 in 230,000. Of 21 MSUD patients currently being treated at the Korea Genetics Research Center, 19 were detected through newborn screening program, and 2 were diagnosed by the symptoms. 14 MSUD patients had confirmed genetic mutations; 6 (43%) were BCKDHA and 8 (57%) were BCKDHB. In one case, a large deletion was observed. 4 patients had leucine levels above 2,000 (umo/L), and post-dialysis diet therapy was initiated in the newborn period. No patient required further dialysis as diet therapy and regular monitoring proved highly effective. Most MSUD patients were growing normally; weight and height growth were above the 50th percentile in 76% of the cases while BMI values were higher than normal in 71% of cases. Developmental delays were observed only in 2 cases (10%) and anticonvulsant use in 3 cases (14%). With newborn screening available to all Korean infants, early diagnosis and intervention should allow most patients to remain asymptomatic. However, ongoing surveillance, dietary management and continued patient compliance as well as rapid correction of acute metabolic decompensations remain critical to a favorable long-term prognosis.