• 대한유전성대사질환학회지
• /
• 제12권1호
• /
• pp.58-63
• /
• 2012

고전적 갈락토오스혈증(classical galactosemia; OMIM #230400)은 상염색체 열성 유전의 갈락토오스 대사장애로, 9번 염색체에 위치하는 GALT 유전자(OMIM *606999)로부터 전사되는 galactose-1-phosphate uridylyltransferase (GALT; E.C.2.7.7.12)의 심각한 결손으로 인해 유발되는 질환이다. GALT의 결함은 galactose-1-phosphate의 체내 축적을 일으켜, 신생아 시기부터 구토, 수유 곤란, 황달, 복수, 경련 발작, 기면 상태 등의 증상을 유발하고, 장기적으로는 백내장, 성장지연, 지능저하를 초래한다. 반면 Duarte형 갈락토오스혈증은 적혈구에서의 GALT 효소 활성도가 감소되어 있어 혈중 galactose와 galactose-1-phosphate의 농도가 증가하지만 임상적으로는 거의 증상을 보이지 않는 아형이다. 최근 신생아 선별검사의 발달과 함께, 무증상의 양성 판정 환아들이 늘고 있으며, 이들 환아들이 Duarte형 갈락토오스혈증일 가능성이 제기되고 있다. 이에 저자들은 국내에서 그 동안 드물게 보고되었던 N314D와 -119_-116delGT CA/E363K이형접합체의 Duarte형 갈락토오스혈증 일란성 쌍둥이 1례를 보고하고자 한다.

• 한국수산과학회지
• /
• 제11권2호
• /
• pp.55-64
• /
• 1978

The present investigations were carried out for the purpose of making clear the fundamental features of the seasonal variations in carrageenan content and its chemical composition. The samples, Chondrus ocellatus, were collected every month from the same locality on the coast of Haewundae from March, 1975 to February, 1976, and analyzed monthly to determine their carrageenan content, sulphate and 3,6-anhydro-D-galactose over a year period. En addition, some chemical characteristics were also tested on the fractions separated by the different concentrations of potassium chloride. In seasonal variation, the maximum carrageenan content occurred from summer through autumn, and the minimum in winter, especially in February. It is noted in the seasonal variations of the sulphate content of total carrageenan and three fractions that the maximums appreared in October and minimums in february. With seasonal variations of percent 3,6-anhydro-D-galactose of total carrageenan and three fractions, maximum values occur in June and minimum values in December. Seasonal variations in sulphate and 3,6-anhydro-D-galactose contents of the three fractions were on the whole similar in mode of variation, and there could be no substancial differences. Seeing seasonal variations in the sulphate and 3,6-anhydro-D-galactose contents of three fractions, carrageenan extracted from the algae gathered in spring shelved higher portion of fraction I than that gathered in autumn. In these respects, it seems that a more weakly gelling k-carrageenan exists in the spring than in the autumn.

• 대한한방내과학회지
• /
• 제25권4호
• /
• pp.129-139
• /
• 2004

Objectives : This study was done to examine the antioxidant actions of free radicals caused by Banryong-hwan in blood.. Methods : Twelve week-old SD rats were divied into normal group, control group and HTG group. Control and HTG groups were age-induced with D-galactose, and extract of Banryong-hwan(BRH) was administerd to BRH group for six weeks. After then, blood was taken, and activities of SOD and GSH-px in erythrocytes were measured, as well as TBARS levels and concentrations of total lipid tryglyceride in plasma. Results : 1. The activities of SOD and GSH-px in erythrocytes were significantly increased in the BRH group compared with control group. 2. The concentration of total lipid was significantly decreased in the BRH group compared with control group. The values of TBARS and the concentrations of tryglyceride in plasma showed a tendency to decrease but they were not remarkable. Conclusions : Judging from the above findings, it is suggested that Banryong-hwan decrease the activities of free radical, the concentrations of lipid in plasma and generate enzyme which form lipid peroxide.

• International Journal of Industrial Entomology and Biomaterials
• /
• 제23권2호
• /
• pp.201-206
• /
• 2011

Using proteases, we produced a peptide mixture from fibroin of the silkworm $Bombyx$ $mori$. Mice received D-galactose by subcutaneous injection for 8 weeks to accelerate aging, and then received the peptide mixture orally for 7 weeks. In the mice aged with D-galactose, the coefficient of friction of hair increased significantly up to 1.6-fold, but in the mice subsequently given the peptide mixture, it was normal. Scanning electron microscopy showed improved hair cuticles in the latter mice too. These results indicate that oral administration with peptides from $Bombyx$ fibroin counters the aging of hair cuticles.

• 대한의용생체공학회:학술대회논문집
• /
• 대한의용생체공학회 1996년도 춘계학술대회
• /
• pp.123-125
• /
• 1996

The orientation effect of galactose ligand on hepatocyte attachment was investigated. Poly(N-p-vinyl benzyl-o-${\beta}$-D-galactopyranosyl-D-gluconamide) (PVLA), a ${\beta}$-galactose-carrying styrene homo-polymer, was used as a model ligand for the asialoglycoprotein receptors on hepatocytes. PYVA was transferred onto the poly(${\gamma}$-benzyl L-glutamate)(PBLG) or PBLG/ poly(ethylene glycol)(PEG)/PBLG Langmuir-Blodgett (LB) films as the monolayer level. The dichroic fluorescence values of confocal microscope indicated that the PVLA transferred onto the LB films was located with a preferential orientation of its molecular axes with regard to the direction of the a-helix of polypeptide. Hepatocyte recognized well-oriented galactose moieties of the surface of PVLA through asialoglycoprotein receptors.

• Journal of Microbiology and Biotechnology
• /
• 제3권3호
• /
• pp.168-173
• /
• 1993

The expression kinetics of human lipocortin I (LCI), a potential anti-inflammatory agent, was studied in the shake-flask and fermenter cultures of Saccharomyces cerevisiae carrying a galactose-inducible expression system. The cell growth, expression level of LCI, and the plasmid stability were investigted under various galactose induction conditions. The expression of LCI was repressed by the presence of a very small amount of dextrose in the culture medium, but it was induced by galactose after dextrose became completely depleted. The optimal ratio of dextrose to galactose for lipocortin I production was found to be 1.0 (10 g/l dextrose and 10 g/l galactose). With optimal D/G ratio of 1.0 and the addition of galactose prior to dextrose depletion, LCI of about 100~130 mg/l was produced. LCI at a concentration of 174 mg/l was porduced in the fed-batch culture, which was nearly a twice as much of that produced in the batch culture. The plasmid stability was very high in all culture cases, and thus was considered to be not an important parameter in the expression of LCI.

• Nutrition Research and Practice
• /
• 제14권3호
• /
• pp.188-202
• /
• 2020

BACKGROUND/OBJECTIVES: Brain aging is a major risk factor for severe neurodegenerative diseases. Conversely, L-histidine and L-carnosine are known to exhibit neuroprotective effects. The aim of this study was to examine the potential for L-histidine, L-carnosine, and their combination to mediate anti-brain aging effects in neuronal cells subjected to D-galactose-induced aging. MATERIALS/METHODS: The neuroprotective potential of L-histidine, L-carnosine, and their combination was examined in a retinoic acid-induced neuronal differentiated SH-SY5Y cell line exposed to D-galactose (200 mM) for 48 h. Neuronal cell proliferation, differentiation, and expression of anti-oxidant enzymes and apoptosis markers were subsequently evaluated. RESULTS: Treatment with L-histidine (1 mM), L-carnosine (10 mM), or both for 48 h efficiently improved the proliferation, neurogenesis, and senescence of D-galactose-treated SH-SY5Y cells. In addition, protein expression levels of both neuronal markers (β tubulin-III and neurofilament heavy protein) and anti-oxidant enzymes, glutathione peroxidase-1 and superoxide dismutase-1 were up-regulated. Conversely, protein expression levels of amyloid β (1-42) and cleaved caspase-3 were down-regulated. Levels of mRNA for the pro-inflammatory cytokines, interleukin (IL)-8, IL-1β, and tumor necrosis factor-α were also down-regulated. CONCLUSIONS: To the best of our knowledge, we provide the first evidence that L-histidine, L-carnosine, and their combination mediate anti-aging effects in a neuronal cell line subjected to D-galactose-induced aging. These results suggest the potential benefits of L-histidine and L-carnosine as anti-brain aging agents and they support further research of these amino acid molecules.

• Biomolecules & Therapeutics
• /
• 제19권2호
• /
• pp.224-230
• /
• 2011

In order to develop silk peptide (SP) preparations possessing cognition-enhancing effect, several candidates were screened through in vitro assays, and their effectiveness was investigated in facilitated brain aging model rats. Incubation of brain acetyl-cholinesterase with SP-PN (1-1,000 ${\mu}g/ml$) led to inhibition of the enzyme activity up to 35%, in contrast to a negligible effect of SP-NN. The expression of choline acetyltransferase (ChAT) mRNA of neural stem cells expressing ChAT gene (F3.ChAT) was increased by 24-hour treatment with 10 and 100 ${\mu}g/ml$ SP-NN (1.35 and 2.20 folds) and SP-PN (2.40 and 1.34 folds). Four-week subcutaneous injections with D-galactose (150 mg/kg) increased activated hippocampal astrocytes to 1.7 folds (a marker of brain injury and aging), decreased acetylcholine concentration in cerebrospinal fluid by 45-50%, and thereby impaired learning and memory function in passive avoidance and water-maze performances. Oral treatment with SP preparations (50 or 300 mg/kg) for 5 weeks from 1 week prior to D-galactose injection exerted recovering activities on acetylcholine depletion and brain injury/aging as well as cognitive deficit induced by D-galactose. The results indicate that SP preparations restore cognitive functions of facilitated brain aging model rats by increasing the release of acetylcholine, in addition to neuroprotective activity.

• 대한의생명과학회지
• /
• 제27권4호
• /
• pp.323-328
• /
• 2021

Naegleria fowleri is a free-living amoeba causing primary amoebic encephalitis. In this study, we analyzed how the N-aceytlglucosamine (GlcNAc) and D-galactose affected the interaction between Naegleria fowleri and methicillin-resistant Staphylococcus aureus (MRSA) or Escherichia coli O157:H7, and the interaction with bacteria when monosaccharides were treated with N. fowleri for a longer pre-incubation time. When GlcNAc was treated with N. fowleri for 1 hr, the E. coli association was almost the same as that of the control not treated with GlcNAc until the concentration of GlcNAc was 25 mM. However, the E. coli association was reduced by approximately 91% with 100 mM GlcNAc. E. coli invasion into N. fowleri showed statistical significance only in the group treated with 100 mM GlcNAc. The interaction when treated with galactose showed a very different pattern in the 50 mM galactose group than when treated with GlcNAc. In the MRSA interaction, a statistically significant decrease in association (76.3% by GlcNAc and 88.7% by galactose) and invasion (3.6% by GlcNAc and 9.3% by galactose) was shown by the concentration of two 100 mM monosaccharides. The group treated with monosaccharides at the same time showed almost no difference in all interactions from the group treated with monosaccharides at the same time. Taken together, it suggested that the effect of monosaccharides on the interaction of several Gram-negative or positive bacteria and the evidence that the interaction could be enhanced by longer pre-incubation time.

• 한국식품과학회지
• /
• 제21권1호
• /
• pp.164-172
• /
• 1989

Streptococcus thermophilus 510의 ${\beta}-galactosidase$는 유당을 가수분해 하는 동시에 galactose전이반응을 일으켜 세 종류의 galactooligosaccharides를 형성 하였다. Oligosaccharide의 최대 생성 조건은 40% 유당 용액에 효소 50 ONPG units/ml를 첨가한 혼합액을 $37^{\circ}C$에서 4시간 반응시켰을 때 이며, 이 조건에서 유당이 약 94% 가수분해 되었고 생성된 oligosaccharides의 양은 총 당의 약 30% 이었다. 생성된 총 oligosaccharides의 69%는 $6-o-{\beta}-D-galactopyranosyl-D-glucose(allolactose)$, 23%는 $6-o-{\beta}-D-galactopyranosyl-D-galactose(isogalactobiose)$ 이었으며, 형성된 galactooligosaccharides을 Bio-Gel P-2 gel permeation chromatography 방법에 의해서 순수하게 분리 정제하여 구조를 조사하여 본 결과 glucose, galactose, allolactose, 그리고 isogalactobiose 임을 확인할 수 있었다.