• 대한유전성대사질환학회지
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• 제15권3호
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• pp.118-126
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• 2015

Background and objectives: A deficiency of BH4 (tetrahydrobiopterin) not only causes the classical phenylketonuric phenotype, but also is the source of neurological signs and symptoms due to impaired syntheses of L-Dopa and serotonin. The treatment of BH4 deficiency usually consists of replacement with BH4 and the neurotransmitters. We performed this study to finding out long-term follow-up clinical symptoms and prognosis of BH4 deficiency. Methods: Clinical and biochemical, genetic analysis were done retrospectively from January 1999 to July 2015 in Soonchunhyang University Hospital. Results: In our study, total 207 patients were confirmed to hyperphenylalaninemia. Among them, 10 patients were BH4 deficiency. 9 patients were 6-pyruvoyl-tetrahydropterin (PTPS) deficiency and one patient was dihydropteridine reductase (DHPR) deficiency. The patients who received delayed treatment, most of our patients suffered from severe psychomotor retardation, hypotonia and seizure. c.259C>T mutation was identified most commonly in PTPS gene analysis. A patient with DHPR deficiency had a mental retardation, dystonia, seizure. His seizure semiology was dialeptic feature. His EEG showed generalized spike wave patterns. All patients had treated with tolerate L-Dopa, BH4 and 5-hydroxytryptophan. Most of the early treated patients have a good tolerance for drugs well. But some patients had neurologic symptoms, despite early detection and treatment. Conclusion: BH4 deficiency patients who had delayed treatment tend to have severe psychomotor problem and neurologic deficits.

• Journal of Microbiology and Biotechnology
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• 제27권2호
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• pp.342-349
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• 2017

Polylactic acid (PLA) has been highlighted as an alternative renewable polymer for the replacement of petroleum-based plastic materials, and is considered to be biodegradable. On the other hand, the biodegradation of PLA by terminal degraders, such as microorganisms, requires a lengthy period in the natural environment, and its mechanism is not completely understood. PLA biodegradation studies have been conducted using mainly undefined mixed cultures, but only a few bacterial strains have been isolated and examined. For further characterization of PLA biodegradation, in this study, the PLA-degrading bacteria from digester sludge were isolated and identified using a polymer film-based screening method. The enrichment of sludge on PLA granules was conducted with the serial transference of a subculture into fresh media for 40 days, and the attached biofilm was inoculated on a PLA film on an agar plate. 3D optical microscopy showed that the isolates physically degraded the PLA film due to bacterial degradation. 16S rRNA gene sequencing identified the microbial colonies to be Pseudomonas sp. MYK1 and Bacillus sp. MYK2. The two isolates exhibited significantly higher specific gas production rates from PLA biodegradation compared with that of the initial sludge inoculum.

• Journal of Ginseng Research
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• 제20권3호
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• pp.241-247
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• 1996

The effects of ginseng total saponin, ginsenoside-Rb, and -Rb, on the reduction of chmlesterol level and the myNA expression rates of HMG CoA reductase and LDL receptor in Hep G2 were investigated and compared with that of lovastatin, a competitive HMG CoA reductase Inhibitor. The amounts of cholesterol in Hep G2 decreased in total saponin-and ginsenoside-treated groups as compared with that of control group, while there was no significant reduction in lovastatin-treated group. The mRNA expression rates of HMG CoA reductase increased in total saponin and gin- senoside groups except for ginsenoside-Rb, (10-3%) group and decreased in lovastatin group com- pared with that of control group. The mRNA expression rates of LDL receptor generally increased In all of the test groups except for total saponin (10-5%) group compared with that of control group. Because the ginseng components tested were more effective in the reduction of cholesterol level in Hep G2 than lovastatin and induced the gene expression of LDL receptor, we suggest the possibility that they could be used as a replacement agent for lovastatin which can not be prescribed especially to patients with hepatic diseases.

• 농업과학연구
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• 제47권2호
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• pp.219-227
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• 2020

Among insect species, black soldier fly larvae (BSFL) is a promising ingredient for animal feed as a dietary source. Moreover, BSFL contains a high content of lauric acid (C12:0), which has antimicrobial effects. Therefore, this study evaluated the effect of BSFL oil (BSFLO) as a partial or total replacement of soybean oil (SBO) on the cecal microbiota in broilers. A total of 210 male broiler chickens (Ross 308) at one-day of age were randomly allotted to 3 dietary treatments (10 replicates and 7 birds/group): a basal control diet (CON), the basal diet in which SBO was replaced by 50% (50 BSFLO) or 100% (100 BSFLO) BSFLO. At the end of the study (d 35), 18 birds (6 broilers/treatment) were randomly selected and slaughtered. Samples of cecal digesta were collected to verify their cecal microbiota. Overall, 235,978 gene sequences were generated, and a total of 4,398 operational taxonomic units were identified in the three groups. At the phylum level, Firmicutes was the dominant phyla in all three groups. At the genus level, Faecalibacterium was the dominant genera in all the treatments. There were no significant differences in the relative abundances of all the genera between the BSFLO groups and CON. However, the genus Erysipelatoclostridium was more abundant in the 50 BSFLO group than in the CON (p < 0.05). In conclusion, the substitution of SBO with BSFLO in broiler diets had no negative effect on the cecal microbiota of broilers.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권4호
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• pp.263-267
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• 2017

Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts. A dried blood spot test revealed markedly decreased activity of LAL. LIPA gene sequencing identified the presence of a novel homozygous mutation (p.Thr177Ile). The patient's elevated liver enzymes and dyslipidemia improved with enzyme replacement therapy. This is the first report of a Korean child with LAL deficiency, and our findings suggest that this condition should be considered in the differential diagnosis of children with hepatosplenomegaly and dyslipidemia.

• Animal Bioscience
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• 제34권9호
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• pp.1479-1490
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• 2021

Objective: Our recent findings confirmed the effectiveness of sodium metabisulfite (SMB) in controlling the growth of undesirable microorganisms in fruit and vegetable discards (FVD); however, lactic acid bacteria (LAB) are susceptible to its antibacterial effects. Two series of experiments were conducted to enable the survivability of LAB during silage fermentation of a total mixed ration (TMR) containing SMB-treated FVD. Methods: In Exp. 1, the objective was to isolate a strain of LAB tolerable to the toxic effect of SMB. In Exp. 2, the SMB load was minimized through its partial replacement with a chemical mixture (CM) based on sodium benzoate (57%), potassium sorbate (29%), and sodium nitrite (14%). FVD was treated with SMB + CM (2 g each/kg biomass) and added to the TMR at varying levels (0%, 10%, or 20%), with or without KU18 inoculation. Results: The KU18 was screened as a presumptive LAB strain showing superior tolerance to SMB in broth medium, and was identified at the molecular level using 16S rRNA gene sequence analysis as Lactobacillus plantarum. Inoculation of KU18 in TMR containing SMB was not successful for the LAB development, biomass acidification, and organoleptic properties of the resultant silage. In Exp. 2, based on the effectiveness and economic considerations, an equal proportion of SMB and CM (2 g each/kg FVD) was selected as the optimal loads for the subsequent silage fermentation experiment. Slight differences were determined in LAB development, biomass acidification, and sensorial characteristics among the experimental silages, suggesting the low toxicity of the preservatives on LAB growth. Conclusion: Although KU18 strain was not able to efficiently develop in silage mass containing SMB-treated FVD, the partial substitution of SMB load with the CM effectively alleviated the toxic effect of SMB and allowed LAB development during the fermentation of SMB + CM-treated FVD in TMR.

• Molecules and Cells
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• 제42권9호
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• pp.637-645
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• 2019

Effector-triggered immunity (ETI) is an effective layer of plant defense initiated upon recognition of avirulence (Avr) effectors from pathogens by cognate plant disease resistance (R) proteins. In rice, a large number of R genes have been characterized from various cultivars and have greatly contributed to breeding programs to improve resistance against the rice blast pathogen Magnaporthe oryzae. The extreme diversity of R gene repertoires is thought to be a result of co-evolutionary history between rice and its pathogens including M. oryzae. Here we show that Pii is an allele of Pi5 by DNA sequence characterization and complementation analysis. Pii-1 and Pii-2 cDNAs were cloned by reverse transcription polymerase chain reaction from the Pii-carrying cultivar Fujisaka5. The complementation test in susceptible rice cultivar Dongjin demonstrated that the rice blast resistance mediated by Pii, similar to Pi5, requires the presence of two nucleotide-binding leucine-rich repeat genes, Pii-1 and Pii-2. Consistent with our hypothesis that Pi5 and Pii are functionally indistinguishable, the replacement of Pii-1 by Pi5-1 and Pii-2 by Pi5-2, respectively, does not change the level of disease resistance to M. oryzae carrying AVR-Pii. Surprisingly, Exo70F3, required for Pii-mediated resistance, is dispensable for Pi5-mediated resistance. Based on our results, despite similarities observed between Pi5 and Pii, we hypothesize that Pi5 and Pii pairs require partially distinct mechanisms to function.

• Annals of Clinical Neurophysiology
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• 제21권2호
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• pp.79-86
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• 2019

Background: Magnetic resonance (MR) images are useful for diagnosing myopathy. The purpose of this study was to determine the usefulness of lower-limb MR images in Korean patients with distal myopathy. Methods: We reviewed medical records in the myopathy database from January 2002 to October 2016. We selected 21 patients from 91 unrelated families with distal myopathy: four with GNE myopathy, 11 with dysferlinopathy, and six with ADSSL1 myopathy. Results: Ten (48%) of the 21 patients were men. The ages of the participants at symptom onset and imaging were $19.2{\pm}9.5$ and $30.4{\pm}9.0$ years (mean${\pm}$standard deviation), respectively. Their grade on the modified Gardner-Medwin and Walton grade was $3.3{\pm}1.7$. The strength grade of the knee extensors was not correlated with the Mercuri scale for the quadriceps (r = -0.247, p = 0.115). However, the Medical Research Council grades of the knee flexors, ankle dorsiflexors, and ankle plantar flexors were significantly correlated with the Mercuri scale ratings of the knee flexors (r = -0.497, p = 0.001), tibialis anterior (r = -0.727, p < 0.001), and ankle plantar flexors (r = -0.620, p < 0.001), respectively. T1-weighted MR images showed characteristic fatty replacement patterns that were consistent with the causative genes. Unsupervised hierarchical clustering of the Mercuri scale showed that the main factors contributing to the dichotomy were the causative gene and the clinical severity. Conclusions: This study is the first to reveal the usefulness of lower-limb MR images in the differential diagnosis of distal myopathy in Korea.

• 한국수산과학회지
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• 제53권6호
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• pp.900-908
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• 2020

This study was conducted to partially replace fish meal with black soldier fly (BSF) larvae or mealworm (MW) larvae in the diet for Pacific white shrimp Litopenaeus vannamei. A tuna by-product meal (27%) was used as the protein source in a control (Con) diet, which was replaced with 20%, 40%, or 60% BSF (designated as BSF20, BSF40, and BSF60, respectively), or MW (MW20, MW40, and MW60, respectively). The shrimp (average body weight, 0.09 g) were randomly stocked in 28 acrylic tanks and quadruplicate groups were fed one of the experimental diets six times daily. After 57 days of the feeding trial, growth and feed efficiency were significantly higher in shrimp that were fed the BSF40 and BSF60 diets than those in shrimp fed the Con diet. Phenoloxidase, superoxide dismutase, glutathione peroxidase and gene expression of crustin were significantly higher in shrimp fed BSF or MW diets than those in shrimp fed the Con diet. The results indicate that BSF or MW could be used as a fish meal replacement or as a functional protein source in diets and can help improve the growth, feed utilization, innate immunity and antioxidant capacity of Pacific white shrimp.

• 한국버섯학회지
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• 제12권1호
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• pp.41-51
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• 2014

${\beta}$-glucan 함량이 높다고 알려진 꽃송이버섯의 농가재배 활성화를 위하여 푸른곰팡이 내성균주를 선발하고자 푸른곰팡이 대치배양에 의한 꽃송이버섯 균사생장 특성을 확인하였으며, 또한 생장이 우수한 신품종을 개발하고자 교잡육종 균주에 대한 유전 다양성을 분석하였다. 먼저 푸른곰팡이 대치배양에 의한 꽃송이버섯 균사의 생장 특성을 확인한 결과, 6951 (T. viride) 균주에서는 대치선을 형성한 후 별다른 변화를 보이지 않았고, 6952 (T. spp.) 균주에서는 대치선을 형성한 다음 보다 많은 포자를 형성하는 것이 관찰되었다. 그러나 6426 (T. harzianum) 균주에서는 꽃송이버섯 균사가 생장하고 있던 부분까지 모두 덮어버리는 것이 확인되었다. 그 중 특이하게도 구례에서 채집선발한 균주인 JF02-06 균주에서는 다른 균주에 비해 푸른곰팡이 포자가 형성되지 않는 것을 확인되어 다소 푸른곰팡이에 대한 저항성을 갖는 것으로 사료되었다. 전남 산림자원연구소에서 보유 중인 균주 중 생장 및 자실체 발생이 우수한 모균주를 선발하여 교잡을 실시하여 생장특성을 조사한 결과, 미송톱밥배지에서 JF02-47, 49, 50 균주의 균사생장량이 우수한 것으로 확인되었다. 이러한 교잡육종 균주의 유전 다양성을 분석하기 위하여 ITS1, 5.8S와 ITS4 영역에 대한 염기서열을 분석한 결과 Genebank에 등록된 다른 꽃송이버섯 균주와 높은 유의성을 갖는 것으로 확인되었다. 이러한 꽃송이버섯의 포자 및 균사를 현미경으로 관찰하여 생장 특성을 확인한 결과, 포자의 크기는 장경 $6{\mu}m$, 단경 $5{\mu}m$의 물방울 모양으로 확인되었고, 균사에서 3가지 형태의 꺽쇠가 관찰되었다. 균사의 폭은 $3{\mu}m$이며 꽃송이버섯 균사의 특징으로는 약 50% 정도 꺽쇠에서 균사가 뻗어나가는 특성을 갖고 있음이 확인되었다. 균사의 생장 속도는 $0.507{\mu}m/min$이며, 2차 균사는 $0.082{\mu}m/min$의 속도로 생장하다가 모균사와 평행을 이루는 시점에서는 모균사의 생장속도와 유사한 속도로 생장하였다. 꺽쇠발생은 약 5시간 동안 균사 내부 전해질의 이동이 관찰된 후 작은 꺽쇠를 형성하였다. 약 3시간 후 격막이 형성되기 시작하였으며, 그로부터 2시간 후 최종적으로 완성되었다. 이러한 특성을 갖는 꽃송이버섯의 푸른곰팡이 저항성을 확인하고, 교잡균주의 유전 다양성 및 균사의 생장 특성을 확인하여 꽃송이버섯에 대한 기초적인 이해를 높이고, 더 나아가 버섯산업 발전에 이바지하고자 한다.