AnatomyDiseasesChemicals and Drugs
Glycogen Storage Disease Type IGlycogenGlycogen SynthaseLiver GlycogenGlycogen Synthase Kinase 3Glycogen PhosphorylaseGlycogen Synthase KinasesPhosphorylasesGlycogen Storage DiseaseGlycogen Debranching Enzyme SystemGlucose-6-PhosphateGlucoseGlucosephosphatesGlycogen Storage Disease Type IIPhosphorylase bPhosphorylase aGlycogen Phosphorylase, Liver FormGlycogen Phosphorylase, Muscle FormUridine Diphosphate GlucoseLiverMuscle, Skeletal
PhosphorylaseEnzymeBrain glycogen levelsAccumulationMetabolismStarchDeficiencyHepatic glycogenForm of glucoseIncluding glycogen synthaseEnzymesDepletionHypoglycemiaGram of glycogenAmount of glycogenAmounts of glycogenMoleculesBlood glucoseReleases glycogenTissuesGSDsExcessDiseasesMusclesMoleculeMuscleDepositionCarbohydratesGlucagonGlycosidic bondSupercompensationCarbohydrateLinkagesConcentrationCleaveStore of glucoseBiochemicalPolymerMolecularClinicalVivoGramsContentTypeDetection
Phosphorylase20
Enzyme22
  • Insulin also activates the enzyme glycogen synthase, which synthesizes glycogen by adding one glucose at a time to the glycogen chain. (tripod.com)
  • The GBE1 gene provides instructions for making the glycogen branching enzyme. (medlineplus.gov)
  • This enzyme is involved in the production of glycogen, which is a major source of stored energy in the body. (medlineplus.gov)
  • GBE1 gene mutations that cause GSD IV lead to a shortage (deficiency) of the glycogen branching enzyme. (medlineplus.gov)
  • Generally, the severity of the disorder is linked to the amount of functional glycogen branching enzyme that is produced. (medlineplus.gov)
  • The diagnosis is established in a proband by the demonstration of glycogen branching enzyme (GBE) deficiency in liver, muscle, or skin fibroblasts or the identification of biallelic pathogenic variants in GBE1 on molecular genetic testing. (nih.gov)
  • A glycogen storage disease (GSD) results from an enzyme defect. (medscape.com)
  • thus, an enzyme deficiency results in glycogen accumulation in specific tissues. (medscape.com)
  • Glycogen storage disease type IV (GSD IV), or Andersen disease, is an autosomal recessive disorder caused by mutations in the gene-encoding glycogen-branching enzyme necessary for normal glycogen metabolism. (medscape.com)
  • A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen , which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. (wikidoc.org)
  • [1] When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. (wikidoc.org)
  • When glucosyltransferase and glucosidase are catalyzed by distinct enzymes, "glycogen debranching enzyme" usually refers to the glucosidase enzyme . (wikidoc.org)
  • Indicated in combination with cipaglucosidase alfa, a hydrolytic lysosomal glycogen-specific enzyme, for adults with late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency) weighing ≥40 kg who are not improving on their current enzyme replacement therapy (ERT). (medscape.com)
  • Glycogen storage disease type III (GSD-III) is an inborn error of glycogen metabolism caused by a deficiency of the glycogen debranching enzyme, amylo-1,6-glucosidase,4-α-glucanotransferase (AGL). (degruyter.com)
  • Glycogen storage diseases (GSDs) are a group of inborn errors of metabolism, typically caused by enzyme defects, resulting in a buildup of glycogen in the liver, muscles, and other organs. (arupconsult.com)
  • A glycogen storage disease (GSD) is the result of an enzyme defect. (medscape.com)
  • Glycogen synthase kinase-3 (GSK-3) a magic enzyme: it's role in diabetes mellitus and glucose homeostasis, interactions with fluroquionlones. (scielo.br)
  • This disease is mainly regulated by various enzymes and hormones among which Glycogen synthase kinase-3 (GSK-3) is a principle enzyme and insulin is the key hormone regulating it. (scielo.br)
  • One Unit of isoamylase activity is defined as the amount of enzyme required to release one µmole of glucose reducing sugar equivalents per minute from oyster glycogen (10 mg/mL) in MES buffer (100 mM, pH 6.0) plus 1 mM CaCl 2 at 40 o C. (megazyme.com)
  • Pure Isoamylase (Glycogen 6-glucanohydrolase) ( Flavobacterium odoratum ) for use in biochemical enzyme assays and in vitro diagnostic analysis. (megazyme.com)
  • A. α-1,6-glucosidase (a glycogen debranching enzyme) deficiency is most commonly associated with Cori disease (type III). (boardvitals.com)
  • It is caused by deficient activity of glycogen debranching enzyme, which is a key enzyme in glycogen degradation. (qxmd.com)
Brain glycogen levels2
  • Our findings suggest that plasma glycogenic amino acids are sensitive indicators of brain glycogen levels in endurance exercise. (frontiersin.org)
  • When subjected to hypoglycemia, these rats maintained brain electrical activity 91 ± 14 min longer than rats with normal brain glycogen levels and showed markedly reduced neuronal death. (aspetjournals.org)
Accumulation8
  • Because rodents typically feed at night, the degree of glycogen accumulation within hepatocytes is typically highest in the early morning hours and wanes throughout the day. (nih.gov)
  • The morphologic appearance of glycogen in hepatocytes is distinctive, allowing glycogen accumulation or depletion to be readily diagnosed in most cases. (nih.gov)
  • In some cases, particularly when differences in the amount of glycogen are accompanied by other cytoplasmic changes, glycogen accumulation or depletion may not be easily discerned. (nih.gov)
  • If, in contrast to the control animals, there is a treatment-related increase in glycogen in the hepatocytes, glycogen accumulation should be diagnosed in the treated animals and assigned a severity grade. (nih.gov)
  • Carbohydrate metabolic pathways are blocked, leading to excess glycogen accumulation in affected tissues and/or disturbances in energy production. (medscape.com)
  • Although this brain adaptation is likely induced due to the accumulation of acute endurance exercise-induced brain glycogen supercompensation, its molecular mechanisms and biomarkers are unidentified. (frontiersin.org)
  • The accumulation of glycogen results in a variety of clinical manifestations, such as exercise intolerance, severe muscle cramps, muscle swelling, weakness, and fatigue. (boardvitals.com)
  • Because free glucose is the product of the hepatic glucose-6 phosphatase reaction, either type leads to accumulation of liver glycogen, accompanied by fasting hypoglycemia. (lu.se)
Metabolism4
  • Techniques to measure the activity of key enzymes of glycogen metabolism are presented, using a simple spectrophotometer operating in the visible range. (jove.com)
  • The protocols are significant, because they avoid the use of radioactive isotopes and remove a barrier that may prevent many workers from studying enzymes of glycogen metabolism. (jove.com)
  • In rodents, 4 weeks of chronic moderate exercise-enhancing endurance and cognition increases brain glycogen in the hippocampus and cortex, which is an adaption of brain metabolism achieved through exercise. (frontiersin.org)
  • To date, however, the understanding of human brain glycogen metabolism is still less clear. (frontiersin.org)
Starch2
  • Glycogen is an analogue of starch, a glucose polymer that functions as energy storage in plants. (wikipedia.org)
  • Glucose is converted into its storage form, glycogen, which is a long string of single sugars stored as a starch, a complex sugar. (encyclopedia.com)
Deficiency12
Hepatic glycogen7
  • Impaired net hepatic glycogen synthesis in insulin-dependent diabetic subjects during mixed meal ingestion. (nih.gov)
  • Hepatic glycogen concentration was measured in six subjects with insulin-dependent diabetes mellitus (IDDM) and nine weight-matched control subjects using 13C nuclear magnetic resonance spectroscopy during a day in which three isocaloric mixed meals were ingested. (nih.gov)
  • glycogen) pathways of hepatic glycogen synthesis were also assessed using [1-13C]glucose in combination with acetaminophen to noninvasively sample the hepatic UDP-glucose pool. (nih.gov)
  • Mean fasting hepatic glycogen content was similar in the two groups. (nih.gov)
  • After each meal, hepatic glycogen content increased, peaking 4-5 h after the meal in both groups. (nih.gov)
  • In conclusion, under mixed meal conditions, subjects with poorly controlled IDDM have a major defect in net hepatic glycogen synthesis and augmented hepatic gluconeogenesis. (nih.gov)
  • Effect of growth hormone treatment on hypoglycemia in a patient with both hepatic glycogen synthase and isolated growth hormone deficiencies. (medscape.com)
Form of glucose4
Including glycogen synthase1
Enzymes2
Depletion8
  • The glycogen supercompensation effect (achieving supraphysiological glycogen levels due to carbohydrate depletion followed by loading) was first demonstrated in 1967. (tripod.com)
  • Endurance athletes benefit from glycogen supercompensation because fatigue in events lasting longer than one hour is related primarily to glycogen depletion. (tripod.com)
  • Glycogen supercompensation is probably of little use to power athletes since fatigue in these events is not related to glycogen depletion and the weight gain may be a liability. (tripod.com)
  • There is a notable depletion of glycogen within hepatocytes. (nih.gov)
  • glycogen depletion should be diagnosed in the treated animals and given a severity grade. (nih.gov)
  • Consequently, interpretation of glycogen depletion should take food consumption and general animal health into consideration. (nih.gov)
  • Endurance exercise induced fatigue factors such as a decrease in blood glucose, an increase in blood lactate, and the depletion of muscle glycogen, but those parameters recovered to basal levels within 6 h after exercise. (frontiersin.org)
  • Hepatic changes were consistent with glycogen depletion. (cdc.gov)
Hypoglycemia5
  • Meticulous adherence to a dietary regimen to maintain a euglycemic state and prevent the formation of excessive glycogen may reduce the liver size, prevent hypoglycemia, reduce symptoms, and allow growth and development. (medscape.com)
  • Individuals with glycogen storage disease type III present with hepatomegaly, hypoglycemia, hyperlipidemia, and growth retardation. (qxmd.com)
  • Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0. (medscape.com)
  • the deficiencies may occur in the liver or muscles and cause hypoglycemia or deposition of abnormal amounts or types of glycogen (or its intermediate metabolites) in tissues. (msdmanuals.com)
  • With an unavailability of fatty acids, the body resorts to using glucose and glycogen for its energy, which then leads to hypoglycemia. (cdc.gov)
Gram of glycogen1
  • Glycogen in muscle, liver, and fat cells is stored in a hydrated form, composed of three or four parts of water per part of glycogen associated with 0.45 millimoles (18 mg) of potassium per gram of glycogen. (wikipedia.org)
Amount of glycogen1
  • The amount of glycogen stored in the body mostly depends on oxidative type 1 fibres, physical training, basal metabolic rate, and eating habits. (wikipedia.org)
Amounts of glycogen2
  • Small amounts of glycogen are also found in other tissues and cells, including the kidneys, red blood cells, white blood cells, and glial cells in the brain. (wikipedia.org)
  • In the context of a toxicity study, some treated animals may have a depressed appetite and will have decreased amounts of glycogen in the liver, in contrast to the concurrent controls. (nih.gov)
Molecules5
  • Glucose molecules are added to the chains of glycogen as long as both insulin and glucose remain plentiful. (wikipedia.org)
  • Glycogen is essentially a long chain of glucose (sugar) molecules that are attached end to end with an occasional cross linkage. (tripod.com)
  • In order to derive energy from glycogen the body must liberate individual glucose molecules to use for energy production. (tripod.com)
  • Abnormal glycogen molecules called polyglucosan bodies accumulate in cells, leading to damage and cell death. (medlineplus.gov)
  • Value calculated by dividing halftime of spontaneous polysaccharide hydrolysis at 25°C, 4.7×10^6 years (BNID 105353 ) by number of glucose molecules in glycogen. (harvard.edu)
Blood glucose4
  • in fasting individuals, blood glucose is maintained constant at this level at the expense of glycogen stores in the liver and skeletal muscle. (wikipedia.org)
  • however, the breakdown of muscle glycogen impedes muscle glucose uptake from the blood, thereby increasing the amount of blood glucose available for use in other tissues. (wikipedia.org)
  • The process by which liver glycogen is converted into blood glucose is related to the actions of the pancreas, which monitors blood glucose levels. (encyclopedia.com)
  • Glycogen breakdown is highly regulated in the body, especially in the liver , by various hormones including insulin and glucagon , to maintain a homeostatic balance of blood-glucose levels. (wikidoc.org)
Releases glycogen2
Tissues2
  • Glycogen levels in the tissues of all metal-exposed carps significantly decreased. (tubitak.gov.tr)
  • Diagnosis of glycogen storage diseases is suspected by history, examination, and detection of glycogen and intermediate metabolites in tissues by MRI or biopsy. (msdmanuals.com)
GSDs2
Excess5
  • When muscle and liver glycogen levels are replenished, the excess glucose is burned preferentially to fat. (tripod.com)
  • In other words, consuming excess carbohydrates (more than is needed to replenish glycogen stores) shifts the body from burning primarily fats at rest to consuming primarily carbohydrates at rest. (tripod.com)
  • When the intake of carbohydrates exceeds that which can be stored and converted to energy as glycogen or glucose, the body will store the excess carbohydrates as fat, often leading to weight gain. (encyclopedia.com)
  • Glycogen is the form in which most of the body's excess glucose is stored. (jrank.org)
  • The excess glucose that is converted into glycogen may be used by the immune system when nutrients are low. (concordia.ca)
Diseases6
  • Therefore, brain glycogen dynamics during and following exercise can be a valuable parameter for exercises as training/conditioning for athletes and/or a therapeutic strategy for neurodegenerative diseases. (frontiersin.org)
  • How well do you know Glycogen Storage Diseases (GSD)? (boardvitals.com)
  • We've noticed this tends to be a difficult topic for our users that's why we've asked our partners at MedCram to explain a Glycogen Storage Diseases practice question from our USMLE Step 1 and COMLEX Level 1 question banks in the past. (boardvitals.com)
  • To insure a comprehensive understanding of this topic, check out our other video explanation on another Glycogen Storage Diseases practice question . (boardvitals.com)
  • Chen YT, Burchell A. Glycogen storage diseases. (medscape.com)
  • Prognosis for and treatment of glycogen storage diseases vary by type, but treatment typically includes dietary supplementation with cornstarch to provide a sustained source of glucose for the hepatic forms of GSD and exercise avoidance for the muscle forms. (msdmanuals.com)
Muscles8
  • Glycogen supercompensation occurs only in muscles that were trained and is maximal at a carbohydrate intake of approximately 25 grams per hour for average adults and possibly 40 grams or more per hour for bodybuilders. (tripod.com)
  • Because carbohydrates play such an important role in producing energy for the body, they are stored in the form of glycogen in both skeletal muscles and in the liver. (tripod.com)
  • The average person would store about 400 grams of glycogen in their muscles and 100 grams in their liver. (tripod.com)
  • Once stored in the muscles, glycogen cannot be released into the bloodstream, but will be utilized as fuel to produce ATP by the muscle itself. (encyclopedia.com)
  • The liver is capable of containing up to 10% of its volume in glycogen, in contrast to the 1% storage by volume carried on in the skeletal muscles. (encyclopedia.com)
  • In 1919, Otto Meyerhof was able to show that glycogen is converted into lactic acid in working muscles. (jrank.org)
  • So my conclusion is that you will deplete your leg muscles to about 50%, but you don't have to completely deplete your muscle glycogen to have lipolysis (fat release) due to the exercise. (stackexchange.com)
  • Glycogen - Key Points: Your muscles and your liver are your two main stores of glycogen. (strengthlog.com)
Molecule3
  • Glycogen is a branched biopolymer consisting of linear chains of glucose residues with an average chain length of approximately 8-12 glucose units and 2,000-60,000 residues per one molecule of glycogen. (wikipedia.org)
  • Glycogen is a non-osmotic molecule, so it can be used as a solution to storing glucose in the cell without disrupting osmotic pressure. (wikipedia.org)
  • A glycogen molecule would consist of thousands of these linkages with occasional cross linkages as illustrated in Figure 2. (tripod.com)
Muscle20
  • In humans, glycogen is made and stored primarily in the cells of the liver and skeletal muscle. (wikipedia.org)
  • In skeletal muscle, glycogen is found in a low concentration (1-2% of the muscle mass): the skeletal muscle of an adult weighing 70 kg stores roughly 400 grams of glycogen. (wikipedia.org)
  • Different levels of resting muscle glycogen are reached by changing the number of glycogen particles, rather than increasing the size of existing particles though most glycogen particles at rest are smaller than their theoretical maximum. (wikipedia.org)
  • therefore a doubling in glycogen stores can increase the bodybuilder s apparent muscle mass. (tripod.com)
  • Glycogen makes up about 1-2% of muscle weight and 6-10% of liver weight. (tripod.com)
  • Although the liver has a higher concentration of glycogen than muscle there is more glycogen stored in muscle tissue because muscle tissue is more abundant than liver tissue. (tripod.com)
  • Since 1 gram of carbohydrate contains 4 Calories, the body stores approximately 2000 Calories in the form of muscle and liver glycogen. (tripod.com)
  • The glycogen stored in muscle and liver comes from dietary carbohydrates if sufficient quantities of dietary carbohydrates are consumed. (tripod.com)
  • The inability of muscle cells to break down glycogen for energy leads to muscle weakness and wasting. (medlineplus.gov)
  • Both the liver and muscle are able to store glycogen, with muscle glycogen used primarily to fuel muscle contractions and liver glycogen used (when necessary) to replenish the bloodstream's dwindling supply of glucose. (jrank.org)
  • It was not until the 1930s, however, that the complicated process by which glycogen, stored in the liver and muscle, is broken down in the body and resynthesized was discovered by Czech-American biochemists Carl Cori and Gerty Cori. (jrank.org)
  • With intense exercise, glucose from glycogen stores in muscle becomes the predominant resource. (medscape.com)
  • Following the metal exposures, total and heat-stable protein levels in the liver, muscle and gill and glycogen contents in the liver and muscle of fish were measured. (tubitak.gov.tr)
  • Additionally, WWBV+DHEA supplementation improved exercise performance, testosterone levels and glycogen contents of both liver and muscle. (medsci.org)
  • Would this deplete the muscle glycogen in my legs? (stackexchange.com)
  • I agree that it's broad enough to be two questions -- one is about what it takes to deplete muscle glycogen, one is about what depleting glycogen does for fat loss. (stackexchange.com)
  • Of this, approximately 400 grams are stored as muscle glycogen, 90-110 grams as liver glycogen, and 25 grams circulate in the blood as glucose. (stackexchange.com)
  • This results in the inability of the muscle to metabolize glycogen to glucose. (boardvitals.com)
  • Glycogen storage disease type III is a rare disease of variable clinical severity affecting primarily the liver, heart, and skeletal muscle. (qxmd.com)
  • Your body prefers to use muscle glycogen to fuel intense workouts and other demanding physical work. (strengthlog.com)
Deposition1
  • However, appropriate fixation and definitive special staining are necessary for unequivocal evidence of glycogen deposition. (nih.gov)
Carbohydrates1
  • Complex carbohydrates are composed of complex sugars known as polysaccharides, of which glycogen is the most prominent example. (encyclopedia.com)
Glucagon1
  • Patients with glycogen-storage disease type 0 have normal-to-increased response to glucagon in the fed state, with hyperglycemia and lactic acidemia. (medscape.com)
Glycosidic bond1
  • Halftime of hydrolysis event of glycosidic bond in glycogen at 100°C is 12 hours. (harvard.edu)
Supercompensation4
  • Glycogen supercompensation occurs only when a low carbohydrate diet is combined with vigorous exercise followed by a high carbohydrate diet. (tripod.com)
  • Since noradrenaline synthesized from blood-borne tyrosine activates not only glycogenolysis but also glycogenesis in astrocytes, we hypothesized that blood tyrosine is a mechanistic-based biomarker of acute exercise-induced brain glycogen supercompensation. (frontiersin.org)
  • Brain glycogen decreased during endurance exercise and showed supercompensation within 6 h after exercise. (frontiersin.org)
  • This is the first study to produce a broad picture of plasma metabolite changes due to endurance exercise-induced brain glycogen supercompensation. (frontiersin.org)
Carbohydrate1
  • The energy required to power the human body begins with the consumption of food, and the subsequent extraction by the body of the carbohydrate-based sugars, known as glucose and glycogen. (encyclopedia.com)
Linkages1
  • Hydrolysis of (1,6)-α-D-glucosidic branch linkages in glycogen, amylopectin and their β-limit dextrins. (megazyme.com)
Concentration1
  • These studies establish a novel approach for manipulating brain glycogen concentration in normal, awake animals and provide in vivo confirmation that astrocyte glycogen supports neuronal function and survival during glucose deprivation. (aspetjournals.org)
Cleave1
  • It binds to mannose-6-phosphate receptors and then is transported into lysosomes, then undergoes proteolytic cleavage that results in increased enzymatic activity and ability to cleave glycogen. (medscape.com)
Store of glucose1
Biochemical1
  • Biochemical and molecular investigation of two Korean patients with glycogen storage disease type III" Clinical Chemistry and Laboratory Medicine , vol. 46, no. 9, 2008, pp. 1245-1249. (degruyter.com)
Polymer3
  • Glycogen is a high molecular weight polymer having branched-chain structure composed ofD-Glycopyranose residues. (ewg.org)
  • Glycogen is a hyperbranched glucose polymer, serving as a major energy reservoir in animals. (lu.se)
  • Glycogen is a branched polymer of glucose units that is formed in times of nutrient sufficiency and it is utilized in times of need. (concordia.ca)
Molecular1
  • Glycogen structure shows fewer branching points and longer peripheral chains upon molecular analysis. (medscape.com)
Clinical2
  • The clinical manifestations of glycogen storage disease type IV (GSD IV) discussed in this entry span a continuum of different subtypes with variable ages of onset, severity, and clinical features. (nih.gov)
  • Glycogen storage disease type III manifests a wide clinical spectrum. (qxmd.com)
Vivo1
  • The physiological function of this energy store has been difficult to establish because of the difficulty in manipulating brain glycogen concentrations in vivo. (aspetjournals.org)
Grams1
Content4
  • Changes in the amount of hepatocellular glycogen are frequently accompanied by other cytoplasmic alterations, such as fatty change or cytoplasmic vacuolization, and alterations in glycogen content can be difficult to distinguish from the accompanying changes. (nih.gov)
  • Rats treated with CP-316,819 had an 88 ± 3% increase in brain glycogen content. (aspetjournals.org)
  • In the presence of high glucose, lymphocytes build up glycogen stores, but the fate of this content is not very well understood. (concordia.ca)
  • The objective of this work is to demonstrate the presence of glycogen in activated human peripheral blood mononuclear cells (PBMCs) and to investigate the impact of low nutrient levels on the glycogen content of these cells. (concordia.ca)
Type12
Detection2
  • To test this hypothesis, we used a rat model of endurance exercise, a microwave irradiation for accurate detection of glycogen in the brain (the cortex, hippocampus, and hypothalamus), and capillary electrophoresis mass spectrometry-based metabolomics to observe the comprehensive metabolic profile of the blood. (frontiersin.org)
  • Glycogen concentrations were measured through periodic acid Schiff's staining (PAS) method and by using an enzymatic detection kit, in various time points. (concordia.ca)