scholarly journals Age-related augmentation of phosphorylase b kinase in hepatic tissue from the glycogen-storage-disease (gsd/gsd) rat

1986 ◽  
Vol 238 (3) ◽  
pp. 811-816 ◽  
Author(s):  
D G Clark ◽  
S D Neville ◽  
M Brinkman ◽  
P V Nelson ◽  
R J Illman ◽  
...  
Keyword(s):  
Food Deprivation ◽  
Glycogen Content ◽  
Storage Disease ◽  
Liver Weight ◽  
Liver Glycogen ◽  
Glycogen Storage ◽  
Hepatic Tissue ◽  
Blood Metabolites ◽  
Hepatic Glycogen ◽  
Age Related

The effects of food deprivation on body weight, liver weight, hepatic glycogen content, glycogenolytic enzymes and blood metabolites were compared in young and old phosphorylase b kinase-deficient (gsd/gsd) rats. Although the concentration of glycogen in liver from 9-week-old female gsd/gsd rats (730 mumol of glucose equivalents/g wet wt.) was increased by 7-8% during starvation, total hepatic glycogen was decreased by 12% after 24 h without food. In 12-month-old male gsd/gsd rats the concentration of liver glycogen (585 mumol of glucose equiv./g wet wt.) was decreased by 16% and total hepatic glycogen by nearly 40% after food deprivation for 24 h. Phosphorylase b kinase and phosphorylase a were present at approx. 10% of the control activities in 9-week-old gsd/gsd rats, but both enzyme activities were increased more than 3-fold in 12-month-old affected rodents. It is concluded that the age-related ability to mobilize hepatic glycogen appears to result from the augmentation of phosphorylase b kinase during maturation of the gsd/gsd rat.

scholarly journals Effects in vivo of food deprivation and 3-mercaptopicolinate in the glycogen-storage-disease (gsd/gsd) rat

1985 ◽  
Vol 231 (3) ◽  
pp. 755-759 ◽  
Author(s):  
D G Clark ◽  
M Brinkman ◽  
S D Neville ◽  
W D Haynes
Keyword(s):  
Blood Glucose ◽  
Food Deprivation ◽  
Intraperitoneal Injection ◽  
Storage Disease ◽  
Liver Glycogen ◽  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Glycogen Stores ◽  
Female Control

Intraperitoneal injection of 3-mercaptopicolinate into 24 h-food-deprived 27-week-old female control (GSD/GSD) rats lowered the concentration of circulating glucose by 66%, but glycerol and lactate concentrations were increased up to 3- and 4-fold respectively. In phosphorylase b kinase-deficient (gsd/gsd) rats the corresponding changes for blood glucose, lactate and glycerol were half those observed in the controls. Although the concentration of liver glycogen (approx. 12%, w/w) in the gsd/gsd rats was not altered during food deprivation, total hepatic glycogen was decreased by 17%. It is suggested that the gradual breakdown of the extensive hepatic glycogen stores during starvation assists in the maintenance of normoglycaemia in the gsd/gsd rat.

scholarly journals The Phenotypic and Genetic Spectrum of Glycogen Storage Disease Type VI

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1205
Author(s):  
Sarah Catharina Grünert ◽  
Luciana Hannibal ◽  
Ute Spiekerkoetter
Keyword(s):  
Liver Cirrhosis ◽  
Glycogen Storage Disease ◽  
Glycogen Phosphorylase ◽  
Storage Disease ◽  
Glycogen Storage Disease Type ◽  
Glycogen Storage ◽  
Disease Type ◽  
Hepatic Glycogen ◽  
Laboratory Findings ◽  
Poor Growth

Glycogen storage disease type VI (GSD VI) is an autosomal recessive disorder of glycogen metabolism due to mutations in the glycogen phosphorylase gene (PYGL), resulting in a deficiency of hepatic glycogen phosphorylase. We performed a systematic literature review in order to collect information on the clinical phenotypes and genotypes of all published GSD VI patients and to compare the data to those for GSD IX, a biochemically and clinically very similar disorder caused by a deficiency of phosphorylase kinase. A total of 63 genetically confirmed cases of GSD VI with clinical information were identified (median age: 5.3 years). The age at presentation ranged from 5 weeks to 38 years, with a median of 1.8 years. The main presenting symptoms were hepatomegaly and poor growth, while the most common laboratory findings at initial presentation comprised elevated activity of liver transaminases, hypertriglyceridemia, fasting hypoglycemia and postprandial hyperlactatemia. Liver biopsies (n = 37) showed an increased glycogen content in 89.2%, liver fibrosis in 32.4% and early liver cirrhosis in 10.8% of cases, respectively. No patient received a liver transplant, and one successful pregnancy was reported. Our review demonstrates that GSD VI is a disorder with broad clinical heterogeneity and a small number of patients with a severe phenotype and liver cirrhosis. Neither clinical nor laboratory findings allow for a differentiation between GSD VI and GSD IX. Early biochemical markers of disease severity or clear genotype phenotype correlations are missing. Given the overall benign and unspecific phenotype and the need for enzymatic or genetic analyses for confirmation of the diagnosis, GSD VI is likely underdiagnosed. With new treatment approaches in sight, early, pre-symptomatic diagnosis, especially with respect to hepatic cirrhosis, will become even more important.

Glycogen Storage Disease Previously Reported as Familial Hyperlipidemia

PEDIATRICS ◽  
1965 ◽  
Vol 36 (6) ◽  
pp. 956-956
Author(s):  
Smilja Jakovcic ◽  
Walter Fuhrmann ◽  
David Yi-Yung Hsia
Keyword(s):  
Histological Examination ◽  
Glycogen Storage Disease ◽  
Clinical Studies ◽  
Inborn Error ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Liver Biopsies ◽  
Familial Hyperlipidemia

In the April 1959 issue of this journal, under the title of "An Inborn Error of Lipid Metabolsim, "clinical studies were reported on two brothers with hyperlipidemia.1 Although these children presented several of the clinical and laboratory manifestations on glycogen storage disease of Von Gierke's type, this diagnosis was ruled out when histological examination of two liver biopsies done on one of the children at a three year's interval were reported as containing low or normal amounts of hepatic glycogen.

Contribution of net hepatic glycogenolysis to glucose production during the early postprandial period

1996 ◽  
Vol 270 (1) ◽  
pp. E186-E191 ◽  
Author(s):  
K. F. Petersen ◽  
T. Price ◽  
G. W. Cline ◽  
D. L. Rothman ◽  
G. I. Shulman
Keyword(s):  
Magnetic Resonance ◽  
Glycogen Content ◽  
Hepatic Glucose Production ◽  
Liver Volume ◽  
Glucose Production ◽  
Liver Glycogen ◽  
Whole Body ◽  
Hepatic Glycogen ◽  
Resonance Spectroscopy ◽  
13C Nuclear Magnetic Resonance

Relative contributions of net hepatic glycogenolysis and gluconeogenesis to glucose production during the first 12 h of a fast were studied in 13 healthy volunteers by noninvasively measuring hepatic glycogen content using 13C nuclear magnetic resonance spectroscopy. Rates of net hepatic glycogenolysis were calculated by multiplying the change in liver glycogen content with liver volume determined by magnetic resonance imaging. Rates of gluconeogenesis were calculated as the difference between rates of glucose production determined with an infusion of [6,6-2H]-glucose and net hepatic glycogenolysis. At 6 P.M. a liquid mixed meal (1,000 kcal; 60% as glucose) was given, to which [2-2H]glucose was added to trace glucose absorption. Hepatic glycogen content was measured between 11 P.M. and 1 A.M. and between 3 and 6 A.M. At 11 P.M. the concentration was 470 mM and it decreased linearly during the night. The mean liver volume was 1.47 +/- 0.06 liters. Net hepatic glycogenolysis (5.8 +/- 0.8 mumol.kg body wt-1.min-1) accounted for, on average, 45 +/- 6% and gluconeogenesis for 55 +/- 6% of the rate of whole body glucose production (12.6 +/- 0.6 mumol.kg body wt-1.min-1). In conclusion, this study shows that, even early in the phase of the postabsorptive period when liver glycogen stores are maximal, gluconeogenesis contributes approximately 50% to hepatic glucose production.

scholarly journals POLYCYSTIC OVARIES AND GLUCOSETOLERANCE IN HEPATIC GLYCOGEN STORAGE DISEASE

1993 ◽  
Vol 33 ◽  
pp. S14-S14 ◽  
Author(s):  
P I Lee ◽  
A Patel ◽  
P C Hindmarsh ◽  
C G D Brook ◽  
J V Leonard
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Polycystic Ovaries

Effects of maternal exercise on liver and skeletal muscle glycogen storage in pregnant rats

1991 ◽  
Vol 71 (3) ◽  
pp. 1015-1019 ◽  
Author(s):  
M. F. Mottola ◽  
P. D. Christopher
Keyword(s):  
Skeletal Muscle ◽  
Muscle Glycogen ◽  
Glycogen Content ◽  
Liver Glycogen ◽  
Glycogen Storage ◽  
Treadmill Running ◽  
Control Group ◽  
Skeletal Muscle Glycogen ◽  
Maternal Exercise ◽  
White Gastrocnemius

To examine the effects of maternal exercise on liver and skeletal muscle glycogen storage, female Sprague-Dawley rats were randomly divided into control, nonpregnant runner, pregnant nonrunning control, pregnant runner, and prepregnant exercised control groups. The exercise consisted of treadmill running at 30 m/min on a 10 degree incline for 60 min, 5 days/wk. Pregnancy alone, on day 20 of gestation, decreased maternal liver glycogen content and increased red and white gastrocnemius muscle glycogen storage above control values (P less than 0.05). In contrast, exercise in nonpregnant animals augmented liver glycogen storage and also increased red and white gastrocnemius glycogen content (P less than 0.05). By combining exercise and pregnancy, the decrease in liver glycogen storage in the pregnant nonexercised condition was prevented in the pregnant runner group and more glycogen was stored in both the red and white portions of the gastrocnemius than all other groups (P less than 0.05). Fetal body weight was greatest (P less than 0.05) in the pregnant runner group and lowest (P less than 0.05) in the prepregnant exercise control group. These results demonstrate that chronic maternal exercise may change maternal glycogen storage patterns in the liver and skeletal muscle with some alteration in fetal outcome.

scholarly journals Current status of hepatic glycogen storage disease in Japan: clinical manifestations, treatments and long-term outcomes

2013 ◽  
Vol 58 (5) ◽  
pp. 285-292 ◽  
Author(s):  
Jun Kido ◽  
Kimitoshi Nakamura ◽  
Shirou Matsumoto ◽  
Hiroshi Mitsubuchi ◽  
Toshihiro Ohura ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Clinical Manifestations ◽  
Glycogen Storage ◽  
Current Status ◽  
Hepatic Glycogen ◽  
Long Term Outcomes
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