• 제목/요약/키워드: glycogen metabolism

검색결과 100건 처리시간 0.027초

Dietary Nigella sativa and Peganum harmala Oils Reverses Hyperglycaemia, Hepatotoxicity, and Metabolism in Rats

  • Hamden, Khaled;Carreau, Serge;Jamoussi, Kamel;Ayadi, Fatma;Garmazi, Fadhel;Elfeki, Abdelfattah
    • Food Science and Biotechnology
    • /
    • 제18권3호
    • /
    • pp.739-744
    • /
    • 2009
  • This study aims to evaluate the therapeutic action of administration of Nigella sativa (NS) and Peganum harmala (PH) oils in diabetes and hepatic toxicity. Results show that treatment of diabetic rats with NS oil or PH oil ameliorate hyperglycaemia induced stress oxidative and hepatic dysfunction in diabetic rats. Administration of NS or PH oil to diabetic rats caused an anti-diabetic and antioxidant activities by the decrease in plasmatic glucose level and increase in hepatic superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GPX) activities, reduced glutathione (GSH) and glycogen contents compared to untreated diabetic rats. Besides, NS and PH oils protect the hepatic function observed by decrease of triglyceride (TG), total cholesterol (TCh), and increase of high density lipoprotein-cholesterol (HDL-Ch) levels in serum and hepatic tissues. Moreover, a diminution in the bilirubin, transaminase glutanic pyruvic (TGP), and transaminase pyruvic oxaloacetic (TPO) contents in serum and the thiobarbituric acid-reactive substances levels (TBARs) in hepatic tissues are also detected.

한국인 당원병 제 Ia형 환자의 돌연변이 분석 (Mutation Analysis of Korean Patients with Glycogen Storage Disease Type Ia)

  • 김종원;박지연;서정기
    • Pediatric Gastroenterology, Hepatology & Nutrition
    • /
    • 제4권2호
    • /
    • pp.213-217
    • /
    • 2001
  • 목적: 당원병 Ia형(von Gierke disease)은 glucose 6-phosphatase (G6Pase)의 결함으로 인하여 출생 시부터 복부팽만과 간종대가 나타나고 저혈당, 락트 산혈증(lactic acidemia), 고지방혈증(hyperlipidemia), 고뇨산혈증(hyperuricemia) 등을 초래하며 점차 성장발육부전이 진행되는 상염색체 열성 유전성 질환이다. 본 연구에서는 한국인 GSD Ia 환자 9명을 대상으로 G6Pase 유전자의 돌연변이형에 관한 분석을 처음으로 시행하고자 하였다. 방법: 임상적인 증상과 G6Pase 효소 측정결과를 통하여 GSD Ia로 진단된 9명 환자의 혈액에서 genomic DNA를 분리하여 G6Pase 유전자의 5개 exon 부분이 포함되도록 polymerase chain reaction(PCR) 한 후 PCR 산물을 direct sequencing 하였다. 결과: 9명의 GSD Ia 환자 중 7명 환자에서 g727t homozygote 돌연변이를 발견하였고, 한 환자는 heterozygote로서 g727t 돌연변이와 c611g 돌연변이가 발견되었다. 나머지 한 명에서는 g727t 돌연변이 하나만 발견할 수 있었다. 한 개의 allele에서 발견된 c611g 돌연변이는 exon 4의 178번 아미노산 proline이 alanine으로 변경되는 것으로 지금까지 보고된 바 없는 새로운 돌연변이이다. 결론: 현재까지는 GSD Ia병의 정확한 진단을 위해서 환자의 간 생검 조직에서 G6Pase 효소의 활성도를 측정하는 것이 필요하였으나 한국인 GSD Ia 환자의 경우 g727t 돌연변이가 대부분을 차지하고 있기 때문에 앞으로는 상당수의 환자에서 혈액 채취만으로도 G6Pase 유전자 분석을 통한 GSD 진단이 손쉽게 이루어질 수 있으리라고 생각된다.

  • PDF

Hexachlorophene의 간흡충(肝吸?)의 $C-^{14}$ Glucose 및 $C-^{14}$ Glycine 대사(代謝)에 미치는 영향(影響)에 관(關)한 연구(硏究) (Studies of the Effects of Hexachlorophene on the $C^{14}-Glucose$ and $C^{14}-Glucose$ Metabolism of Clonorchis Sinensis)

  • 차충석
    • 대한약리학회지
    • /
    • 제2권2호
    • /
    • pp.23-33
    • /
    • 1966
  • Hexachlorophene was reported previously to have a powerful parasiticidal effects on Clonorchis sinensis in vitro and in vivo, but the mechanism of its effect was not known. In the present report it was observed that there was an influence of hexachlorophene on the oxygen consumption, the glycolysis, the glycogenesis and the protein synthesis of C. sinensis. A hundred mg. of C. sinensis collected from the biliary tracts of the infested rabbits was incubated in 2 ml of K.R.P. medium with vavious concentration of hexachlorophene, $glucose-1-C^{14}$ and $glycine-1-C^{14}$ in a 25 ml incubation flask with central well. The oxygen consumption was observed by Warburg manometer, the glycogenolysis by measurement of radioactivities of extracted glycogen and protein from C. sinensis incubated with $C^{14}-glucose$ or$C^{14}-glycine$. 1) The oxygen consumption by C. sinensis was markedly inhibited during all stages of incubation in concentration of $10^{-4}$ and $10^{-5}g/ml$ of hexachlorophene, but in $10^{-6}$, slightly increased initially and gradually decreased after 3 hours of incubation. 2) Hexachlorophene inhibited the glycolysis by C. sincnsis markedly in the concentration of $10^{-4}$, $10^{-5}$, $10^{-6}$ and $10^{-7}g/ml$. 3) The protein synthesis by C. sinensis from glycine was inhibited in the concentration of $10^{-5}$, $10^{-6}$ and $10^{-7}g/ml$ of hexachlorophene. 4. The glycogen synthesis by C. sinensis in each concentration of $10^{-4}$, $10^{-5}$ and $10^{-6}g/ml$ of hexachlorophene was inhibited markedly. The speed of inhibition was more rapid in high concentration than in low, and in low concentration even the glycogen itself which had synthesized in their stage in their body was consumed in later stage. 5) The effects of oxygen consumption, glycolysis and glycogen synthesis were not influenced in the concentration of $10^{-5}g/ml$ of chloroquine phosphate, whereas hexachlorophene and dithiazanine inhibited markedly in same concentration, and the former was more potent than the latter.

  • PDF

당원 축적병 9D (GSD9D) 환자의 신규 PHKA1 돌연변이 (A Novel PHKA1 Mutation in a Patient with Glycogen Storage Disease Type IXD)

  • 김혜진;남수현;김상범;정기화;최병옥
    • 생명과학회지
    • /
    • 제30권8호
    • /
    • pp.672-679
    • /
    • 2020
  • 원위 근병증은 원위 근육의 퇴행성 질환이며 임상적, 유전적으로 이질적인 그룹이다. 당원 축적병 9D (GSD9D)는 원위 근병증 중 하나이며, 근육의 포스포릴라아제키나아제(phosphorylase kinase) 결핍을 특징으로 하는 대사 근병증이다. GSD9D 환자는 운동 후 근육 약화, 근육 변성, 경련과 비정상적인 근육통 및 근육 경직이 발생될 수 있다. GSD9D는 글리코겐 대사의 주요 조절 효소 인 근육 포스포릴라아제키나아제의 알파 소단위를 암호화하는 PHKA1 유전자의 돌연변이로 유발된다. 이 연구에서 우리는 한국인 GSD9D 가족에 대해 PHKA1 유전자에서 c.3314T> C (p.I1105T) 돌연변이를 동정하였다. 이 돌연변이는 이전에 어떠한 돌연변이 데이터베이스에서도 보고되지 않았으며 500명의 건강한 대조군에서도 발견되지 않았다. 이 돌연변이 영역은 다양한 다른 종 내에서 잘 보존되었으며 in silico 분석에서 돌연변이가 병원성일 가능성이 있다고 예측했다. 현재까지 PHKA1 유전자에는 보고된 병원성 돌연변이가 7개뿐이며 한국에서는 보고된 사례가 없다. 따라서 이 연구는 한국 GSD9D 환자의 첫번째 사례이다. 또한 이 연구는 이전에 보고된 환자와 한국 환자의 임상 증상과 병리 상태를 비교하고 설명하고자 하였다. 아울러 우리는 본 연구가 GSD9D의 분자 진단에 유용하게 활용될 것으로 기대한다.

Feather corticosterone 분석을 통한 일반농장과 동물복지농장의 스트레스 비교 (Comparison of Stress in General Farms and Animal Welfare Farms Using Feather Corticosterone Analysis)

  • 김종륜;최호성;심관섭
    • 생명과학회지
    • /
    • 제32권9호
    • /
    • pp.706-711
    • /
    • 2022
  • 육계는 도축 전에 다양한 스트레스에 노출되며, 스트레스는 육계의 복지 수준과 도체 품질에 영향을 미친다. 따라서 농장의 사육 시스템은 육계의 스트레스를 감소시키고 동물복지를 향상시키기 위해서 매우 중요하다. 본 연구는 일반농장과 동물복지농장의 차이를 평가하고 스트레스를 측정하기 위한 지표로 feather corticosterone를 제시하기 위해 수행되었다. 육계의 스트레스 수준과 에너지 대사를 나타내는 HSP 70, glycogen, L-lactate와 함께 feather과 serum에서 추출한 corticosterone를 분석하기 위해 도축장에서 28일 된 육계의 샘플(혈액, 깃털, 근육)을 수집했다. Feather corticosterone은 일반농장군이 복지농장군보다 유의하게 (p<0.01) 높았으나 serum corticosterone에서는 유의성이 없었다. HSP70은 muscle과 feather에서 유의적인 차이가 없었다. Glycogen은 일반농장군이 복지농장군보다 유의하게 높았으나(p<0.01), L-lactate에서는 차이가 없었다. 본 연구 결과는 feather corticosterone 이 일반농장과 동물복지농장의 차이를 평가하고 장기간의 스트레스를 평가할 수 있는 지표임을 시사한다.

Gynostemma pentaphyllum extract and Gypenoside L enhance skeletal muscle differentiation and mitochondrial metabolism by activating the PGC-1α pathway in C2C12 myotubes

  • Kim, Yoon Hee;Jung, Jae In;Jeon, Young Eun;Kim, So Mi;Oh, Tae Kyu;Lee, Jaesun;Moon, Joo Myung;Kim, Tae Young;Kim, Eun Ji
    • Nutrition Research and Practice
    • /
    • 제16권1호
    • /
    • pp.14-32
    • /
    • 2022
  • BACKGROUND/OBJECTIVES: Peroxisome proliferator-activated receptor-gamma co-activator-1α (PGC-1α) has a central role in regulating muscle differentiation and mitochondrial metabolism. PGC-1α stimulates muscle growth and muscle fiber remodeling, concomitantly regulating lactate and lipid metabolism and promoting oxidative metabolism. Gynostemma pentaphyllum (Thumb.) has been widely employed as a traditional herbal medicine and possesses antioxidant, anti-obesity, anti-inflammatory, hypolipemic, hypoglycemic, and anticancer properties. We investigated whether G. pentaphyllum extract (GPE) and its active compound, gypenoside L (GL), affect muscle differentiation and mitochondrial metabolism via activation of the PGC-1α pathway in murine C2C12 myoblast cells. MATERIALS/METHODS: C2C12 cells were treated with GPE and GL, and quantitative reverse transcription polymerase chain reaction and western blot were used to analyze the mRNA and protein expression levels. Myh1 was determined using immunocytochemistry. Mitochondrial reactive oxygen species generation was measured using the 2'7'-dichlorofluorescein diacetate assay. RESULTS: GPE and GL promoted the differentiation of myoblasts into myotubes and elevated mRNA and protein expression levels of Myh1 (type IIx). GPE and GL also significantly increased the mRNA expression levels of the PGC-1α gene (Ppargc1a), lactate metabolism-regulatory genes (Esrra and Mct1), adipocyte-browning gene fibronectin type III domain-containing 5 gene (Fndc5), glycogen synthase gene (Gys), and lipid metabolism gene carnitine palmitoyltransferase 1b gene (Cpt1b). Moreover, GPE and GL induced the phosphorylation of AMP-activated protein kinase, p38, sirtuin1, and deacetylated PGC-1α. We also observed that treatment with GPE and GL significantly stimulated the expression of genes associated with the anti-oxidative stress response, such as Ucp2, Ucp3, Nrf2, and Sod2. CONCLUSIONS: The results indicated that GPE and GL enhance exercise performance by promoting myotube differentiation and mitochondrial metabolism through the upregulation of PGC-1α in C2C12 skeletal muscle.

Manipulation of Tissue Energy Metabolism in Meat-Producing Ruminants - Review -

  • Hocquette, J.F.;Ortigues-Marty, Isabelle;Vermorel, M.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제14권5호
    • /
    • pp.720-732
    • /
    • 2001
  • Skeletal muscle is of major economic importance since it is finally converted to meat for consumers. The increase in meat production with low costs of production may be achieved by optimizing muscle growth, whereas a high meat quality requires, among other factors, the optimization of intramuscular glycogen and fat stores. Thus, research in energy metabolism aims at controling muscle metabolism, but also liver and adipose tissue metabolism in order to optimize energy partitioning in favour of muscles. Liver is characterized by high anabolic and catabolic rates. Metabolic enzymes are regulated by nutrients through short-term regulation of their activities and long-term regulation of expression of their genes. Consequences of liver metabolic regulation on energy supply to muscles may affect protein deposition (and hence growth) as well as intramuscular energy stores. Adipose tissues are important body reserves of triglycerides, which result from the balance between lipogenesis and lipolysis. Both processes depend on the feeding level and on the nature of nutrients, which indirectly affect energy delivery to muscles. In muscles, the regulation of rate-limiting nutrient transporters, of metabolic enzyme activities and of ATP production, as well as the interactions between nutrients affect free energy availability for muscle growth and modify muscle metabolic characteristics which determine meat quality. The growth of tissues and organs, the number and the characteristics of muscle fibers depend, for a great part, on early events during the fetal life. They include variations in quantitative and qualitative nutrient supply to the fetus, and hence in maternal nutrition. During the postnatal life, muscle growth and characteristics are affected by the age and the genetic type of the animals, the feeding level and the diet composition. The latter determines the nature of available nutrients and the rate of nutrient delivery to tissues, thereby regulating metabolism. Physical activity at pasture also favours the orientation of muscle metabolism, towards the oxidative type. Consequently, breeding systems may be of a great importance during the postnatal life. Research is now directed towards the determination of individual tissue and organ energy requirements, a better knowledge of nutrient partitioning between and within organs and tissues. The discovery of new molecules (e. g. leptin), of new molecular mechanisms and of more powerful techniques (DNA chips) will help to achieve these objectives. The integration of the different levels of knowledge will finally allow scientists to formulate new types of diets adapted to sustain a production of high quality meat with lower costs of production.

인삼(人蔘) 및 오가피(五加皮) 장기투여(長期投與)가 가계대사(家鷄代謝)에 미치는 영향(影響) (The effects of long-term administration of Panax ginseng and Acanthopanax on the metabolism of hens)

  • 오세기;홍사악
    • 대한약리학회지
    • /
    • 제12권2호
    • /
    • pp.21-32
    • /
    • 1976
  • The influence of Panax ginseng and Acanthopanax on the metabolism of hens are studied experimentally. The hens are fed with three dose levels of ethanol extract of Panax ginseng and Acanthopanax for 24 months and for comparing, there are control hens and young control hens. The results are as follows. 1. Hemoglobin, hematocrit and erythrocyte sedimentation rate values of hens fed with Panax ginseng and Acanthopanax are not significantly. different from control group. There is tendency of decreasing of erythrocyte sedimentation rate with aging. 2. The serum total lipid values of hens fed with Panax ginseng and Acanthopanax have tendency of increment compared with control group, especially in large dose treated hens of Panax ginseng and it is statistically significant. The serum cholesterol levels of all treated hens seem to be increased without significance statistically. Serum triglyceride level is higher in young hens compared with aged, and Panax ginseng and Acanthopanax treated hens reveal much higher values than control hens. So the increment of triglyceride levels in the Panax ginseng and Acanthopanax treated hens may be mainly contribute to increment of total lipid content. 3. Long-term administration of Panax ginseng and Acanthopanax seem to increase total serum protein content. Such increment seem to be due to the increment of globulin, especially gamma globulin, which is statistically significant in the large dose group of Acanthopanax. There are no differences of serum albumin levels of all the groups. 4. The blood glucose level and the glycogen content of liver and muscle have no difference among all the experimental groups. To summarize the results, the effects of Panax ginseng and Acanthopanax on the metabolism of hens have similarity in the majority of data of this experiment, and seem to be mainly influence to lipid and protein metabolisms by increasing serum triglyceride and globulin, especially gamma globulin.

  • PDF

북극 지의류 Cladonia종에서 분리한 Caballeronia sordidicola균주 PAMC 26577의 유전체 서열 분석 (Genome sequence of Caballeronia sordidicola strain PAMC 26577 isolated from Cladonia sp., an Arctic lichen species)

  • 양정안;홍순규;오현명
    • 미생물학회지
    • /
    • 제53권2호
    • /
    • pp.141-143
    • /
    • 2017
  • Caballeronia sordidicola 균주 PAMC 26577은 다산 기지 근처에서 채집된 지의류인 Cladonia 종에서 분리되었다. Illumina 방식으로 분석한 균주 PAMC 26577의 초안 유전체 서열은 182개의 콘티그로 이루어졌으며, N50값은 159,226 염기쌍 길이에 해당하였다. 초안 유전체로 총 8,334,211 염기쌍을 확인하였으며, 59.4% G+C 함량을 나타냈다. 유전체는 단백질을 코드하지 않는 8개의 rRNA 유전자와 51개의 tRNA 유전자를 포함하였다. 8,065개의 단백질 유전자는 기본 대사 과정뿐 아니라 부탄올/부티르산 생합성, 폴리하이드록시부티르산 대사, serine cycle methylotrophy 및 글라이코겐 대사 유전자들을 가지고 있었다. 2백개 이상의 막 전달 단백질은, 인산화 전달 시스템과 TRAP 전달시스템이 부재하였다. PAMC 26577은 CRISPR 관련 서열 및 단백질이 없었으며, 파아지 유전자의 감염흔적으로 인한 11개의 파아지 관련 유전자를 찾아낼 수 있었다.

Distinctive Features of Hepatic Steatosis in Children: Is It Primary or Secondary to Inborn Errors of Metabolism?

  • Karhan, Asuman Nur;Hizarcioglu-Gulsen, Hayriye;Gumus, Ersin;Akcoren, Zuhal;Demir, Hulya;Saltik-Temizel, Inci Nur;Orhan, Diclehan;Ozen, Hasan
    • Pediatric Gastroenterology, Hepatology & Nutrition
    • /
    • 제24권6호
    • /
    • pp.518-527
    • /
    • 2021
  • Purpose: The incidence of hepatic steatosis among children has been increasing; however, data distinguishing simple steatosis from a more complex disorder are lacking. Methods: This study identified the etiologies resulting in hepatic steatosis through a retrospective review of pediatric liver biopsies performed in the last 10 years. A total of 158 patients with hepatic steatosis proven by histopathological evaluation were enrolled in the study, and baseline demographic features, anthropometric measurements, physical examination findings, laboratory data, ultrasonographic findings, and liver histopathologies were noted. Results: The two most common diagnoses were inborn errors of metabolism (IEM) (52.5%) and nonalcoholic fatty liver disease/steatohepatitis (NAFLD/NASH) (29.7%). The three most common diseases in the IEM group were glycogen storage disorders, Wilson's disease, and mitochondrial disease. The rates of consanguineous marriage (75.6%; odds ratio [OR], 26.040) and positive family history (26.5%; OR, 8.115) were significantly higher (p=0.002, p<0.001, respectively) in the IEM group than those in the NAFLD/NASH group. Younger age (p=0.001), normal anthropometric measurements (p=0.03), increased aspartate aminotransferase levels (p<0.001), triglyceride levels (p=0.001), and cholestatic biochemical parameters with disrupted liver function tests, as well as severe liver destruction of hepatic architecture, cholestasis, fibrosis, and nodule formation, were also common in the IEM group. Conclusion: Parents with consanguinity and positive family history, together with clinical and biochemical findings, may provide a high index of suspicion for IEM to distinguish primary steatosis from the consequence of a more complex disorder.