• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.58-63
• /
• 2012

Classical galactosemia (OMIM# 230400) is an autosomal recessive disorder of carbohydrate metabolism, due to a complete loss in galactose-1-phosphate uridyltransferase (GALT; E.C.2.7.7.12) enzyme activity. It caused by mutations in the GALT gene (OMIM$^*$ 606999) that is located at chromosome 9p13. The GALT enzyme deficiency results in a build-up of galactose and galactose-1-phosphate, causing life threatening complications such as feeding problems, failure to thrive, hepatocellular damage, bleeding and sepsis. However, Duarte galactosemia, a variant form of GALT deficiency, has residual GALT enzyme activities in erythrocytes and do not have manifest the symptoms of classical galactosemia. Since the advent of newborn screening (NBS) for galactosemia, we rarely encounter such overwhelmingly ill newborns. The positive NBS with no symptoms indicates the possibility of Duarte galactosemia besides a simple false positive and it has to be differentiated from classical galactosemia which is a medical emergency. In Korea, detection rate of Duarte galactosemia is very low and its genetic information is restrictive, too. We report a case of monozygotic twins with D/G galactosemia compound heterozygote in proven by the mutational analysis of GALT gene, which revealed N314D polymorphism and -119 to -116 delGTCA.

• Korean Journal of Food Science and Technology
• /
• v.21 no.1
• /
• pp.136-144
• /
• 1989

The purification of myrosinase from radish roots was performed using Concannavalin A-Sepharose affinity chromatography and gel permeation HPLC, which gave a 22-fold-purification(S.P.A.=39,000 units/mg) with 50% recovery, SDS-polyacrylamide gel electrophoresis showed a single major band, suggestive of a relatively pure myrosinase, and the M.W. of the enzyme determined on gel permeation HPLC was ca. 124K. The enzyme showed on optimum pH of 6.5 and was stable at pH 6 to 7 at room temperature, but unstable below pH 4. The enzyme possessed an optimum temperature of $37^{\circ}C$, and gave a Vmax value of $40\;{\mu}moles/mg{\cdot}min$ and a Km value of 0.12mM for sinigrin. The purified myrosinase was activated maximally by 0.6mM of ascorbic acid, but somewhat inhibited by more than 2 mM ascorbic acid. The activities of myrosinase present in the peelings and the peeled radish amounted to approximately 1,333 units/g and 140 units/g weight, respectively and the peelings contained much more myrosinase activity than the peeled radish.

• Korean Journal of Food Science and Technology
• /
• v.16 no.2
• /
• pp.235-241
• /
• 1984

Two fractions of pectinesterase from Chinese cabbage were isolated by ammonium sulfate fractionation, ion exchange chromatography on DEAE-cellulose and Sephadex G-150 gel filtration. The fraction F-A and F-B were purified approximately 340- and 10-fold. The similar salt effects and pH optima (pH 7.5-8.0) were obtained for the two pectinesterase fractions. The maximum activity of both two. fractions were obtained at 20-50mM of divalent rations and at 250mM of monovalent rations. The apparent Michaelis constant of the F-A was 0.01% for citrus pectin. The temperature optima for F-A and F-B were $48^{\circ}$ and $55^{\circ}C$, respectively and both fractions were stable in the region of pH 5.0-8.0 at room temperature. The thermal inactivation of the two fractions followed the first order reaction kinetics. From D and Z-values obtained the thermal resistance of the two fractions were characterized.

• Food Science and Preservation
• /
• v.11 no.1
• /
• pp.106-110
• /
• 2004

The objective of this study was carried out to investigate nutritional characteristics and biological activities effects of Korean leaf of Rosa davurica Pall. in vitro. They were extracted with methanol and then further fractionated to n-hexane, chloroform, ethyl-acetate, n-butanol and water from methanol extracts. Methods of the antigenotoxic used in this experiment were UVA/UVB absorption property and DPPH radical scavenge. The proximate compositions of leaves of Rosa dauvrica Pall were 67.5％ of crude Moisture, 0.7％ of crude fat, 6.8％ of crude protein, 6.1％ of crude ash, and 20.8％ of crude fiber. The major minerals were K (1637.2 mg％), Ca (219.5 mg％), P (182.1 mg％), and Mg (135.1 mg％). Most of the fractions of methanol extract which leaves of Rosa dauvrica Pall. have strong absorbency at UVB region (308 nm) and UV A region (350nm). These fractions have a good absorbency property as synthetic filter and could be served as substitutes for synthetic UV sunscreen agents. All fractions (n-hexane, ethyl-acetate, n-butanol and water) from methanol extracts except chloroform fraction exhibited DPPH radical scavenging activity with IC$\_$50/ of 35.3, 6.0, 14.0, and 18.0 $\mu\textrm{g}$/mL.

• Food Science and Preservation
• /
• v.19 no.4
• /
• pp.594-603
• /
• 2012

This study developed a high-utility type of vinegar from Rubus coreanus by optimizing its fermentation conditions. In the alcohol fermentation process, the optimal conditions for the maximization of the alcohol contents were an initial sugar concentration of 15 $^{\circ}Brix$, a temperature of $30^{\circ}C$ and 4 days. The optimal conditions for the acetic acid fermentation were 9 days of fermentation at $30^{\circ}C$ and 200 rpm, with 6% alcohol and 2% initial acidity. The sucrose, fructose, and glucose contents were 952.90, 491.01, and 386.62 mg%, respectively. The free organic acids were acetic, malic, succinic, malonic, oxalic, and lactic acids. The total free amino acid content was 104.33 ${\mu}g/mL$, with alanine, glutamic acid, ${\gamma}$-amino-N-butyric acid, and o-phospho-ethanolamine as the major amino acids. The K, Na, and Mg contents were 1,686.10, 172.50, and 69.33 ppm, respectively. The total phenolic and anthocyanin contents were 25.19 and 80.71 mg/100 mL, respectively. The DPPH- and $ABTS^{.+}$ radical scavenging activities were approximately 65 and 94%, respectively. Moreover, the vinegar's ${\beta}$-carotene bleaching activity and reducing power showed that it had strong anti-oxidant properties. These results show that Rubus coreanus vinegar has anti-oxidant properties and may be used as functional food.

• Journal of Physiology & Pathology in Korean Medicine
• /
• v.35 no.2
• /
• pp.56-63
• /
• 2021

This study was conducted to find a candidate prescription with anti-inflammatory efficacy of 7 herbal prescriptions known to be effective in atopic dermatitis and sleep disorders in Korean medicine. The anti-inflammatory of the 7 herbal prescriptions extracts were evaluated by ELISA assay and Western blot assay. 7 herbal prescriptions, Seongyutang(SU), Danseontoetang(DST), Sotosajahwan(ST), Jisiljagyagsan(JJ), Seokchangpo(SCP), Wiryeongseon(WLS), Gogojohwan(GGJ) 30 % EtoH extract was used for in vitro experiments. In TNF-α + IFN-γ(TI) stimulated HaCaT cells, all 7 herbal prescriptions reduced TARC and MDC production, and JJ strongly reduced TARC and MDC production at 100 ㎍/ml concentration. SCP strongly reduced MDC production at 10 ㎍/ml and 100 ㎍/ml concentration. In addition, in substance P(SP)/CRH stimulated HMC-1 cells, JJ strongly inhibited VEGF production at both 10 and 100 ㎍/ml concentrations. In LPS/CRH stimulated Raw264.7 cells, all 7 herbal prescriptions significantly inhibited TNF-α. PMA + Ionomycin(PI)/CRH stimulated EL4 cells, SU significantly reduced IL-4 production at both concentrations of 10 and 100 ㎍/ml. WLS significantly reduced IL-17 production at both concentrations of 10 and 100㎍/ml. This suggests that 7 herbal prescriptions have anti-inflammatory effects by reducing inflammatory cytokines in keratinocytes, mast cells and macrophages caused by inflammation. Therefore, it is expected that 7 herbal prescriptions that are effective for sleep disorders and atopic dermatitis can be used as therapeutic materials for sleep disorders and diseases associated with atopic dermatitis.

• Molecules and Cells
• /
• v.44 no.1
• /
• pp.26-37
• /
• 2021

Human papillomaviruses (HPVs) cause cellular hyperproliferation-associated abnormalities including cervical cancer. The HPV genome encodes two major viral oncoproteins, E6 and E7, which recruit various host proteins by direct interaction for proteasomal degradation. Recently, we reported the structure of HPV18 E7 conserved region 3 (CR3) bound to the protein tyrosine phosphatase (PTP) domain of PTPN14, a well-defined tumor suppressor, and found that this intermolecular interaction plays a key role in E7-driven transformation and tumorigenesis. In this study, we carried out a molecular analysis of the interaction between CR3 of HPV18 E7 and the PTP domain of PTPN21, a PTP protein that shares high sequence homology with PTPN14 but is putatively oncogenic rather than tumor-suppressive. Through the combined use of biochemical tools, we verified that HPV18 E7 and PTPN21 form a 2:2 complex, with a dissociation constant of 5 nM and a nearly identical binding manner with the HPV18 E7 and PTPN14 complex. Nevertheless, despite the structural similarities, the biological consequences of the E7 interaction were found to differ between the two PTP proteins. Unlike PTPN14, PTPN21 did not appear to be subjected to proteasomal degradation in HPV18-positive HeLa cervical cancer cells. Moreover, knockdown of PTPN21 led to retardation of the migration/invasion of HeLa cells and HPV18 E7-expressing HaCaT keratinocytes, which reflects its protumor activity. In conclusion, the associations of the viral oncoprotein E7 with PTPN14 and PTPN21 are similar at the molecular level but play different physiological roles.

• Journal of Veterinary Science
• /
• v.23 no.1
• /
• pp.16.1-16.13
• /
• 2022

Background: Xylazole (Xyl) is a veterinary anesthetic that is structurally and functionally similar to xylazine. However, the effects of Xyl in vitro remain unknown. Objectives: This study aimed to investigate the anesthetic mechanism of Xyl using fetal rat nerve cells treated with Xyl. Methods: Fetal rat nerve cells cultured for seven days were treated with 10, 20, 30, and 40 ㎍/ mL Xyl for 0, 5, 10, 15, 20, 25, 30, 45, 60, 90, and 120 min. Variations of amino acid neurotransmitters (AANTs), Nitric oxide-Cyclic GMP (NO-cGMP) signaling pathway, and ATPase were evaluated. Results: Xyl decreased the levels of cGMP and NO in nerve cells. Furthermore, Xyl affected the AANT content and Na⁺-K⁺-ATPase and Ca²⁺-Mg²⁺-ATPase activity in nerve cells. These findings suggested that Xyl inhibited the NO-cGMP signaling pathway in nerve cells in vitro. Conclusions: This study provided new evidence that the anesthetic and analgesic effects of Xyl are related to the inhibition of the NO-cGMP signaling pathway.

• Korean journal of food and cookery science
• /
• v.21 no.5
• /
• pp.725-732
• /
• 2005

This study attempted to investigate if the soy isoflavone, genistein, influences bone metabolism in ovariectomized, 4-week-old female Wister rats. All the rats were divided into sham (SH) and ovariectomized (OVX) groups consisting of OVX-17${\beta}$-estradiol($10\;{\mu}g/kg$ b.w.), OVX-1mg or 5 mg or 10mg of genistein/kg b.w. The rates were allowed ad libitum access to foods and water for 8 weeks. The Results showed that body weight had significantly increased in the OVX group compared to the SH group (p<0.05) and was not different among the OVX-GEN and OVX-ES groups and the OVX group. The liver and uterus weights in the OVX groups were slighter than those in SH group (p<0.05). The kidney weight in the OVX groups was decreased compared to in that in the SH group but was not significantly different among all OVX groups. Femoral length in the OVX groups was longer than in the SH group and was not different. Rats in the OVX groups had higher creatinine levels than those in the SH group and hydroxyproline level did not differ significantly among any of the groups. Serum ALP activity and Ca in bone, feces, urine and serum did not change among the groups. Bone mineral density (BMD) decreased in the OVX groups compared to the SH group and was slightly increased by feeding genistein (p>0.05). Breaking force and stiffness did not change by ovariectomy and feeding genistein. Hence, these results suggested that estrogen may affect bone mineralization in growing rats and that genistein be may involved in the prevention of bone loss. However, more studies are needed to identify the proper mechanism of genistein and bone formation.

• KOREAN JOURNAL OF CROP SCIENCE
• /
• v.62 no.3
• /
• pp.203-213
• /
• 2017

This study investigated the changes in yields, antioxidant compounds, and antioxidant activities among 11 adzuki bean cultivars cultivated in a paddy field with somewhat poorly drained soil. The adzuki bean cultivars were cultivated in the paddy field from 2015 to 2016 in Milyang, Gyeongsangnam, Korea. Average soil moisture content was 16.5% in 2015 and 31.0% in 2016 at the experimental site during adzuki bean cultivation. As the soil moisture content increased, most of the adzuki bean cultivars showed deceases in stem height, first setting pod node, number of pods, 100 seed weight, and yield. Chungju-pat produced the greatest yields among the 11 cultivars in 2015 and 2016, whereas Hongeon had the smallest yields. Adzuki bean yields after paddy field cultivation was lower for all cultivars than for adzuki bean yields from the same cultivars after upland field cultivation. Chungju-pat and Chilbo-pat showed the smallest decreases in yields after paddy field cultivation, whereas Hongeon and Kumsil showed the greatest yield reductions. There were no significant differences in proximate composition. Some mineral components ($P_2O_5$, Ca, and Mg) were statistically different across cultivars. Chungju-pat had the highest Ca and Mg contents, but antioxidant components (polyphenol and flavonoids) and antioxidant activities (ABTS and DPPH) were highest in Saegil and lowest in Jungbu-pat. PCA and clustering analyses, based on the growth, yield, and antioxidant component measurements, performed to identify which variables contributed the most to separating adzuki bean cultivars or to grouping cultivars with similar characteristics. These analyses showed that the antioxidant components and antioxidant activities had the most influence on grouping cultivars together. Among the 11 cultivars, Saegil was statistically different from the other cultivars, but the other 10 cultivars were not significantly different under paddy field cultivation. Soil moisture content affected adzuki bean yield and antioxidant component contents. An increase in soil moisture led to a decrease in yield, but an increase in antioxidant components. These results provide information that will improve the selection of an appropriate adzuki bean cultivar for use in paddy fields.