• Journal of Genetic Medicine
• /
• v.4 no.1
• /
• pp.72-79
• /
• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• The Korean Journal of Nuclear Medicine
• /
• v.36 no.6
• /
• pp.317-324
• /
• 2002

Purpose: The use of statistical parametric mapping (SPM) program has increased for the analysis of brain PET and SPECT images. Montreal Neurological Institute (MNI) coordinate is used in SPM program as a standard anatomical framework. While the most researchers look up Talairach atlas to report the localization of the activations detected in SPM program, there is significant disparity between MNI templates and Talairach atlas. That disparity between Talairach and MNI coordinates makes the interpretation of SPM result time consuming, subjective and inaccurate. The purpose of this study was to develop a program to provide objective anatomical information of each x-y-z position in ICBM coordinate. Materials and Methods: Program was designed to provide the anatomical information for the given x-y-z position in MNI coordinate based on the Statistical Probabilistic Anatomical Map (SPAM) images of ICBM. When x-y-z position was given to the program, names of the anatomical structures with non-zero probability and the probabilities that the given position belongs to the structures were tabulated. The program was coded using IDL and JAVA language for 4he easy transplantation to any operating system or platform. Utility of this program was shown by comparing the results of this program to those of SPM program. Preliminary validation study was peformed by applying this program to the analysis of PET brain activation study of human memory in which the anatomical information on the activated areas are previously known. Results: Real time retrieval of probabilistic information with 1 mm spatial resolution was archived using the programs. Validation study showed the relevance of this program: probability that the activated area for memory belonged to hippocampal formation was more than 80%. Conclusion: These programs will be useful for the result interpretation of the image analysis peformed on MNI coordinate, as done in SPM program.

• The Korean Journal of Nuclear Medicine
• /
• v.36 no.6
• /
• pp.381-391
• /
• 2002

Purpose: The presence of perfusion defect may influence the left ventricular mass (LVM) measurement by quantitative gated myocardial perfusion SPECT (QGS), and ischemic myocardium, usually showing perfusion defect may produce post-stress LV dysfunction. This study was aimed to evaluate the effects of extent and reversibility of perfusion defect on the automatic measurement of LVM by QGS and to investigate the effect of reversibility of perfusion defect on post-stress LV dysfunction. Subjects and Methods: Forty-six patients (male/female=34:12, mean age=64years) with perfusion defect on myocardial perfusion SPECT underwent rest and post-stress QGS. Forty patients (87%) showed reversible defect. End-diastolic volume (EDV), end-systolic volume (ESV), LV ejection fraction (EF), and LV myocardial volume were obtained from QGS by AutoQUANT program, and LVM was calculated by multiplying the LV myocardial volume by the specific gravity of myocardium. Results: LVMs measured at rest and post-stress QGS showed good correlation, and higher correlation was founded in the subjects with fixed perfusion defect and with small defect (smaller than 20%). There were no significant differences in EDVs, ESVs and EFs between obtained by rest and post-stress QGS un patients with fixed myocardial defect. Whereas, EF obtained by post-stress QGS was lower than that by rest QGS in patients with reversible defect and 10 (25%) of them showed decreases in EF more than 5% in post-stress QGS, as compared to that of rest QGS. Excellent correlations of EDVs, ESVs, EFs between rest and post-stress QGS were noted. Patients with fixed defect had higher correlation between EDVs, ESVs, EFs than patients with reversible defect. Conclusion: These results suggest that perfusion defect can affect LVM measurement by QGS and patients with reversible defect shows post-stress LV dysfunction more frequently than patients with fixed perfusion defect.

• The Korean Journal of Nuclear Medicine
• /
• v.34 no.4
• /
• pp.303-311
• /
• 2000

Purpose: Thallium behaves similarly to potassium in vivo. Potassium channel opener (K-opener) opens ATP-sensitive $K^+$-channel located at cell membrane, resulting in potassium efflux from cytosol. We have previously reported that K-opener can alter biokinetics of Tl-201 in cultured cells and in vivo. Malignant tumor cells have high Na-K ATPase activity due to increased metabolic activities and dedifferentiation, and differential delineation of malignant tumor can be possible with Tl-201 imaging. K-opener may affect tumoral uptake of Tl-201 in vivo. To investigate the effects of pinacidil (one of the potent K-openers) on the localization of the tumor with Tl-201 chloride, we evaluated the changes in biodistribution of Tl-201 with pinacidil treatment in tumor-bearing mice. Materials and Methods: Baltic mice received subcutaneous implantation of murine breast cancer cells in the thigh and were used for biodistribution study 3 weeks later. $100{\mu}g$ of pinacidil dissolved in $200{\mu}l$ DMSO/PBS solution was injected intravenously via tail vein at 10 min after 185 KBq ($5{\mu}Ci$) Tl-201 injection. Percentage organ uptake and whole body retention ratio of Tl-201 were measured at various periods after injection, and values were compared between control and pinacidil-treated mice. Results: Pinacidil treatment resulted in mild decrease in blood levels of Tl-201, but renal uptakes were markedly decreased at 30-min, 1- and 2-hour, compared to control group. Hepatic, intestinal and muscular uptake were not different. Absolute percentage uptake and tumor to blood ratios of Tl-201 were lower in pinacidil treated mice than in the control group at all time points measured. Whole body retention ratio of Tl-201 was lower in pinacidil treated mice ($58{\pm}4%$ ), than in the control group ($67{\pm}3%$) at 24 hours after with injection of $100{\mu}g$ pinacidil. Conclusion: K-opener did not enhance, but rather decreased absolute tumoral uptake and tumor-to-blood ratios of Tl-201. Decreased whole body retention ratio and renal uptake were observed with pinacidil treatment in tumor-bearing mice.

• The Korean Journal of Nuclear Medicine Technology
• /
• v.21 no.1
• /
• pp.70-75
• /
• 2017

Purpose Radioactive iodine thyroid uptake (RAIU) rate is an examination which determines and seeks about general functions of thyroid gland. The size of thyroid gland is normally different between each person, also patients having thyroid diseases have had a variety of size of thyroid gland compared with others. The purpose of this study will investigate about the counting rate which is effected by the geometric factors through the length and volume changes of the source in RAIU rate. Materials and Methods I-131 185 kBq ($5{\mu}Ci$) were placed in a cylindrical phantom of 0.5 cm, 1 cm, 1.5 cm, and 3 cm in diameter, respectively, and saline was added to gradually increase the length by 1 cm in the horizontal and vertical directions to give a change in volume. The source was measured 20 times for 20 seconds from a distance of 25 cm to $364.4keV{\pm}20%$ energy ROI with Captus 3000 thyroid uptake system (Capintec, NJ, USA). Results When the source was located in the transverse direction of the detector, the consequence of one-way ANOVA is that even though the length of source is increased each diameter, there is mostly no significant difference. When the source was located in the longitudinal direction and the counting rate of length 1 cm at all diameter is set to 100%, the average is 92.57% for length 2 cm, 86.1% for 3 cm, 80.69% for 4 cm, 74.82% for 5 cm, and 69.68% at 6 cm. Conclusion According to this study, it is expected that the gap of RAIU rate has been depended on the thickness of thyroid gland as well as the diameter of the beaker. We know that the change of the volume with the increase of the length of the source had less effect on the change of the counting rate. Thus, in order to reduce the error in the measurement of the counting rate with the thyroid uptake rate equipment, an accurate counting rate can be relatively measured if the counting rate which is measured is corrected by thickness or the distance between the thyroid and the thyroid uptake rate equipment is changed.

• Horticultural Science & Technology
• /
• v.28 no.3
• /
• pp.335-342
• /
• 2010

Sulforaphane (SFN) and total phenolics (TPC) contents and antioxidant activity (AA) were analyzed from 13 radish genotypes (Rhaphanus sativus L.), cultivated at 3 locations with different altitudes (Gangneung: asl 5 m, Jinbu: asl 550 m, and Daegwallyeong: asl 750 m). SFN varied greatly from 0.1 to $120.5{\mu}g{\cdot}g^{-1}$ in dry weight test and was significantly affected by location ($P{\leq}0.001$), genotype ($P{\leq}0.001$) and $location{\times}genotype$ interaction ($P{\leq}0.01$). Radishes, cultivated at Daegwallyeong site, showed higher SFN than those of other locations. Among different genotypes, the root of 'Black radish' and leaves of 'Purunmu' of Daegwallyeong had the highest SFN (107.8 and $120.5{\mu}g{\cdot}g^{-1}$, respectively). TPC in root was affected by genotype ($P{\leq}0.001$), and $location{\times}genotype$ interaction ($P{\leq}0.01$), but not by location. In leaves, TPC was affected by location ($P{\leq}0.01$), genotype ($P{\leq}0.001$), and $location{\times}genotype$ interaction ($P{\leq}0.001$). AA expressed as electron donating ability was significantly influenced by location, genotype and $location{\times}genotype$ interaction and correlated positively with TPC ($Pearson's$ $r$=0.897) in root. These results suggest that radish could be a good source of functional food and high altitude location such as Daegwallyeong has potential for the production of radish with high content of health promoting factors.

• Horticultural Science & Technology
• /
• v.31 no.1
• /
• pp.56-64
• /
• 2013

Recent unusual weather due to global warming causes shortage of daily sunlight and constitutes one of the primary reasons for agricultural damages. LED light sources are frequently utilized to compensate for the shortage of sunlight in greenhouse agriculture. The present study is aimed at evaluating formations of phytochemicals as well as growth characteristics of mature strawberry fruits ('Daewang' cultivar) during cultivation in a closed growth chamber equipped with artificial LED light as a sole light source. Each LED light of blue (448 nm), red (634 and 661 nm) or mixed blue plus red (blue:red = 3:7) was separately supplied and the intensity of each light was adjusted to $200{\pm}1{\mu}mol{\cdot}m^{-2}{\cdot}s^{-1}$ at plant level with a photoperiod consisted of 16 hours light and 8 hours darkness. Strawberries grown under mixed LED light of blue and red wavelengths showed a higher production of fruits than those grown under other LED treatments. Fructose, one of the free sugars, increased in mixed LED light-grown fruits. Anthocyanin contents were elevated remarkably in the mixed LED light-grown fruits compared with those in other LED treatments. Contrastingly, contents of total phenolics and flavonoids were not of much different from one another among the fruits treated with various LED lights. On the other hand, ripening of strawberry fruits was found to be faster when grown under blue LED light compared with other LED treatments. Moreover, antioxidant activities of blue or red LED light-grown fruits, respectively, were significantly higher than those of mixed LED light-grown fruits. We suggest that when daylight is in shortage during cultivation in a greenhouse, supplementation of sunlight with LED light, which is composed of blue and red wavelengths, could be useful for the enhancement of productivity as well as of free sugar content in strawberry fruits. In addition, for the strawberry culture in the plant factory, selective adoption of LED light wavelength would be required to accomplish the purpose of controlling fruit maturation time as well as of enhancing contents of sugars and antioxidants of fruits.

• Journal of Animal Environmental Science
• /
• v.7 no.1
• /
• pp.1-12
• /
• 2001

This study was conducted to develope an automatic liquid feeder for early weaning piglets and to test the efficacy of the system. The liquid feeder consists of water heating and discharge unit, dry diet storing and discharge unit, mixing and discharge unit, mixed liquid feed-delivering unit, and the central control part which control each unit, feeding frequency and the amount of feeding. For investigating the possibility of practical use, a feeding trial was carried out using eighteen three way crossbred piglets weaned on 19 days of age for the experimental period of six weeks. Experimental diet was provided in liquid form using the automatic liquid feeder for the first three weeks and in dry form for the later three weeks. The water heating and discharge unit exactly supplied warm water by 27 $m\ell$/s, into the mixing unit. The dry diet storing and discharge unit supplied dry feed by 3.7g/s, into the mixing unit. Being compared with the standard growth rate suggested by NRC, average daily gain of the piglets during the first three weeks of liquid feeding was lower by 10%, while it was higher during three weeks of dry feeding and over the whole experimental period by 24 and 17%, respectively. Feed/gain was 1.09, 2.14 and 1.89 for the first 3 weeks, later 3 weeks, and whole period, respectively. Diarrhea was observed for three days from day 3 to day 7 after feeding liquid diet, but no pig died of it. In conclusion, a preliminary test for the newly developed an automatic liquid feeder using 19 days of age weaning piglets showed that the unit was successfully operated without any major problems. Piglets raised on a liquid diet through the unit developed grew less during the first three weeks, but their growth and feed intake were greatly improved thereafter, indicating the developed automatic liquid feeder may be practically used in swine industry.

• Korean Journal of Food Science and Technology
• /
• v.42 no.2
• /
• pp.204-209
• /
• 2010

This study was conducted to investigate the anti-diabetic activity of Kocat-D1, which is widely used in traditional medicine to treat diabetes in Shandong, China. Sprague Dawley rats (8 weeks of age) were separated into 4 groups: a normal control, streptozotocin (STZ)-induced diabetic rat group (DM control), Kocat-D1-1 (diabetic rat treated with 0.25 g/kg/day hot water extract), and Kocat-D1-2 (diabetic rat treated with 1 g/kg/day hot water extract). After eight weeks of treatment, the fasting blood glucose levels of the Kocat-D1-1 ($334.3{\pm}32.9\;mg/dL$) and Kocat-D1-2 group ($259.5{\pm}35.0\;mg/dL$) were significantly lower when compared to the DM control group ($451{\pm}42.6\;mg/dL$). Furthermore, the levels of glutamate oxaloacetate transaminase (GOT), glutamate pyruvate transaminase (GPT), albumin and high-density lipoprotein (HDL) cholesterol in the serum of the Kocat-D1-2 group were significantly normalized when compared to the DM control group. However, significant differences were not observed between the Kocat-D1-1 group and the DM control group. Histochemical staining of the liver of the Kocat-D1-2 group revealed no fat accumulation. The insulin level was significantly upregulated in the Kocat-D1-2 group ($0.13{\pm}0.02\;ng/mL$) when compared to the DM control group ($0.05{\pm}0.04\;ng/mL$). The relative volume of $\beta$-cells in the pancreas of the Kocat-D1-2 group ($49.4{\pm}4.2%$) also increased significantly when compared to the DM control group ($12.9{\pm}7.9%$). These results suggest that Kocat-D1 exerts an anti-hyperglycemic effect through the enhancement of insulin secretion.

• Food Science of Animal Resources
• /
• v.27 no.4
• /
• pp.409-415
• /
• 2007

This experiment was conducted to investigate the effects of dietary enzyme mixture fortified with ${\beta}-glucanase$ on the growth performance, serum components and meat quality of broiler chicks. 31,800 Ross 208 male broiler chicks were randomly allotted into 2 groups, the control and 0.3% enzyme diet with ${\beta}-glucanase$ supplementation groups. Control group chicks were fed the control (corn-soybean meal based) diet and the treatment group chicks were fed the 0.3% enzyme mixture supplemented with ${\beta}-glucanase$. The growth performance, serum components and meat qualities such as pH, color, water holding capacity, cooking loss, and shearing force of meats were investigated. The results showed that the growth performance of chicks fed the 0.3% enzyme mixture diet were improved compared to that of the control group, as much as 5% in growth rate, 19% in average weight, 6.8% in performance index, and 5.5% in feed efficiency. Although, there were no significant differences in the muscle color degrees ($L^*a^*b^*$) and shearing force between the control group and experimental group, the water holding capacity and cooking loss of the experimental group were significantly higher than those of control group (p<0.05). The antibody titers in serum against the antigens of Newcastle disease and Infectious Bursal disease were higher in the experimental group than in the control group. Altogether, these suggest that the broiler diet containing 0.3% enzyme mixture fortified with ${\beta}-glucanase$ activity can improve the growth performance, immune reaction, and meat quality of broiler chicks.