We investigated the reproductive cycle with gonad developmental phases of Solen grandis by histological observations. Seasonal changes in biochemical components of the adductor muscle, visceral mass, foot muscle and mantle were studied by biochemical analysis, from January to December, 2005. The reproductive cycle of this species can be classified into five successive stages: early active stage (December to January), late active stage (January to March), ripe stage (March to July), partially spawned stage (June to July) and spent/inactive stage (July to December). Total protein content was the highest in the foot muscle, the content was high in January (early active stage), the lowest in April (ripe stage), and was the highest in August (partially spawned stage). In the visceral mass, total protein content began to increase in February (late active stage) and reached a maximum in March (ripe stage). Thereafter, it gradually decreased between June and July (partially spawned stage). There was a strong negative correlation in total protein contents between visceral mass and mantle (r = -0.594, p = 0.042). Meanwhile there was a positive correlation between the adductor muscle and foot muscle, the correlation was not statistically significant (r = 0.507, p = 0.093). Total lipid content was the highest in the visceral mass; it was more than 2 to 5-fold higher than that in the adductor muscle, foot muscle, and mantle. Monthly changes in total lipid content were also most dynamic in the visceral mass. It was relatively higher between January and February, showed a maximum in March (the ripe stage), decreased rapidly from April to July (ripe and partially spawned stage), and gradually decreased from September to December (spent/inactive stage). There was a strong positive correlation in total lipid content between foot muscle and adductor muscle (r = 0.639, p = 0.025). Tthough a negative correlation was found between visceral mass and mantle (r = -0.392), the correlation was not statistically significant (p = 0.208). Glycogen contents changed within relatively narrow range and were similar among different tissues. There was no statistically significant correlation in glycogen contents among tissues.
Journal of The Korean Society of Inherited Metabolic disease
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v.5
no.1
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pp.18-22
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2005
Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). This enzyme defect results in lysosomal glycogen accumulation in multiple tissues and cell types, with cardiac, skeletal, and smooth muscle cells the most seriously affected. Infantile-onset Pompe disease is uniformly lethal. Affected infants present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, followed by death from cardiorespiratory failure or respiratory infection, usually by 1 year of age. Late-onset forms is characterized by a lack of severe cardiac involvement and a less severe short-term prognosis. Enzyme replacement therapy for Pompe disease is intended to address directly the underlying metabolic defect via intravenous infusions of recombinant human GAA to provide the missing enzyme. We experienced one case of Pompe disease in 3-years old boy that has improved his exercise ability and cardiac function after GAA enzyme replacement therapy.
The purpose of this study was to investigate the effect of vitamin B6 deficiency on the concentration of energy metabolite in streptozotocin-induced diabetic rats. Thirty rats were fed a vitamin B6 deficient diet(-B6) or a control diet(+B6) for 5 weeks and then subdivided into 3 groups respectively ; base group, one day diabetic group and three day diabetic group. Diabetes of rats were induced by streptozotocin injection into the tail vein. Glucose, glycogen, protein, alanine, triglyceride and free fatty acids were compared in plasma, liver skeletal muscle of rats. Also, the total urinary nitrogen and glucose excretion were compared. Compared with +B6 rats, the increase of plasma glucose in -B6 rats due to the diabetes was smaller. After diabetes was induced, the level of plasma alamine was not changed in -B6 rats while increased significantly(p<0.05) in +B6 rats. The increase of urinary nitrogen excretion was smaller and the increase of muscle protein was larger in -B6 rats at the first day diabetes was induced. The levels of plasma free fatty acid and liver triglyceride were significantly (p<0.05) higher in -B6 rats after diabetes was induced. These results suggest that vitamin B6 deficiency may impair the adaptation of animals to the energy metabolism related due to a decrease of the body protein catabolism of fatty acid oxidation in diabetes and aggravate fatty liver which is one of the chronic complications of diabetes.
We conducted a series of investigations in order to elucidate role of nutritional status in regulating GLUT expression and energy metabolism in porcine muscle. Firstly, the role of mild undernutrition in regulating muscle GLUT gene expression and function was studied in growing pigs (3 wk of age) on a high (H) or low (L) food intake (H = 2L) at $35^{\circ}C$ or $26^{\circ}C$. Low food intake selectively upregulates GLUT1 and GLUT4 gene expression; mRNA levels were elevated in longissimus dorsi (L. dorsi) and rhomboideus muscles but not in diaphragm or cardiac muscles. Our next step was to determine whether dietary lysine, a major primary limiting amino acid in diets for pigs, affects muscle GLUT4 expression. Pigs of 6 wk of age were pair-fed a control or low lysine (LL) diet. The control diet contained optimal amounts of all essential amino acids, including 1.15% lysine. The LL diet was similar but contained only 0.70% lysine. GLUT4 mRNA expression was upregulated by the LL diet in L. dorsi and rhomboideus muscles, whereas that in cardiac muscle was unaffected. GLUT4 protein abundance was also higher in rhomboideus muscle of animals on the LL diet. We conducted another investigation in order to elucidate effects of the LL diet on post-GLUT4 glucose metabolism. Activity of hexokinase was unaffected by dietary lysine levels while that of citrate synthase was higher both in L. dorsi and rhomboideus muscles of pigs fed on the LL diet. Glucose 6-phosphate content was higher in L. dorsi msucle in the LL group. Glycogen content was higher both in L. dorsi and rhomboideus muscles in the LL group. Further, we determined the effects of dietary lysine levels on accumulation of intramuscular fat (IMF) in L. dorsi muscle of finishing pigs. A low lysine diet (lysine content was 0.40%) meeting approximately 70% of the requirement of lysine was given to finishing pigs for two months. IMF contents in L. dorsi of the pigs given the low lysine diet were twice higher than those of the pigs fed on a control diet (lysine content was 0.65%). Finally, we proved that a well known effect of breadcrumbs feeding to enhance IMF of finishing pigs could be attributed to shortage of amino acids in diets including breadcrumbs.
Hocquette, J.F.;Ortigues-Marty, Isabelle;Vermorel, M.
Asian-Australasian Journal of Animal Sciences
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v.14
no.5
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pp.720-732
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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.
Lee, Ju Young;Shim, Jeong Ok;Yang, Hye Ran;Chang, Ju Young;Shin, Choong Ho;Ko, Jae Sung;Seo, Jeong Kee;Kim, Woo Sun;Kang, Gyeong Hoon;Song, Jeong Han;Kim, Jong Won
Clinical and Experimental Pediatrics
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v.51
no.6
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pp.650-654
/
2008
Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.
Park, Sun-Min;Ahn, Seung-Hee;Choi, Mi-Kyung;Choi, Soo-Ran;Choi, Soo-Bong
Korean Journal of Food Science and Technology
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v.33
no.5
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pp.619-625
/
2001
We determined whether the supplementation of Polygonatum Odoratum (Mill) Druce (POD) extract had a good effect on insulin resistance in peripheral tissues of 90% pancreatectomized (Px) and sham-operated (Sham) male Sprague Dawley rats. Px and Sham rats were divided into two groups; one group daily consumed 0.3 g of POD extracts per 1 ㎏ body weight for two months, and the other group had a placebo. All rats freely consumed a 40% fat diet. At the end of the experiment, a euglycemic hyperinsulinemic (EH) clamp was performed in a fasting, awake, and unstressed state to determine insulin resistance. At EH clamp, body weights were higher in Sham rats than Px rats, and serum glucose levels of baseline were affected by diabetic status and POD administration. Serum insulin concentrations were higher in Sham rats than Px rats, and POD administration decreased them in Sham rats compared to P. Glucose disposal rates in peripheral tissues increased with POD in both Px (n=10) and Sham (n=10) rats. But glycogen deposits in soleus muscle increased with POD administration in Px and Sham rats, and total glycogen synthase activity and fraction velocity were higher in POD groups. Triglyceride contents in quadriceps muscles decreased with POD in Px rats. In conclusions, POD improves insulin resistance by enhancing glucose utilization with increasing glycogen deposit and decreasing triglyceride contents in muscles.
Kim, Seung-Suk;Koo, Jung-Hoon;Kwon, In-Su;Oh, Yoo-Sung;Lee, Sun-Jang;Kim, Eung-Joon;Kim, Won-Kyu;Lee, Jin;Cho, Joon-Yong
Nutrition Research and Practice
/
v.5
no.3
/
pp.205-213
/
2011
Exercise training (ET) and selenium (SEL) were evaluated either individually or in combination (COMBI) for their effects on expression of glucose (AMPK, PGC-$1{\alpha}$, GLUT-4) and lactate metabolic proteins (LDH, MCT-1, MCT-4, COX-IV) in heart and skeletal muscles in a rodent model (Goto-Kakisaki, GK) of diabetes. Forty GK rats either remained sedentary (SED), performed ET, received SEL, ($5\;{\mu}mol{\cdot}kg$ body $wt^{-1}{\cdot}day^{-1}$) or underwent both ET and SEL treatment for 6 wk. ET alone, SEL alone, or COMBI resulted in a significant lowering of lactate, glucose, and insulin levels as well as a reduction in HOMA-IR and AUC for glucose relative to SED. Additionally, ET alone, SEL alone, or COMBI increased glycogen content and citrate synthase (CS) activities in liver and muscles. However, their effects on glycogen content and CS activity were tissue-specific. In particular, ET alone, SEL alone, or COMBI induced upregulation of glucose (AMPK, PGC-la, GLUT-4) and lactate (LDH, MCT-1, MCT-4, COX-IV) metabolic proteins relative to SED. However, their effects on glucose and lactate metabolic proteins also appeared to be tissue-specific. It seemed that glucose and lactate metabolic protein expression was not further enhanced with COMBI compared to that of ET alone or SEL alone. These data suggest that ET alone or SEL alone or COMBI represent a practical strategy for ameliorating aberrant expression of glucose and lactate metabolic proteins in diabetic GK rats.
This study was designed to examine the effects of Salicornia herbacea L. (glasswort: GW) on the lipid peroxidation and mineral levels in diabetic rats. Diabetes mellitus was induced in male Sprague-Dawley rats weighing 200-220 g by an injection of streptozotocin (STZ) dissolved in a citrate buffer into the tail vein at a dose of 45 mg/kg of body weight. Sprague-Dawley rats were fed an AIN-93 recommended diet and the experimental groups were fed a modified diet containing 10% and 20% of glasswort powder for 4 weeks. The experimental groups were divided into 6 groups which consisted of normal (N)-control group, N-GW 10% and N-GW 20% treated groups, STZ-control, STZ-GW 10% and STZ-GW 20% treated groups. The rats' liver and muscle glycogen, liver and kidney protein, cholesterol and triglyceride (TG) in liver, malondialdehyde (MDA) in liver and kidney values were measured, along with the hepatic of chromium (Cr), iron (Fe), and zinc (Zn) content. The liver glycogen levels was significantly affected in N-GW 20% group among all the experimental groups. The liver MDA levels of the STZ-GW 10% and STZ-GW 20% groups were significantly lower than for the STZ-control group. There were significant differences between the N-control group and the STZ-control group in the hepatic of Zn levels. The hepatic of Cr levels in the N-GW 20% and STZ-GW 10% and STZ-GW 20% groups were significanly higher than for the each control groups. These results exhibited dose related effect of glasswort and it may have favorable influence on lipid peroxidation in the liver.
The hypoglycemic effects of butanol(BuOH) fraction of Polygonatum odoratum with vitamin E in streptozotocin(STZ)-induced diabetic rats were investigated. Sprague-Dawley rats weighing 200-230g were devided into five groups, and four groups induced diabetes mellitus by the STZ injection(45mg/kg b.w.) into the tail vein : Normal, diabetic-control, and three diabetic experimental groups(p. odoratum group, P. od-vit. E group and Vit. E group). All groups were fed on a AIN-76 diet, and the experimental groups were orally administered with the BuOH fraction of Polygonatum odoratum(500mg/kg b.w.) and vitamin E(10mg/kg b.w.) for 14 days. The body weight, diet intake and organ weights were monitored. The plasma levels of glucose, insulin, cholesterol, triglyceride, HDL-cholesterol and aspartate and alanine aminotransferase activities were analyzed. The levels of glycogen in liver and muscle, cholesterol in liver were determined. The malondialdehyde(MDA) levels in liver, kidney and lung were assayed. The body weight loss was seen in P. odoratum group, P. od-vit. E group, Vit. E group and diabetic control group, while the loss in P. odoratum group was much less than that in the diabetic control group. The plasma glucose levels were significantly lowered in P. odoratum group compared to diabetic control group. The plasma insulin levels were noticeably higher in P. odoratum and Vit. E groups. The rats in P. odoratum and P. od-vit. E group showed higher liver glycogen levels than in the diabetic control group. The MDA levels in liver, kidney and lung were also significantly reduced in P. od-vit. E and Vit. E groups compared to the diabetic control group. The results suggest that the administration of BuOH fraction of Polygonatum odoratum along with vitamin E reduced blood glucose levels and peroxidative tissue damage in STZ-induced diabetic rats, showing the possibility of preventive and therapeutic use of the wild edible plant to the diabetes mellitus. (Korean J Nutrition 31(9) : 1385-1393, 1998)
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