• Title/Summary/Keyword: laboratory animals

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The Ability of Cervus Elaphus Sibiricus Herbal Acupuncture to Inhibit the Generation of Inflammatory Enzymes on Collagen-induced Arthritis Mice (녹용약침(鹿茸藥鍼)이 CIA 모델 생쥐의 염증인자 생성억제에 미치는 영향)

  • Hwang, Jong-Soon;Hwang, Ji-Hye;Lee, Hyun-Jin;Lee, Dong-Gun;Kang, Min-Joo;Back, Song-Ook;Cho, Hyun-Seok;Kim, Kyung-Ho;Kim, Kap-Sung
    • Journal of Acupuncture Research
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    • v.24 no.6
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    • pp.1-14
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    • 2007
  • Backgrounds : Rheumatoid Arthritis(RA) is known as the chronic inflammatory diseasethat induces persistent inflammation in the joint cavity. The destruction of cartilage occurs as the result of bones destoyed by pannus, several influential cytokines induced by the synovial capsulitis, varieties of proteinases, $O_2$ radicals, and the secondary degenerative changes of articular cartilage. The type 2 collagen-induced arthritis model is used in recent experimental research on rheumatoid arthritis. Cervus elaphus sibiricus (Nockyong) has the effect of relieving pain by nourishing the muscles, joints, and bones. It is also known to be efficacious in promoting and enhancing the immune system. The objective of this study was to investigate the effect of Cervus elaphus sibiricus herbal acupuncture to inhibit the generation of proinflammatory enzyme on type 2 collagen-induced arthritis. I investigated the inhibition of mRNA transcription of MIF(macrophage migration inhibitory factor), $TNF-{\alpha}$(Tumor necrosis $factor-{\alpha}$) and MMP-9 (matrix metalloproteinase-9) of Cervus elaphus sibiricus herbal acupuncture using an in vitro test. Also investigated was the inhibition of differentiation of Th 1 cells and activation of cytokines(MIF, $TNF-{\alpha}$, IL-6, MMP-9), which are known to cause initial RA ,and are also related to the morphology of the synovial membranes of the joint capsule, by an in vivo test, using CIA(collagen induced arthritis) model mice. Materials & methods : The laboratory animals used in this experiment were 4 week-old DBA female mice, weighing approximately 20 grams, and adjusted to the laboratory environment. The experiment was divided into the normal group(NOR)-no treated group, control group(CON)-CIA induced group, and sample group(SAM)-Cervus elaphus sibiricus herbal acupuncture treated group. RA was induced in the mice via injection of $50{\mu}{\ell}$ C II mixed CFA. The Cervus elaphus sibiricus herbal acupuncture solution was applied on $GB_{35}$(陽陵泉) for 26 days from the 3rd day of RA inducement. The concentration of the solution was determined via a MTT assay. To research the effect on the expression of MIF, $TNF-{\alpha}$ and MMP-9 mRNA, RT-PCR was performed on synovial membrane cells from the knee joint of CIA mice. C II induced RA knee joint's histo-chemical synovial membrane was observed using a specimen model via the Hematoxilin and Eosin dying technique. Results : The expression of mRNA of RA-related cytokines such as MIF, $TNF-{\alpha}$, and MMP-9 dosedependently decreased in the cell from the synovial membranes of the joint, which is treated with Cervus elaphus sibiricus herbal acupuncture solution. In mice treated with Cervus elaphus sibiricusherbal acupuncture, the damage of synovial membranes of the joint was lessened, and differentiation of Th 1 cells was suppressed. The activation of RA-related cytokines such as MIF was suppressed, and the generation of $TNF-{\alpha}$ and MMP-9 showed a statistically significant decreas. Conclusions : It is speculated that Cervus elaphus sibiricus herbal acupuncture has the therapeutic effect of palliating the damage of the tissue impaired by RA by inhibition of the initial RA progression and by regulating excessive differentiation of Th 1 cell as it suppresses the generation of RA-related cytokines during the highest stage of RA by acting on pro-inflammatory enzymes.

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Application of a Single-pulsatile Extracorporeal Life Support System for Extracorporeal Membrane Oxygenation -An experimental study - (단일 박동형 생명구조장치의 인공폐 적용 -실험연구-)

  • Kim, Tae-Sik;Sun, Kyung;Lee, Kyu-Baek;Park, Sung-Young;Hwang, Jae-Joon;Son, Ho-Sung;Kim, Kwang-Taik;Kim. Hyoung-Mook
    • Journal of Chest Surgery
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    • v.37 no.3
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    • pp.201-209
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    • 2004
  • Extracorporeal life support (ECLS) system is a device for respiratory and/or heart failure treatment, and there have been many trials for development and clinical application in the world. Currently, a non-pulsatile blood pump is a standard for ECLS system. Although a pulsatile blood pump is advantageous in physiologic aspects, high pressure generated in the circuits and resultant blood cell trauma remain major concerns which make one reluctant to use a pulsatile blood pump in artificial lung circuits containing a membrane oxygenator. The study was designed to evaluate the hypothesis that placement of a pressure-relieving compliance chamber between a pulsatile pump and a membrane oxygenator might reduce the above mentioned side effects while providing physiologic pulsatile blood flow. The study was performed in a canine model of oleic acid induced acute lung injury (N=16). The animals were divided into three groups according to the type of pump used and the presence of the compliance chamber, In group 1, a non-pulsatile centrifugal pump was used as a control (n=6). In group 2 (n=4), a single-pulsatile pump was used. In group 3 (n=6), a single-pulsatile pump equipped with a compliance chamber was used. The experimental model was a partial bypass between the right atrium and the aorta at a pump flow of 1.8∼2 L/min for 2 hours. The observed parameters were focused on hemodynamic changes, intra-circuit pressure, laboratory studies for blood profile, and the effect on blood cell trauma. In hemodynamics, the pulsatile group II & III generated higher arterial pulse pressure (47$\pm$ 10 and 41 $\pm$ 9 mmHg) than the nonpulsatile group 1 (17 $\pm$ 7 mmHg, p<0.001). The intra-circuit pressure at membrane oxygenator were 222 $\pm$ 8 mmHg in group 1, 739 $\pm$ 35 mmHg in group 2, and 470 $\pm$ 17 mmHg in group 3 (p<0.001). At 2 hour bypass, arterial oxygen partial pressures were significantly higher in the pulsatile group 2 & 3 than in the non-pulsatile group 1 (77 $\pm$ 41 mmHg in group 1, 96 $\pm$ 48 mmHg in group 2, and 97 $\pm$ 25 mmHg in group 3: p<0.05). The levels of plasma free hemoglobin which was an indicator of blood cell trauma were lowest in group 1, highest in group 2, and significantly decreased in group 3 (55.7 $\pm$ 43.3, 162.8 $\pm$ 113.6, 82.5 $\pm$ 25.1 mg%, respectively; p<0.05). Other laboratory findings for blood profile were not different. The above results imply that the pulsatile blood pump is beneficial in oxygenation while deleterious in the aspects to high pressure generation in the circuits and blood cell trauma. However, when a pressure-relieving compliance chamber is applied between the pulsatile pump and a membrane oxygenator, it can significantly reduce the high circuit pressure and result in low blood cell trauma.

Chronological observation of intestinal lesions of rots experimentally infected with Echinostoma hortense (흰쥐의 실험적 호르틴스극구흡충 감염에 있어서 장 병변에 대한 경시적 관찰)

  • 이순형;노태영
    • Parasites, Hosts and Diseases
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    • v.28 no.1
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    • pp.45-52
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    • 1990
  • Intestinal histopathological changes due to infection with Echinostcma hortense (Trematoda) were studied in rats after experimental infection with the metacercariae. The metacercariae were obtained from the tadpoles of Rana nigrcmaculata, a second intermediate host infected in the laboratory. Total 18 albino rats(Sprague-Dawley) were given 200 matacercariae each and sacrificed on the day 1, 3, 7, 11, 22 or 44 post-infection(PI) Segments of- the small intestine at 1, 3, 5, 8 and 30 cm posterior to the pylorus(PTP) were rejected and studied histopathologically. 1. The flukes were seen to have intruded into the intervillous space in the upper small intestine at early stages(1∼3 days PI), however, they were located mainly in the intestinal lumen at later stages(7∼44 days PI) . The flukes were sucking and destroying the epithelial layers of villi with their oral and ventral suckers. 2. Histopathological changes of the intestine were recognizable in as early as 1∼3 days after infection, and the changes became severer as the infection progressed. 3. The intestinal mucosa was histopathologically characterized by villous atrophy and crypt hyperplasia throughout the infection period. Major villous changes were blunting, fusion, severe destruction and loss of epithelial layers of villi. Villous/crypt(V/C) height ratio was remarkably reduced from 3 : 1 in controls to 1 : 1 in severely infected animals. In the stroma of villi, inaamma- tory cell infiltrations, vascular congestion, edema, and/or fibrosis were recognized. The goblet cells were increased in number after 11 days PI. It was revealed in the present study that the pathological changes in the intestine of rats infected with E. hortense were chieay confined to the mucosal layer of the upper small intestine, however, the changes were very severe accompanying remarkable destruction of villi and loss of mucosal integrity, and persistent until 44 days PI.

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Microbe Hunting: A Curious Case of Cryptococcus

  • Bartlett, Karen H.;Kidd, Sarah;Duncan, Colleen;Chow, Yat;Bach, Paxton;Mak, Sunny;MacDougall, Laura;Fyfe, Murray
    • Proceedings of the Korean Environmental Health Society Conference
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    • 2005.06a
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    • pp.45-72
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    • 2005
  • C. neoformans-associated cryptococcosis is primarily a disease of immunocompromised persons, has a world-wide distribution, and is often spread by pigeons in the urban environment. In contrast, C. gattii causes infection in normal hosts, has only been described in tropical and semi-tropical areas of the world, and has a unique niche in river gum Eucalyptus trees. Cryptococcosis is acquired through inhalation of the yeast propagules from the environment. C. gattii has been identified as the cause of an emerging infectious disease centered on Vancouver Island, British Columbia, Canada. No cases of C. gattii-disease were diagnosed prior to 1999; the current incidence rate is 36 cases per million population. A search was initiated in 2001 to find the ecological niche of this basidiomycetous yeast. C. gaftii was found in the environment in treed areas of Vancouver Island. The highest percentage of colonized-tree clusters were found around central Vancouver Island, with decreasing rates of colonization to the north and south. Climate, soil and vegetation cover of this area, called the Coastal Douglas fir biogeoclimatic zone, is unique to British Columbia and Canada. The concentration of airborne C. gattii was highest in the dry summer months, and lowest during late fall, winter, and early spring, months which have heavy rainfall. The study of the emerging colonization of this organism and subsequent cases of environmentally acquired disease will be informative in planning public health management of new routes of exposure to exotic agents in areas impacted by changing climate and land use patterns. Cryptococcosis is an infection associated with an encapsulated, basidiomycetous yeast Cryptococcus neoformans. The route of entry for this organism is through the lungs, with possible systemic spread via the circulatory system to the brain and meninges. There are four cryptococcal serogroups associated with disease in humans and animals, distinguished by capsular polysaccharide antigens. Cryptococcus neoformans: variety grubii (serotype A), variety neoformans (serotype D), and variety gattii (serotypes B and C) (Franzot et at. 1999). C. neoformans variety gattii has recently been elevated to species status, C. gattii. C. neoformans val. grubii and var. neoformans have a world-wide distribution, and are particularly associated with soil and weathered bird droppings. In contrast, C. gattii (CG) is not associated with bird excrement, is primarily found in tropical and subtropical climates, and has a restricted environmental niche associated with specific tree species. (Ellis & Pfiffer 1990) Ellis and Pfeiffer theorize that, as a basidiomycete, CG requires an association with a tree in order to become pathogenic to mammals. In Australia, CG has been found to be associated with five species of Eucalypts, Eucalyptus camaldulensis, E. tereticornis, E. blakelyi, E. gomphocephala, and E. rudis. Eucalypts, although originally native to Australia, now have a world-wide distribution. CG has been found associated with imported eucalypts in India, California, Brazil, and Egypt. In addition, in Brazil and Columbia, where eucalypts have been naturalized, native trees have been shown to harbour CG (Callejas et al. 1998; Montenegro et al. 2000). In British Columbia, Canada, since the beginning of 1999, there have been 120 confirmed cases of cryptococcal mycoses associated with CG in humans, including 4 fatalities (data from British Columbia Centre for Disease Control), and over 200 cases in animal pets in BC (data from Central Laboratory for Veterinarians). What is remarkable about the BC outbreak of C. gattii-cryptococcosis is that all of the cases have been residents of, or visitors to, a narrow area along the eastern coast of Vancouver Island, BC, from the tip of the island in the south (Victoria) to Courtenay on the north-central island as illustrated in Figure 1. Of the first 38 human cases, 58% were male with a mean age of 59.7 years (range 20 - 82): 36 cases (95%) were Caucasian. Ten cases (26%) presented with meningitis, the remainder presented with respiratory symptoms. Cultures recovered from cases of cryptococcosis associated with the outbreak were typed as serogroup B, which is specific to CG (Bartlett et al. 2003). This was the first reported outbreak of CVG in Canada, or indeed, the world. Where infection with CG is endemic, for example, Australia, the incidence of cryptococcosis ranges from 1.8 - 4.7 per million between the southern and northern states (Sorrell 2001). However, the overall incidence of cryptococcosis in immunocompenent individuals has been estimated at 0.2 per million population per year (Kwon-Chung et al. 1984). The population of Vancouver Island is approximately 720,000,consequently, even if the organism were endemic, one would expect a maximum of 0.15 cases of cryptococcal disease annually.

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Effect of Fermented (Oenanthe stolonifera DC) Extract on the Activity of Enzymes Related to Liver Function of Alcohol-administered Rats and Mice (밭미나리 발효액이 알콜투여 흰쥐의 간기능관련효소활성에 미치는 영향)

  • Whang, Tay-Eak;Lim, Hyun-Ock;Lee, Jae-Wa
    • Korean Journal of Medicinal Crop Science
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    • v.7 no.2
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    • pp.107-114
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    • 1999
  • It has long been recognized that dropwort contains specific funtional subtances for protecting human liver and preventing and curing its diseases, and thus it has been widely utilized in traditional folk remedy. In the present study. fresh of fermented extract of dropwort shoots grown on dryland and fresh extract of those grown on flooded fie1d were fed to the rats suffering from acute, subacute or chronical toxication induced by alcohol administration, and their affects were investigated. Administration of alcohol to rats and mice for 2 days at 5ml of 30% EtOH/kg/day raised total cholesterol and total glyceride which were, however, great1y supressed when alcohol was administered to the laboratory animals previously fed on fresh or fermented extract of dryland dropwort, or fresh extract of flooded field-grown dropwort for 20 days, without significant differences among the extracts. The levels of alanine aminotransferase and aspartate aminotransferase which were raised by alcohol adminstration were also lowered by feeding dropwort extract, among which that of fermented dryland-grown one was more effective than the other two. Chronic alcohol toxication was induced to rats by administering 10% alcohol for 10 months and fermented dropwort extract or tap water was fed to the rats for 5 days. The rats fed on fermented dropwort extract were lower in total cholesterol by 40% and in tota1 glyceride by 60% than the control. Alanine aminotransferase and aspartate aminotransferase in the rats fed on fermented dropwort extract were decreased by 87.2% and 91.7%, respectively, compared to the control, and the rats recovered almost to normal. Activity of alkaline phosphatase, catalase, superoxide dismutase or glutathione peroxidase changed greatly by alcohol administration in the rats suffering from chronic as well as acute toxication. The extract of fermented dry land dropwort significantly lowed the activity of those enzymes, especially, alkaline phosphatase and superoxide dismutase. The present results suggesting the possible medicinal effect of fermented dropwort extract to liver diseases.

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Histological Changes in the Normal Tissues of Rat after Local Application of the Holmium-166-Chitosan Complex attached to Biodegradable Solid Material (생분해성 고형물에 흡착시켜 실험동물에 국소 투여한 홀미움- 166-키토산 복합체의 투여량, 기간 및 부위에 따른 조직의 괴사 정도와 양상)

  • Lee, Jong-Seok;Jeon, Dae-Geun;Cho, Wan-Hyung;Lee, Soo-Yong;Oh, Jung-Moon;Kim, Jin-Wook
    • The Journal of the Korean bone and joint tumor society
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    • v.9 no.2
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    • pp.190-199
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    • 2003
  • Purpose: The aim of this study was to find out a clinically appliable method to insert a biodegradable solid material containing holmium-166-chitosan complex into the surgical field, and to evaluate the histological changes in the normal tissues after ${\beta}$ -ray irradiation from holmium-166 according to the dose, period and type of tissues. Materials and Methods: 3.0 mCi, 50 ${\mu}l$ of the liquid state $^{166}$Ho-chitosan complex was attached to the absorbable gelatin sponge. The radiation activity measured by dose caliberator was 1.5 mCi. These $^{166}$Ho-chitosan complex containing absorbable gelatin sponges were inserted into the thigh muscles and over the femur bones of the Wistar rats. The cases were evaluated at 2 weeks after insertion, and 4, 6 weeks with respect to the histological changes of the soft tissues and bone, the depth of the tissue necrosis, and the changes of the $^{166}$Ho-chitosan complex containing absorbable gelatin sponges. Results: At 2 weeks, the muscles showed coagulation necrosis, degenerating myocytes, regenerating myocytes, intermuscular edema, and inflammatory cells. The necrosis depth was 3.3 mm. In the bones, there was no osteocyte in the lacuna of cortex (empty lacuna), marrow fibrosis, inflammation. The necrosis depth was 2.9 mm. At 4 weeks, in the muscle, calcification and increased fibrosis with necrosis depth by 3.3 mm were the additional findings. In the bone, marrow fibrosis with necrosis depth by 3.3 mm were detected. At 6 weeks, soft tissue shrinkage, increased fibrosis and granulation tissue formation, and nearly resolving inflammatory reaction were the findings. Conclusion: The local application of the $^{166}$Ho-chitosan complex attached to biodegradable gelatin material with surgery in the laboratory animals resulted in no mortality and morbidity, and satisfactory tissue necrosis. Holmium-166 can be applied to the treatment of the malignant tumor patients.

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Variation Patterns of the Blood Lipid Levels on the Sprague-Dawley-Rats Fed with Kochujang Extracts (고추장 추출물을 급여한 Sprague-Dawley-Rat에서의 혈중지질 농도의 변화 양상)

  • Woo, Yong-Ku;Kim, Tae-Hyo;Koh, Jong-Ho
    • The Korean Journal of Food And Nutrition
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    • v.26 no.4
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    • pp.737-744
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    • 2013
  • The present study was investigated to evaluate on the health improving effect of Kochujang (Fermented Korean hot pepper paste), which was famous as Korean traditional healthy food among foreigners as it was served together with Bibimbap. For comparison, observation was made on the experimental animals (Sprague-Dawley rat) as breeding during 5 weeks, which were divided three groups as into the control groups (N-group) fed with normal feed only and high cholesterol feed only (H-group) and the K-group with combined diet of normal feed and Kochujang extracts (Water soluble complex materials). The most outstanding result was found as the definite fatty liver lesions found from all (100%) of the rats of the H-group at the abdominal opening examination. On the other hand, there was any finding on fatty liver lesions in the N-group as well as the K-group. Therefore, it was more than enough to identify the hypolipidemic effect by visual finding only. Furthermore, from the results of blood plasma analysis from SD-rats fed with Kochujang extract (K-group), more prominent differential findings were found, the value of HDL-cholesterol, which was good for preventing the arteriosclerosis, was more or less higher than that of H-groups. But the level of LDL-cholesterol, which was one of the hazardous agents of hyperlipidemia and arteriosclerosis, was more significantly reduced to the level even lower than that of N-group, based on the statistical analysis, in especial. According to the present results, we could find that the unidentified Korean traditional Kochujang-derived complex components had expressed both the positive health improving effects and hypolipidemic effects on the SD-rat.

Development of Artificial Pulmonary Nodule for Evaluation of Motion on Diagnostic Imaging and Radiotherapy (움직임 기반 진단 및 치료 평가를 위한 인공폐결절 개발)

  • Woo, Sang-Keun;Park, Nohwon;Park, Seungwoo;Yu, Jung Woo;Han, Suchul;Lee, Seungjun;Kim, Kyeong Min;Kang, Joo Hyun;Ji, Young Hoon;Eom, Kidong
    • Progress in Medical Physics
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    • v.24 no.1
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    • pp.76-83
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    • 2013
  • Previous studies about effect of respiratory motion on diagnostic imaging and radiation therapy have been performed by monitoring external motions but these can not reflect internal organ motion well. The aim of this study was to develope the artificial pulmonary nodule able to perform non-invasive implantation to dogs in the thorax and to evaluate applicability of the model to respiratory motion studies on PET image acquisition and radiation delivery by phantom studies. Artificial pulmonary nodule was developed on the basis of 8 Fr disposable gastric feeding tube. Four anesthetized dogs underwent implantation of the models via trachea and implanted locations of the models were confirmed by fluoroscopic images. Artificial pulmonary nodule models for PET injected $^{18}F$-FDG and mounted on the respiratory motion phantom. PET images of those acquired under static, 10-rpm- and 15-rpm-longitudinal round motion status. Artificial pulmonary nodule models for radiation delivery inserted glass dosemeter and mounted on the respiratory motion phantom. Radiation delivery was performed at 1 Gy under static, 10-rpm- and 15-rpm-longitudinal round motion status. Fluoroscpic images showed that all models implanted in the proximal caudal bronchiole and location of models changed as respiratory cycle. Artificial pulmonary nodule model showed motion artifact as respiratory motion on PET images. SNR of respiratory gated images was 7.21. which was decreased when compared with that of reference images 10.15. However, counts of respiratory images on profiles showed similar pattern with those of reference images when compared with those of static images, and it is assured that reconstruction of images using by respiratory gating improved image quality. Delivery dose to glass dosemeter inserted in the models were same under static and 10-rpm-longitudinal motion status with 0.91 Gy, but dose delivered under 15-rpm-longitudinal motion status was decreased with 0.90 Gy. Mild decrease of delivered radiation dose confirmed by electrometer. The model implanted in the proximal caudal bronchiole with high feasibility and reflected pulmonary internal motion on fluoroscopic images. Motion artifact could show on PET images and respiratory motion resulted in mild blurring during radiation delivery. So, the artificial pulmonary nodule model will be useful tools for study about evaluation of motion on diagnostic imaging and radiation therapy using laboratory animals.

Blood Picture of the Korean Native Goats from Birth to Maturity (한국 재래 염소의 성장에 따르는 혈액상의 변동)

  • Choi, Hi In
    • Korean Journal of Veterinary Research
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    • v.14 no.1
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    • pp.115-133
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    • 1974
  • Although considerable research has been done on the changes associated with age in the blood picture of domestic and laboratory animals, little work has been made of the changes occurring at different age in the blood picture of goats. And a comprehensive survey of the bood picture of Korean native goats has not been made. The object of the present investigation was to suggest standards for the blood picture of Korean native goats at frequent intervals from birth to maturity. The goats were kept under average farming conditions in this country. Observations were made at the following ages: at birth; 2,4 and 7 days; 2, 3, 4, 5, 6, 7, 8 and 9 weeks; 2.5, 3,6 and 12 months. Blood samples were drawn from the jugular vein. It was taken between 8 and 9 a.m. except those for the at-birth period. Erythrocyte and leukocyte enumerations and, determinations of hemoglobin in blood and hematocrit value were made in the usual manner. Reticulocytes were enumerated per 1,000 erythrocytes in blood smears stained with briIliant cresyl blue and counterstained with Wright's stain. Erythrocytes counts declined from $8.7{\times}10^8/mm^3$ at birth to a low of 7.0 at 4 days of age. These values increased to 11.5 at 5 weeks and reached a maximum of 14.0 at 3 months of age; it then fell to 11.5 at 12 months of age. Concentrations of hemoglobin in blood and hematocrit values were not related to the changes of erythrocyte counts. The values at birth were higher than at any other period during the first year of life. These fell from highs of 12.3 g/100 ml and 38.0 ml/100 ml to lows of 9.2 and 29 at 4 weeks for concentration of hemoglobin in blood and hematocrit value, respectively. There was a common pattern for the hematocrit value and hemoglobin in blood which showed three phases-a fall during the first month, a rise to the third month, and a fall to the mature level at 12 months of age. Mean corpuscular volume and mean corpuscular hemoglobin showed a common pattern. The values were $44.2{\mu}m^3$ and 14.2 pg at birth and fell, at first slowly and then rapidly, to reach adult levels of 24.1 and 7.9 at 6 weeks of age for mean corpuscular volume and mean corpuscular hemoglobin, respectively. Mean corpuscular hemoglobin concentration was little affected by age. Reticulocyte was observed from birth to 4 weeks of age. Percentage of reticulocyte decreased from 0.85% at birth to 0.06% at 4 weeks of age. Total leucocyte counts increased from $5.64{\times}10^3/mm^3$ at birth to a maximum of 13.4 at 3 months; it then fell to 11.5 at 12 months of age. In differential counts myelocyte, juvenile and band form decreased with advancing age. No myelocyte and juvenile were seen after the age of 7 and 9 weeks, respectively, and band forms were rare after the age of 3 months. Percentage of mature neutrophil showed a quick decline from 52.5% at birth to reach a minimum level (34.5%) at 3 months of age; it then rose to 38% at 12 months of age. Percentage of lymphocyte increased from 39.2% at birth to maximum of 59% at 3 month of age; it then fell to 54.9% at 12 months of age. Percentage of monocyte was not affected with advance of age. Percentage of eosinophil and basophil were increased with advance of age to reach a maximum at 2 to 3 month of age. It then fell to adult level at 12 month of age.

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Characterization of a Mucolipidosis Type II Mouse Model and Therapeutic Implication of Lysosomal Enzyme Enriched Fraction Derived from Placenta (뮤코지방증 2형 마우스 모델의 특징과 태반에서 추출한 리소좀 효소 투여의 결과)

  • Cho, Sung Yoon;Kim, Ki-Yong;Kim, Su Jin;Sohn, Young Bae;Maeng, Se Hyun;Kim, Chi Hwa;Ko, Ah-Ra;Song, Junghan;Yeau, Sung-Hee;Kim, Kyung-Hyo;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.5-13
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    • 2012
  • I-cell disease (mucolipidosis type II; MIM 252500) and pseudo-Hurler polydystrophy (mucolipidosis type III; MIM 252600) are disorders caused by abnormal lysosomal transport in cells. The presence of numerous inclusion bodies in the cytoplasm of fibroblasts, a lack of mucopolysacchariduria, increased lysosomal enzyme activity in serum, and decreased GlcNAc-phosphotransferase activity are hallmark. Here, we attempted to investigate phenotypical and biochemical characteristics of the knockoutmouse of GlcNAc-phosphotransferase ${\alpha}/{\beta}$ subunits; in addition, we also attempted to determine whether the lysosome enriched fraction derived from placenta can be beneficial to phenotype and biochemistry of the knockout mouse.We found that the knockout mouse failed to thrive and had low bone density, as is the case in human. In addition, skin fibroblasts from the animal had the same biochemical characteristics, including increased lysosomal enzyme activity in the culture media, in contrast to the relatively low enzyme activity within the cells. Intravenous injection of the lysosome rich fraction derived from placenta into the tail vein of the animal resulted in a gain of weight, while saline injected animals didn't.In conclusion, our study demonstrated the phenotypical and biochemical similarities of the knockout mouse to a mucolipidosis type II patient and showed the therapeutic potential of the lysosome enriched fraction. We admit that a larger scale animal study will be needed; however, the disease model and the therapeutic potential of the lysosome enriched fraction will highlight the hope for a novel treatment approach to mucopolipidosis type II, for which no therapeutic modality is available.

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