Lee, Seung Ho;Park, Woo Sung;Lee, Young Seok;Yu, Jeesuk
Journal of The Korean Society of Inherited Metabolic disease
/
v.14
no.2
/
pp.156-162
/
2014
A 25-month-old boy was referred to the hospital due to large head detected on routine physical examination. At visit, dysmorphic facial appearances, including broad nose, prominent forehead, and coarse face, were noted. Nasal obstruction with nasal voice, prominent adenoids, and bilateral middle ear effusions were detected. His abdomen was distended, and liver and spleen were palpated about 3 finger and 2 finger breadths, respectively. He was operated for bilateral inguinal hernias. The motion of both elbow joints was mildly limited on supination and pronation. Urinary level of glycosaminoglycan was elevated and the enzyme activity of iduronate sulfatase in leukocytes was decreased. The mutational analysis of the gene iduronate 2-sulfatase (IDS) revealed c.263G>A (p.Arg88His) mutation. His developmental scale showed delayed development and there was cardiac valvular involvement (tricuspid regurgitation and mitral valve prolapse). After the diagnosis of Hunter syndrome, enzyme replacement therapy started on a weekly basis without progression of any clinical features. Here we report a case of early diagnosed Hunter syndrome detected by large head on routine examination. Thus, it is important to associate Hunter syndrome in the patient with large head especially, if there is the history of bilateral inguinal hernia and prominent adenoids to increase the possibility of early diagnosis and treatment.
The present investigation involves a comparative study of enzymatic activities in various animals. The levels of the liver protein of rat (22.0$\pm$0.01mg/ml and pigeon (22.0$\pm$ 0.16) are twice as high as that of crucian carp (13.0$\pm$ 0.09)(p < 0.01). Generally, the specific acitivity (3.77 $\pm$0.18 unit/mg) of rat glutamic pyruvic transaminase (GPT) is highest, pigeon intermediate (1.93 $\pm$0.01), and crucian carp lowest (0.71$\pm$0.07). On the other hand, the specific activity (8.23$\pm$0.09 unit/mg)of rat glutamic oxaloacetic transaminase (GOT) is highest, pigeon intermediate (3.95$\pm$0.09), and crucian carp lowest (0.92$\pm$0.01) (p < 0.01). Ratios of GOT activity to GPT activity appear no remarkable difference from the levels of various animals. Specific activity of glutamic dehydrogenase(GDH) in pigeon tissue exceeds those of rat and crucian carp. In liver, rat GOT specific activity is greater than crucian carp and pigeon. On the other hand, pigeon GDH specific activity is greater than those of rat and crucian carp. This wouls seem to be in accord with protein metabolic intensity. The patterns for GDH isozyme were remarkably appeared in various animals. Glutamic dehydrogenase isozymes gave different electrophoretic mobilities in various animals. It is interesting that crucian carp, pigeon, and rat would show this difference, which may be indicative of an evolutional pattern. The fact that livers in various animals show quite different enzyme activities would suggest the existence of such a general phylogenetic relationship.
The aim of this study is to investigate the effects of hot water soluble extract from green tea on the components of serum and the liver and the antioxidative effects in accordance with the different protein types. For this purpose, four experimental groups were set up. As for the protein source casein, isolated soy protein was supplemented to the rats, together with hot water soluble extract from green tea. Four experimental groups kept eight Sprague-Dawley rats respectively. The CP group was supplemented with casein only, the CG group was supplemented with casein and hot water soluble extract from green tea, the ISP group was supplemented with isolated soy protein only, and the ISG group was supplemented with the isolated soy protein with hot water soluble extract from green tea. After 4 weeks of feeding with experimental diet, the levels of serum and liver lipid and antioxidant enzyme activity and TBARS in the liver were measured. The results are; 1. Weight gain and FER were higher in the casein group than in the isolated soy protein group in general. In the casein group, the weight gain and FER were reduced significantly when hot water soluble extract from green tea was supplemented (P < 0.05). There was no significant difference in feed intake. 2. In general, total cholesterol and LDL-cholesterol in the serum were higher in the casein group than in the isolated soy protein group, however just the concentration of LDL-cholesterol in the casein group was significantly lower when the hot water soluble extract from green tea was supplemented (P < 0.05). 3. Triglycerides in the liver were higher in the casein group than in the isolated soy protein group general, however when hot water soluble extract from green teas was supplemented only in the isolated soy protein group, the content of triglyceride in liver was significantly lower (P < 0.05). 4. There was no significant difference in antioxidant enzyme activity in the liver in all the groups, however the content of TBARS was low only in the casein group when hot water soluble extract from green tea was supplemented (P < 0.05).
Although iron is an essential mineral, excess iron intake during pregnancy may increase oxidative stress in tissues. This study was conducted to investigate the effects of iron overload during pregnancy on iron status and oxidative stress in maternal rats. Ten week-old female Sprague-Dawley rats were mated with male rats. Non-pregnant (control) and pregnant rats were fed diets containing normal Fe (35 mg/kg diet), high Fe (350 mg/kg diet), or excess Fe (1,050 mg/kg diet) during pregnancy. Rats were sacrificed on pregnancy day 19. No significant difference in weight gain, diet intake, or litter size was observed according to iron intake levels. Furthermore, serum iron, hemoglobin, and hematocrit were not different among the rats administered the three levels of Fe both in the control and pregnant groups. However, the iron levels were lower in pregnant rats than those in the control. The liver and spleen iron contents increased significantly in the excess Fe group. An increase in liver ferritin levels with increasing iron intake was observed. Protein carbonyl content, as a marker of oxidative stress, increased significantly in liver with increasing iron intake but not malondialdehyde. Glutathione peroxidase activity in the liver of pregnant rats fed excess iron decreased significantly. Bcl-2 protein expression in the liver declined remarkably with increasing maternal iron intake in pregnant rats. Taken together, iron overload during pregnancy had little effect on hematology. However, the deposits of iron in the liver and the decline in antioxidant enzyme activity implied increased oxidative stress in tissues of the excess Fe group. These results suggest that excess iron intake during pregnancy increases oxidative stress in maternal tissues and may also affect fetal tissues.
The effects of each component (water extracts, alcohol extracts, lipophillic extracts, total saponin, panaxadiol, panaxatriol) of red ginseng on the antioxidative enzyme activities were investigated in the liver in order to screen antioxidative components of red ginseng. 20∼25g ICR mouse which were pretreated with 50 mg/kg body weight of red ginseng component for 15 days. The ability of red ginseng component to protect against oxidative damage to the mouse liver was examined by determining the level of lipid peroxidation (MDA), hydroperoxide (H$_2$O$_2$) and the activities of superoxide dismutase (SOD), catalase. The hepatic total-SOD activity was highest in lipophilic extracts group and panaxadiol group next (p<0.01). The content of hepatic hydroperoxide was lowest in the order of panaxatriol group and alcohol extracts group (p < 0.01). The hepatic catalase activity in the liver was highest in order of lipophillic extracts group (p <0.01) and total saponin group (p<0.05). Finally the lipid peroxidation (MDA) level was lowest in lipophillic extracts group, alcohol extracts group and panaxadiol next (p <0.01). In conclusion, the order of effectiveness of antioxidants was to be lipophillic extracts>panaxadiol >total saponins.
Recently the measurement of biophoton emission has attracted increasing attention in the study on physiological state of biological systems. We report the measurements of biophoton emission from the mouse fatty liver induced by carbon tetrachloride, CCl$_4$. The hepatotoxin, CCl$_4$ in olive oil, was injected intraperitoneally into two groups of ICR mice which were made of 6 mice in each group. The control groups corresponding to the treated groups were prepared with the injections of olive oil only. After the injections, livers of two groups were extracted and measured biophoton emission in 24 hours and 72 hours later, respectively. We also extracted the plasma in the blood and measured the transaminase activity. Results show that biophoton emission from the livers in 24-hour treated group is 69.3${\pm}$21.2 counts/min/$\textrm{cm}^2$, which is two times more larger than that in 24-hour control group, 29.5${\pm}$5.9 counts/min/$\textrm{cm}^2$ Biophoton emission from the livers in 72-hour treated group is 37.0${\pm}$14.8 counts/min/$\textrm{cm}^2$. These biophoton results correlate with those of the biochemical assays. We conclude that biophoton emission can be used as a biomarker of mouse fatty liver induced by CCl$_4$.
This study aimed to determine whether or not glycoprotein isolated from Cudrania tricuspidata Bureau fruit(CTB glycoprotein) exerts a hepatoprotective effect on liver injury induced by the administration of carbon tetrachloride($CCl_4$, 1.0mL/kg) to A/J mice. Following the administration of CTB glycoprotein(0-20mg/kg), the activities of antioxidant enzymes (superoxide dismutase(SOD), catalase(CAT), and glutathione peroxidase(GPx)), and the quantities of measured thiobarbituric acid reactive substances(TBARS), lactate dehydrogenase(LDH), and nitric oxide(NO) were evaluated from the murine liver tissues and plasma. Additionally, the activity of nuclear factor-kappa B(NF-${\kappa}B$) was assessed after pretreatment with $CCl_4$. When the mice were treated with $CCl_4$ alone, the activities of antioxidative enzymes reduced but amounts of TBARS, LDH, and NO increased. However, the results of treatment with CTB glycoprotein(10 and 20 mg/kg) revealed significantly increased activities of antioxidant enzymes(SOD, CAT, and GPx), as compared with $CCl_4$ alone. On the other hand, the result showed significant diminutions of the quantities of TBARS, LDH, and NO after treatment with CTB glycoprotein(10 and 20 mg/kg), as compared to $CCl_4$ alone. The activity of NF-${\kappa}B$ also declined after pretreatment with CTB glycoprotein, as compared with $CCl_4$ treatment alone. Thus, it is suggested that the CTB glycoprotein exerts a protective effect against $CCl_4$-induced liver injury in A/J mice.
Journal of the Korean Society of Food Science and Nutrition
/
v.45
no.5
/
pp.625-633
/
2016
Allium hookeri is known as a healthy food since it contains larger amounts of sulfur compounds than commonly known alliaceous plants. The antioxidant and anti-inflammatory effects of A. hookeri were compared between two types of extracts, $80^{\circ}C$ water and 95% ethanol extracts of A. hookeri roots. A. hookeri root 95% ethanol extracts displayed superior total polyphenol content, antioxidant activity [1,1-diphenyl-2-picrylhydrazyl and 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonate) radical scavenging activity], and anti-inflammation activity than those of water extracts (P<0.05). We studied the effects of A. hookeri root 95% ethanol extracts (95% ethanol extracts group: AHE) on acute alcohol-induced intoxication in mice. AHE [250, 500, and 1,000 mg/kg body weight (BW)/d] was orally administered to the study group once a day for 1 week. On the last day of AHE treatment, 40% ethanol (10 mL/kg BW) was orally administered to induce acute liver injury. The blood alcohol concentration of mice treated with AHE was significantly lower compared to the control group (P<0.05). The levels of hepatic aspartate aminotransferase and alanine aminotransferase were lower in the AHE-treated group than the control group (P<0.05). The RT-PCR results for alcohol dehydrogenase and aldehyde dehydrogenase measured based on mRNA in liver tissues showed that enzyme activities were higher in the AHE-treated group than in the control group at a low blood alcohol concentration.
These studies were carried out to observe the effect of Aqua-acupuncture with Woolgum extract on the recovery from liver injury of rats. The liver injury of rats induced with 0.5ml/200g carbon tetrachloride. The Aqua-acupuncture with Woolgum extract solution inserted into corresponding locus of the Kansu(BL18.) in Rats. Experimental group was divided into 4 groups: Normal group, $CCI_4$-intoxicated group, Saline treated group after $CCI_4$-intoxicated and Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. Biochemical assays for each serum enzyme activity of GOT, GPT, LDH, total protein, triglyceride, albumin, WBC, neutrophils, lymphocyte were performed. The results were as follows : 1. GOT was decreased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. In GOT activity, the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated showed higher level significant compared with the saline aqua-acupuncture treated group after $CCI_4$-intoxicated. 2. GPT was decreased with statistical significance in the Woolgum aqua-acupuncture treated after $CCI_4$-intoxicated. In GOT activity, the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated showed higher level significant compared with the saline aqua-acupuncture treated group after $CCI_4$-intoxicated. 3. LDH was decreased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. 4. Total protein increased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. 5. Albumin was increased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. The saline aqua-acupuncture treated group after $CCI_4$-intoxicated was not recognized as compared with $CCI_4$-intoxicated group. 6. Triglyceride was increased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. 7. WBC was decreased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. In WBC count, the Woolgum aqua-acupuncture higher level significant compared with the saline aqua-acupuncture treated group after $CCI_4$-intoxicated. 8. Lymphocyte was decreased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. 9. Neutrophils was increased with statistical significance in the Woolgum aqua-acupuncture treated group after $CCI_4$-intoxicated. The saline aqua-acupuncture treated group after $CCI_4$-intoxicated was not recognized as compared with $CCI_4$-intoxicated group. From above results, it is suggested that the Woolgum aqua-acupuncture had significant effects on the injury induced by $CCI_4$.
Journal of the Korean Society of Food Science and Nutrition
/
v.30
no.6
/
pp.1190-1196
/
2001
This study was conducted to examine the effects of dietary xylooligosaccharides on HMG-CoA reductase activity and lipid composition of liver in rat fed high cholesterol diet. Sprague-Dawley male rats weighing 100 $\pm$ 10 g were randomly divided into groups of one normal diet, and four high cholesterol diet containing 1% cholesterol. The high cholesterol (1%) diet groups were classified into xylooligosaccharides free diet (C group), 5% xylooligosaccharides diet (C5XO group), 10% xylooligosaccharides diet (C10XO group), and 15% xylooligosaccharides diet (C15XO grcup) according to the levels of dietary xylooligosaccharides supplementation. The experimental diets were fed ad libitum for 4 weeks. The hepatic lipid contents, cholesterol and triglycerides in xylooligosaccharides supplemented groups were significantly lower than those of C group. An antithrombGsis index, a ratio of n-3 to n-6 fatty acids of liver was significantly increased in 10% xylooligosaccharides supplemented groups compared to that of C group. The activity of hepatic HMG-CoA reductase, a rate limiting enzyme of cholesterol biosynthesis, in xylooligosaccharides supplemented groups was more significantly increased than in C group. These results suggest that dietary xylooligosaccharide may be act as potential substitute for a dietary fiber to improve lipids metabolism in rat fed high cholesterol diet.
본 웹사이트에 게시된 이메일 주소가 전자우편 수집 프로그램이나
그 밖의 기술적 장치를 이용하여 무단으로 수집되는 것을 거부하며,
이를 위반시 정보통신망법에 의해 형사 처벌됨을 유념하시기 바랍니다.
[게시일 2004년 10월 1일]
이용약관
제 1 장 총칙
제 1 조 (목적)
이 이용약관은 KoreaScience 홈페이지(이하 “당 사이트”)에서 제공하는 인터넷 서비스(이하 '서비스')의 가입조건 및 이용에 관한 제반 사항과 기타 필요한 사항을 구체적으로 규정함을 목적으로 합니다.
제 2 조 (용어의 정의)
① "이용자"라 함은 당 사이트에 접속하여 이 약관에 따라 당 사이트가 제공하는 서비스를 받는 회원 및 비회원을
말합니다.
② "회원"이라 함은 서비스를 이용하기 위하여 당 사이트에 개인정보를 제공하여 아이디(ID)와 비밀번호를 부여
받은 자를 말합니다.
③ "회원 아이디(ID)"라 함은 회원의 식별 및 서비스 이용을 위하여 자신이 선정한 문자 및 숫자의 조합을
말합니다.
④ "비밀번호(패스워드)"라 함은 회원이 자신의 비밀보호를 위하여 선정한 문자 및 숫자의 조합을 말합니다.
제 3 조 (이용약관의 효력 및 변경)
① 이 약관은 당 사이트에 게시하거나 기타의 방법으로 회원에게 공지함으로써 효력이 발생합니다.
② 당 사이트는 이 약관을 개정할 경우에 적용일자 및 개정사유를 명시하여 현행 약관과 함께 당 사이트의
초기화면에 그 적용일자 7일 이전부터 적용일자 전일까지 공지합니다. 다만, 회원에게 불리하게 약관내용을
변경하는 경우에는 최소한 30일 이상의 사전 유예기간을 두고 공지합니다. 이 경우 당 사이트는 개정 전
내용과 개정 후 내용을 명확하게 비교하여 이용자가 알기 쉽도록 표시합니다.
제 4 조(약관 외 준칙)
① 이 약관은 당 사이트가 제공하는 서비스에 관한 이용안내와 함께 적용됩니다.
② 이 약관에 명시되지 아니한 사항은 관계법령의 규정이 적용됩니다.
제 2 장 이용계약의 체결
제 5 조 (이용계약의 성립 등)
① 이용계약은 이용고객이 당 사이트가 정한 약관에 「동의합니다」를 선택하고, 당 사이트가 정한
온라인신청양식을 작성하여 서비스 이용을 신청한 후, 당 사이트가 이를 승낙함으로써 성립합니다.
② 제1항의 승낙은 당 사이트가 제공하는 과학기술정보검색, 맞춤정보, 서지정보 등 다른 서비스의 이용승낙을
포함합니다.
제 6 조 (회원가입)
서비스를 이용하고자 하는 고객은 당 사이트에서 정한 회원가입양식에 개인정보를 기재하여 가입을 하여야 합니다.
제 7 조 (개인정보의 보호 및 사용)
당 사이트는 관계법령이 정하는 바에 따라 회원 등록정보를 포함한 회원의 개인정보를 보호하기 위해 노력합니다. 회원 개인정보의 보호 및 사용에 대해서는 관련법령 및 당 사이트의 개인정보 보호정책이 적용됩니다.
제 8 조 (이용 신청의 승낙과 제한)
① 당 사이트는 제6조의 규정에 의한 이용신청고객에 대하여 서비스 이용을 승낙합니다.
② 당 사이트는 아래사항에 해당하는 경우에 대해서 승낙하지 아니 합니다.
- 이용계약 신청서의 내용을 허위로 기재한 경우
- 기타 규정한 제반사항을 위반하며 신청하는 경우
제 9 조 (회원 ID 부여 및 변경 등)
① 당 사이트는 이용고객에 대하여 약관에 정하는 바에 따라 자신이 선정한 회원 ID를 부여합니다.
② 회원 ID는 원칙적으로 변경이 불가하며 부득이한 사유로 인하여 변경 하고자 하는 경우에는 해당 ID를
해지하고 재가입해야 합니다.
③ 기타 회원 개인정보 관리 및 변경 등에 관한 사항은 서비스별 안내에 정하는 바에 의합니다.
제 3 장 계약 당사자의 의무
제 10 조 (KISTI의 의무)
① 당 사이트는 이용고객이 희망한 서비스 제공 개시일에 특별한 사정이 없는 한 서비스를 이용할 수 있도록
하여야 합니다.
② 당 사이트는 개인정보 보호를 위해 보안시스템을 구축하며 개인정보 보호정책을 공시하고 준수합니다.
③ 당 사이트는 회원으로부터 제기되는 의견이나 불만이 정당하다고 객관적으로 인정될 경우에는 적절한 절차를
거쳐 즉시 처리하여야 합니다. 다만, 즉시 처리가 곤란한 경우는 회원에게 그 사유와 처리일정을 통보하여야
합니다.
제 11 조 (회원의 의무)
① 이용자는 회원가입 신청 또는 회원정보 변경 시 실명으로 모든 사항을 사실에 근거하여 작성하여야 하며,
허위 또는 타인의 정보를 등록할 경우 일체의 권리를 주장할 수 없습니다.
② 당 사이트가 관계법령 및 개인정보 보호정책에 의거하여 그 책임을 지는 경우를 제외하고 회원에게 부여된
ID의 비밀번호 관리소홀, 부정사용에 의하여 발생하는 모든 결과에 대한 책임은 회원에게 있습니다.
③ 회원은 당 사이트 및 제 3자의 지적 재산권을 침해해서는 안 됩니다.
제 4 장 서비스의 이용
제 12 조 (서비스 이용 시간)
① 서비스 이용은 당 사이트의 업무상 또는 기술상 특별한 지장이 없는 한 연중무휴, 1일 24시간 운영을
원칙으로 합니다. 단, 당 사이트는 시스템 정기점검, 증설 및 교체를 위해 당 사이트가 정한 날이나 시간에
서비스를 일시 중단할 수 있으며, 예정되어 있는 작업으로 인한 서비스 일시중단은 당 사이트 홈페이지를
통해 사전에 공지합니다.
② 당 사이트는 서비스를 특정범위로 분할하여 각 범위별로 이용가능시간을 별도로 지정할 수 있습니다. 다만
이 경우 그 내용을 공지합니다.
제 13 조 (홈페이지 저작권)
① NDSL에서 제공하는 모든 저작물의 저작권은 원저작자에게 있으며, KISTI는 복제/배포/전송권을 확보하고
있습니다.
② NDSL에서 제공하는 콘텐츠를 상업적 및 기타 영리목적으로 복제/배포/전송할 경우 사전에 KISTI의 허락을
받아야 합니다.
③ NDSL에서 제공하는 콘텐츠를 보도, 비평, 교육, 연구 등을 위하여 정당한 범위 안에서 공정한 관행에
합치되게 인용할 수 있습니다.
④ NDSL에서 제공하는 콘텐츠를 무단 복제, 전송, 배포 기타 저작권법에 위반되는 방법으로 이용할 경우
저작권법 제136조에 따라 5년 이하의 징역 또는 5천만 원 이하의 벌금에 처해질 수 있습니다.
제 14 조 (유료서비스)
① 당 사이트 및 협력기관이 정한 유료서비스(원문복사 등)는 별도로 정해진 바에 따르며, 변경사항은 시행 전에
당 사이트 홈페이지를 통하여 회원에게 공지합니다.
② 유료서비스를 이용하려는 회원은 정해진 요금체계에 따라 요금을 납부해야 합니다.
제 5 장 계약 해지 및 이용 제한
제 15 조 (계약 해지)
회원이 이용계약을 해지하고자 하는 때에는 [가입해지] 메뉴를 이용해 직접 해지해야 합니다.
제 16 조 (서비스 이용제한)
① 당 사이트는 회원이 서비스 이용내용에 있어서 본 약관 제 11조 내용을 위반하거나, 다음 각 호에 해당하는
경우 서비스 이용을 제한할 수 있습니다.
- 2년 이상 서비스를 이용한 적이 없는 경우
- 기타 정상적인 서비스 운영에 방해가 될 경우
② 상기 이용제한 규정에 따라 서비스를 이용하는 회원에게 서비스 이용에 대하여 별도 공지 없이 서비스 이용의
일시정지, 이용계약 해지 할 수 있습니다.
제 17 조 (전자우편주소 수집 금지)
회원은 전자우편주소 추출기 등을 이용하여 전자우편주소를 수집 또는 제3자에게 제공할 수 없습니다.
제 6 장 손해배상 및 기타사항
제 18 조 (손해배상)
당 사이트는 무료로 제공되는 서비스와 관련하여 회원에게 어떠한 손해가 발생하더라도 당 사이트가 고의 또는 과실로 인한 손해발생을 제외하고는 이에 대하여 책임을 부담하지 아니합니다.
제 19 조 (관할 법원)
서비스 이용으로 발생한 분쟁에 대해 소송이 제기되는 경우 민사 소송법상의 관할 법원에 제기합니다.
[부 칙]
1. (시행일) 이 약관은 2016년 9월 5일부터 적용되며, 종전 약관은 본 약관으로 대체되며, 개정된 약관의 적용일 이전 가입자도 개정된 약관의 적용을 받습니다.