Where is glycogen found

Duran J, Guinovart JJ.. Brain glycogen in health and disease. Molec Aspects Med. Krogh A, Lindhard J.. The relative value of fat and carbohydrate as sources of muscular energy: with appendices on the correlation between standard metabolism and the respiratory quotient during rest and work.

Biochem J. Some changes in the chemical constituents of the blood following a marathon—with especial reference to the development of hypoglycemia. Bergstrom J, Hultman E.. Muscle glycogen synthesis after exercise: an enhancing factor localized to the muscle cells in man. Acta Physiol Scand. Muscle glycogen during prolonged severe exercise. Hultman E, Bergstrom J.. Muscle glycogen synthesis in relation to diet studied in normal subjects. Acta Med Scand. Murray R, Sugar, sports drinks, and performance.

In: Rippe JM, ed. New York: Humana Press; — The governor has a sweet tooth—mouth sensing of nutrients to enhance sports performance. Eur J Sports Sci. The effect of carbohydrate mouth rinse on 1-h cycle time trial performance. Hargreaves M. The metabolic systems: carbohydrate metabolism. In: Farrell P, ed.

Ivy J. The regulation and synthesis of muscle glycogen by means of nutrient intervention. In: Maughan R, ed. Sports Nutrition. Smythe C, Cohen P.. The discovery of glycogenin and the priming mechanism for glycogen biogenesis.

Eur J Biochem. The biogenesis of glycogen: nature of the carbohydrate in the protein primer. Biochem Int. The nature of the primer for glycogen synthesis in muscle. FEBS Lett. Proglycogen: a low-molecular-weight form of muscle glycogen. Muscle glycogen: where did you come from, where did you go? J Physiol. Nielsen J, Ortenblad N.. Physiological aspects of the subcellular localization of glycogen in skeletal muscle.

Appl Physiol Nutr Metab. Glycogen availability and skeletal muscle adaptations with endurance and resistance exercise. Nutr Metab London. Ahlborg B, Brohult J.. Immediate and delayed metabolic reactions in well-trained subjects after prolonged physical exercise. Muscle glycogen synthetase in normal subjects. Basal values, effect of glycogen depletion by exercise and of a carbohydrate-rich diet following exercise.

Scand J Clin Lab Invest. Int J Sports Med. Carbohydrate availability and exercise training adaptation: too much of a good thing? Burke LM. Fueling strategies to optimize performance: training high or training low? Scand J Med Sci Sports. Hawley J. Manipulating carbohydrate availability to promote training adaptation. Sports Sci Exchange. Betts JA, Williams C.. Short-term recovery from prolonged exercise: exploring the potential for protein ingestion to accentuate the benefits of carbohydrate supplements.

Amino Acids. Creatine supplementation does not affect human skeletal muscle glycogen content in the absence of prior exercise. Burke LM, Kiens B.. Hermansen L, Vaage O.. Lactate disappearance and glycogen synthesis in human muscle after maximal exercise. Am J Physiol. Prog Mol Biol Transl Sci. Muscle metabolism during intense, heavy-resistance exercise. Carbohydrate intake and resistance-based exercise: are current recommendations reflective of actual need? Br J Nutr.

Insufficient dietary carbohydrate during training: does it impair athletic performance? Int J Sports Nutr. Helge JW. A high carbohydrate diet remains the evidence based choice for elite athletes to optimise performance. Coyle EF. Timing and method of increased carbohydrate intake to cope with heavy training, competition and recovery. J Sports Sci. Muscle glycogen storage after prolonged exercise: effect of the glycemic index of carbohydrate feedings. Signalling mechanisms in skeletal muscle: role in substrate selection and muscle adaptation.

Essays Biochem. Jeukendrup AE. Periodized nutrition for athletes. Glucose, exercise and insulin: emerging concepts. Eccentric exercise decreases glucose transporter GLUT4 protein in human skeletal muscle. Exhaustive running: inappropriate as a stimulus of muscle glycogen super-compensation. Shi X, Passe DH.. Water and solute absorption from carbohydrate-electrolyte solutions in the human proximal small intestine: a review and statistical analysis. Optimal composition of fluid-replacement beverages.

Compr Physiol. High oxidation rates from combined carbohydrates ingested during exercise. Wu CL, Williams C.. A low glycemic index meal before exercise improves endurance running capacity in men. Int J Sports Physiol Perf. J Sci Med Sport. J Sports Med Phys Fit.

Endurance training in fasting conditions: biological adaptations and body weight management. Nutr Hosp. Cox PJ, Clarke K.. Acute nutritional ketosis: implications for exercise performance and metabolism. Targeting an The only available treatment for the disease is enzyme replacement therapy Using a novel technique, researchers have been able What Makes Us Human?

Stem cell researchers have now found a previously overlooked Diffuse reticuloendothelial system involvement in type IV glycogen storage disease with a novel GBE1 mutation: a case report and review.

Aksu T. Cardiac involvement in glycogen storage disease type IV: two cases and the two ends of a spectrum. Case Rep. Sagnelli A. Adult polyglucosan body disease in a patient originally diagnosed with Fabry's disease. Bao Y. Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene. Mochel F. Adult polyglucosan body disease: natural history and key magnetic resonance imaging findings. Billot S. Acute but transient neurological deterioration revealing adult polyglucosan body disease.

Gentry M. Insights into Lafora disease: malin is an E3 ubiquitin ligase that ubiquitinates and promotes the degradation of laforin.

Sankhala R. Dimeric quaternary structure of human laforin. Minassian B. Mutation spectrum and predicted function of laforin in Lafora's progressive myoclonus epilepsy. Ianzano L. Turnbull J. Early-onset Lafora body disease. Burwinkel B. Kato K. Human brain-type glycogen phosphorylase: quantitative localization in human tissues determined with an immunoassay system. Newgard C. Human brain glycogen phosphorylase. Cloning, sequence analysis, chromosomal mapping, tissue expression, and comparison with the human liver and muscle isozymes.

Orngreen M. Lactate and energy metabolism during exercise in patients with blocked glycogenolysis McArdle disease J. Fat metabolism during exercise in patients with McArdle disease. Wagenmakers A. Metabolism of branched-chain amino acids and ammonia during exercise: clues from McArdle's disease. Sports Med. Petrou P. Minimally symptomatic McArdle disease, expanding the genotype-phenotype spectrum.

Muscle Nerve. Quinlivan R. The polymorphic locus for glycogen storage disease VI liver glycogen phosphorylase maps to chromosome Roscher A. The natural history of glycogen storage disease types VI and IX: Long-term outcome from the largest metabolic center in Canada. Ercan-Fang N. Integrated effects of multiple modulators on human liver glycogen phosphorylase a.

Rath V. Activation of human liver glycogen phosphorylase by alteration of the secondary structure and packing of the catalytic core. Munger R. Correlations of glycogen synthase and phosphorylase activities with glycogen concentration in human muscle biopsies.

Evidence for a double-feedback mechanism regulating glycogen synthesis and breakdown. Keppens S. Regulation of glycogen phosphorylase activity in isolated human hepatocytes. Bali D. Variability of disease spectrum in children with liver phosphorylase kinase deficiency caused by mutations in the PHKG2 gene.

Beauchamp N. Glycogen storage disease type IX: high variability in clinical phenotype. Is muscle glycogenolysis impaired in X-linked phosphorylase b kinase deficiency? Echaniz-Laguna A. Muscle phosphorylase b kinase deficiency revisited.

Mizuta K. A new type of glycogen storage disease caused by deficiency of cardiac phosphorylase kinase. Fetal bradycardia at 28 weeks of gestation associated with cardiac glycogen phosphorylase b kinase deficiency. Acta Paediatr. Chen Y. Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease. Human glycogen debranching enzyme gene AGL : complete structural organization and characterization of the 5' flanking region.

Cheng A. Distinct mutations in the glycogen debranching enzyme found in glycogen storage disease type III lead to impairment in diverse cellular functions. Guin S. Role in tumor growth of a glycogen debranching enzyme lost in glycogen storage disease. Cancer Inst. Shen J. Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle.

Inherent lipid metabolic dysfunction in glycogen storage disease IIIa. Gershen L. Pathological characteristics of glycogen storage disease III in skeletal muscle. Preisler N. Exercise intolerance in glycogen storage disease type III: weakness or energy deficiency? Mogahed E. Skeletal and cardiac muscle involvement in children with glycogen storage disease type III. Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III.

Wisselaar H. Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation. Tsuji A. Intracellular transport of acid alpha-glucosidase in human fibroblasts: evidence for involvement of phosphomannosyl receptor-independent system.

Remiche G. Extended phenotype description and new molecular findings in late onset glycogen storage disease type II: a northern Italy population study and review of the literature.

Molecular analysis and protein processing in late-onset Pompe disease patients with low levels of acid alpha-glucosidase activity. Fat and carbohydrate metabolism during exercise in late-onset Pompe disease. Katona I. Glycogenosome accumulation in the arrector pili muscle in Pompe disease. Orphanet J. Rare Dis. Muraoka T.

Novel mutations in the gene encoding acid alpha-1,4-glucosidase in a patient with late-onset glycogen storage disease type II Pompe disease with impaired intelligence. Kishnani P. New therapeutic approaches for Pompe disease: enzyme replacement therapy and beyond.

Mundy H. The effect of l -alanine therapy in a patient with adult onset glycogen storage disease type II. Terzis G. Effect of aerobic and resistance exercise training on late-onset Pompe disease patients receiving enzyme replacement therapy. Boucek D. Natural history of Danon disease. Martin C. Identification and characterization of a human cDNA and gene encoding a ubiquitously expressed glucosephosphatase catalytic subunit-related protein.

Lin B. Human variant glucosephosphate transporter is active in microsomal transport. Cloning and characterization of the human and rat islet-specific glucosephosphatase catalytic subunit-related protein IGRP genes. Chou J. Mutations in the glucosephosphatase-alpha G6PC gene that cause type Ia glycogen storage disease.

Jun H. Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib. Melis D. Involvement of endocrine system in a patient affected by glycogen storage disease 1b: speculation on the role of autoimmunity.

Ross K. Safety and efficacy of chronic extended release cornstarch therapy for glycogen storage disease type I. JIMD Rep. Shah K. Effect of dietary interventions in the maintenance of normoglycaemia in glycogen storage disease type 1a: a systematic review and meta-analysis. Banka S. A clinical and molecular review of ubiquitous glucosephosphatase deficiency caused by G6PC3 mutations. Hayee B. G6PC3 mutations are associated with a major defect of glycosylation: a novel mechanism for neutrophil dysfunction.

Webb B. Structures of human phosphofructokinase-1 and atomic basis of cancer-associated mutations. Al Hasawi N. Phosphofructokinase: a mediator of glycolytic flux in cancer progression. Juvenile-onset permanent weakness in muscle phosphofructokinase deficiency. Sakoda S. Tsujino S. The molecular genetic basis of muscle phosphoglycerate mutase PGAM deficiency.

Kreuder J. Brief report: inherited metabolic myopathy and hemolysis due to a mutation in aldolase A. Beutler E. Red cell aldolase deficiency and hemolytic anemia: a new syndrome. Comi G. Beta-enolase deficiency, a new metabolic myopathy of distal glycolysis. Peshavaria M. Structure of human muscle beta enolase mRNA and protein deduced from a genomic clone. Nucleic Acids Res. Musumeci O. Recurrent rhabdomyolysis due to muscle beta-enolase deficiency: very rare or underestimated?

Miyajima H. Molecular characterization of gene expression in human lactate dehydrogenase-A. Woerle H. Pathways for glucose disposal after meal ingestion in humans. Barosa C. Sources of hepatic glycogen synthesis following a milk-containing breakfast meal in healthy subjects.

Fructose and galactose enhance postexercise human liver glycogen synthesis. Sports Exerc. Bergstrom J. Muscle glycogen synthetase in normal subjects. Basal values, effect of glycogen depletion by exercise and of a carbohydrate-rich diet following exercise.

Kochan R. Glycogen synthase activation in human skeletal muscle: effects of diet and exercise. Koivisto V. Glycogen depletion during prolonged exercise: influence of glucose, fructose, or placebo.

Levine L. Fructose and glucose ingestion and muscle glycogen use during submaximal exercise. Vukovich M. Effect of fat emulsion infusion and fat feeding on muscle glycogen utilization during cycle exercise.

Ryan A. Aerobic exercise plus weight loss improves insulin sensitivity and increases skeletal muscle glycogen synthase activity in older men. A Biol. Wallis G. Postexercise muscle glycogen synthesis with combined glucose and fructose ingestion.

Jentjens R. Addition of protein and amino acids to carbohydrates does not enhance postexercise muscle glycogen synthesis. Detko E. Liver and muscle glycogen repletion using 13C magnetic resonance spectroscopy following ingestion of maltodextrin, galactose, protein and amino acids. Di Camillo B. Leucine modulates dynamic phosphorylation events in insulin signaling pathway and enhances insulin-dependent glycogen synthesis in human skeletal muscle cells.

BMC Cell Biol. Johnson A. Effect of increased free fatty acid supply on glucose metabolism and skeletal muscle glycogen synthase activity in normal man. Research has shown that aerobic endurance is directly related to the initial muscle glycogen stores, that strenuous exercise cannot be maintained once these stores are depleted, and that perception of fatigue during prolonged intense exercise parallels the decline in muscle glycogen 3. Ensure you are optimizing glycogen stores before exercise, maintaining it during exercise, and replenishing it after exercise.

What is Glycogen? The Important Take-home Message Ensure you are optimizing glycogen stores before exercise, maintaining it during exercise, and replenishing it after exercise.