Glycogen

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  Subject Areas on Research

  • A Liver-Specific Thyromimetic, VK2809, Decreases Hepatosteatosis in Glycogen Storage Disease Type Ia. 
  • A beta-blocker, propranolol, decreases the efficacy from enzyme replacement therapy in Pompe disease. 
  • A conserved domain for glycogen binding in protein phosphatase-1 targeting subunits. 
  • A systems genetics approach identifies Trp53inp2 as a link between cardiomyocyte glucose utilization and hypertrophic response. 
  • Acute interleukin-6 administration does not impair muscle glucose uptake or whole-body glucose disposal in healthy humans. 
  • Adenovirus-mediated delivery into myocytes of muscle glycogen phosphorylase, the enzyme deficient in patients with glycogen-storage disease type V. 
  • Adjunctive β2-agonist treatment reduces glycogen independently of receptor-mediated acid α-glucosidase uptake in the limb muscles of mice with Pompe disease. 
  • Aerobic training as an adjunctive therapy to enzyme replacement in Pompe disease. 
  • Age-related metabolic fatigue during low glucose conditions in rat hippocampus. 
  • Airway smooth muscle dysfunction in Pompe (Gaa-/- ) mice. 
  • Alglucosidase alfa enzyme replacement therapy as a therapeutic approach for glycogen storage disease type III. 
  • Allosteric regulation of glycogen breakdown by the second messenger cyclic di-GMP. 
  • Altered carbohydrate, lipid, and xenobiotic metabolism by liver from rats flown on Cosmos 1887. 
  • Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease. 
  • Antibody formation and mannose-6-phosphate receptor expression impact the efficacy of muscle-specific transgene expression in murine Pompe disease. 
  • Antibody-mediated enzyme replacement therapy targeting both lysosomal and cytoplasmic glycogen in Pompe disease. 
  • Assessing disease severity in Pompe disease: the roles of a urinary glucose tetrasaccharide biomarker and imaging techniques. 
  • Bezafibrate induces autophagy and improves hepatic lipid metabolism in glycogen storage disease type Ia. 
  • Biochemical and toxicopathic biomarkers assessed in smallmouth bass recovered from a polychlorinated biphenyl-contaminated river. 
  • Biological activity of complement in vivo. Role of C5 in the accumulation of polymorphonuclear leukocytes in inflammatory exudates. 
  • Bulbar muscle weakness and fatty lingual infiltration in glycogen storage disorder type IIIa. 
  • Calmodulin in endocrine cells and its multiple roles in hormone action. 
  • Cardiac and muscle fatigue due to relative functional overload induced by excessive stimulation, hypersensitive excitation-contraction coupling, or diminished performance capacity correlates with sarcoplasmic reticulum failure. 
  • Carnitine supplementation improves metabolic flexibility and skeletal muscle acetylcarnitine formation in volunteers with impaired glucose tolerance: A randomised controlled trial. 
  • Characterization of a canine model of glycogen storage disease type IIIa. 
  • Characterization of liver GSD IX γ2 pathophysiology in a novel Phkg2^-/- mouse model. 
  • Chinese hamster ovary cell-derived recombinant human acid alpha-glucosidase in infantile-onset Pompe disease. 
  • Chlamydospore formation during hyphal growth in Cryptococcus neoformans. 
  • Clinical and histologic ocular findings in pompe disease. 
  • Clinical and metabolic correction of pompe disease by enzyme therapy in acid maltase-deficient quail. 
  • Clinical outcomes after long-term treatment with alglucosidase alfa in infants and children with advanced Pompe disease. 
  • Correction of Biochemical Abnormalities and Improved Muscle Function in a Phase I/II Clinical Trial of Clenbuterol in Pompe Disease. 
  • Correction of glycogen storage disease type II by an adeno-associated virus vector containing a muscle-specific promoter. 
  • Correction of glycogen storage disease type III with rapamycin in a canine model. 
  • Correction of multiple striated muscles in murine Pompe disease through adeno-associated virus-mediated gene therapy. 
  • Definitive prenatal diagnosis for type III glycogen storage disease. 
  • Delivery of glucose-6-phosphatase in a canine model for glycogen storage disease, type Ia, with adeno-associated virus (AAV) vectors. 
  • Development of type II pneumocytes in rat lung. 
  • Developmental expression of glycogenolytic enzymes in rabbit tissues: possible relationship to fetal lung maturation. 
  • Diagnosis and management of glycogen storage diseases type VI and IX: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG). 
  • Dietary carbohydrates and endurance exercise. 
  • Differential metabolic effects of adenovirus-mediated glucokinase and hexokinase I overexpression in rat primary hepatocytes. 
  • Distinctive regulatory and metabolic properties of glycogen-targeting subunits of protein phosphatase-1 (PTG, GL, GM/RGl) expressed in hepatocytes. 
  • Does the aging skeletal muscle maintain its endocrine function? 
  • Dysregulation of multiple facets of glycogen metabolism in a murine model of Pompe disease. 
  • Early cognitive development in children with infantile Pompe disease. 
  • Early, sustained efficacy of adeno-associated virus vector-mediated gene therapy in glycogen storage disease type Ia. 
  • Eccentric exercise-induced muscle damage impairs muscle glycogen repletion. 
  • Effect of exercise intensity and volume on persistence of insulin sensitivity during training cessation. 
  • Effects of glucagon-like peptide 1 on the hepatic glucose metabolism. 
  • Effects of moderate and high glycemic index meals on metabolism and exercise performance. 
  • Effects of modulation of glycerol kinase expression on lipid and carbohydrate metabolism in human muscle cells. 
  • Efficacious Androgen Hormone Administration in Combination with Adeno-Associated Virus Vector-Mediated Gene Therapy in Female Mice with Pompe Disease. 
  • Efficacy of helper-dependent adenovirus vector-mediated gene therapy in murine glycogen storage disease type Ia. 
  • Elevated hepatocyte levels of the Forkhead box A2 (HNF-3beta) transcription factor cause postnatal steatosis and mitochondrial damage. 
  • Enhanced GLUT4-Dependent Glucose Transport Relieves Nutrient Stress in Obese Mice Through Changes in Lipid and Amino Acid Metabolism. 
  • Enhanced efficacy of an AAV vector encoding chimeric, highly secreted acid alpha-glucosidase in glycogen storage disease type II. 
  • Enhanced efficacy of enzyme replacement therapy in Pompe disease through mannose-6-phosphate receptor expression in skeletal muscle. 
  • Enhanced response to enzyme replacement therapy in Pompe disease after the induction of immune tolerance. 
  • Epidermal growth factor stimulates glycogen synthesis in fetal rat hepatocytes: comparison with the glycogenic effects of insulin-like growth factor I and insulin. 
  • Evasion of immune responses to introduced human acid alpha-glucosidase by liver-restricted expression in glycogen storage disease type II. 
  • Exercise-induced changes in insulin action and glycogen metabolism in elderly adults. 
  • Expression and glycogenic effect of glycogen-targeting protein phosphatase 1 regulatory subunit GL in cultured human muscle. 
  • Expression of glucokinase in cultured human muscle cells confers insulin-independent and glucose concentration-dependent increases in glucose disposal and storage. 
  • Expression of human protein phosphatase-1 in Saccharomyces cerevisiae highlights the role of phosphatase isoforms in regulating eukaryotic functions. 
  • Fenofibrate rapidly decreases hepatic lipid and glycogen storage in neonatal mice with glycogen storage disease type Ia. 
  • Fibroblasts Mobilize Tumor Cell Glycogen to Promote Proliferation and Metastasis. 
  • Follicle-stimulating hormone activation of glycogen phosphorylase in the Sertoli cell-enriched rat testis. 
  • From dietary glucose to liver glycogen: the full circle round. 
  • Fully deleted adenovirus persistently expressing GAA accomplishes long-term skeletal muscle glycogen correction in tolerant and nontolerant GSD-II mice. 
  • Functional units in rainbow trout (Salmo gairdneri) liver: I. Arrangement and histochemical properties of hepatocytes. 
  • GSK3 involvement in amylin signaling in isolated rat soleus muscle. 
  • Gain of glucose-independent growth upon metastasis of breast cancer cells to the brain. 
  • Glucose tetrasaccharide as a biomarker for monitoring the therapeutic response to enzyme replacement therapy for Pompe disease. 
  • Glucose-6-phosphatase overexpression lowers glucose 6-phosphate and inhibits glycogen synthesis and glycolysis in hepatocytes without affecting glucokinase translocation. Evidence against feedback inhibition of glucokinase. 
  • Glycogen accumulation in smooth muscle of a Pompe disease mouse model. 
  • Glycogen debranching enzyme is associated with rat skeletal muscle sarcoplasmic reticulum. 
  • Glycogen storage disease type Ia in two littermate Maltese puppies. 
  • Glycogen storage diseases presenting as hypertrophic cardiomyopathy. 
  • Glycogen storage in multiple muscles of old GSD-II mice can be rapidly cleared after a single intravenous injection with a modified adenoviral vector expressing hGAA. 
  • Glycogen-branching enzyme deficiency leads to abnormal cardiac development: novel insights into glycogen storage disease IV. 
  • Glycogen-targeting subunits and glucokinase differentially affect pathways of glycogen metabolism and their regulation in hepatocytes. 
  • HIF-1α in heart: protective mechanisms. 
  • Hemodynamic and metabolic responses to exercise after adrenoceptor blockade in humans. 
  • Hepatic expression of a targeting subunit of protein phosphatase-1 in streptozotocin-diabetic rats reverses hyperglycemia and hyperphagia despite depressed glucokinase expression. 
  • Hepatic function in rats after spaceflight: effects on lipids, glycogen, and enzymes. 
  • Hepatic mTORC1 Opposes Impaired Insulin Action to Control Mitochondrial Metabolism in Obesity. 
  • Hepatic overexpression of glycerol-sn-3-phosphate acyltransferase 1 in rats causes insulin resistance. 
  • High-level production of recombinant human lysosomal acid alpha-glucosidase in Chinese hamster ovary cells which targets to heart muscle and corrects glycogen accumulation in fibroblasts from patients with Pompe disease. 
  • Human brain glycogen content and metabolism: implications on its role in brain energy metabolism. 
  • Human brain glycogen metabolism during and after hypoglycemia. 
  • Humoral mediators of chemotaxis of mononuclear leukocytes. 
  • IL-6 and TNF-alpha expression in, and release from, contracting human skeletal muscle. 
  • Impaired clearance of accumulated lysosomal glycogen in advanced Pompe disease despite high-level vector-mediated transgene expression. 
  • Improvement of bilateral ptosis on higher dose enzyme replacement therapy in Pompe disease. 
  • In situ mass spectrometry imaging reveals heterogeneous glycogen stores in human normal and cancerous tissues. 
  • Increase in liver protein phosphatase-1 in spontaneously diabetic Chinese hamsters. 
  • Increasing fructose 2,6-bisphosphate overcomes hepatic insulin resistance of type 2 diabetes. 
  • Influence of fasting on carbohydrate and fat metabolism during rest and exercise in men. 
  • Interleukin-6 production in contracting human skeletal muscle is influenced by pre-exercise muscle glycogen content. 
  • Labeling of peripheral blood polymorphonuclear leukocytes with indium-111: a new method for the quantitation of in-vivo accumulation of PMNLs in rabbit skin. 
  • Leptin directly alters lipid partitioning in skeletal muscle. 
  • Leptin opposes insulin's effects on fatty acid partitioning in muscles isolated from obese ob/ob mice. 
  • Links between autophagy and disorders of glycogen metabolism - Perspectives on pathogenesis and possible treatments. 
  • Liver fibrosis during clinical ascertainment of glycogen storage disease type III: a need for improved and systematic monitoring. 
  • Long-term correction of glycogen storage disease type II with a hybrid Ad-AAV vector. 
  • Long-term effects of a high-carbohydrate diet and exercise on insulin action in older subjects with impaired glucose tolerance. 
  • Long-term monitoring of patients with infantile-onset Pompe disease on enzyme replacement therapy using a urinary glucose tetrasaccharide biomarker. 
  • Metabolic factors associated with endotoxin-induced tolerance for hemorrhagic shock. 
  • Metabolic profiling of muscle contraction in lean compared with obese rodents. 
  • Minimum Effective Dose to Achieve Biochemical Correction with Adeno-Associated Virus Vector-Mediated Gene Therapy in Mice with Pompe Disease. 
  • Molecular characterization of glycogen storage disease type III. 
  • Mouse model of glycogen storage disease type III. 
  • Multiple muscles in the AMD quail can be "cross-corrected" of pathologic glycogen accumulation after intravenous injection of an [E1-, polymerase-] adenovirus vector encoding human acid-alpha-glucosidase. 
  • Muscle damage: nutritional considerations. 
  • Muscle glycogen content and glucose uptake during exercise in humans: influence of prior exercise and dietary manipulation. 
  • Mutational analysis of the coding regions of the genes encoding protein kinase B-alpha and -beta, phosphoinositide-dependent protein kinase-1, phosphatase targeting to glycogen, protein phosphatase inhibitor-1, and glycogenin: lessons from a search for genetic variability of the insulin-stimulated glycogen synthesis pathway of skeletal muscle in NIDDM patients. 
  • Mutations of the serine phosphorylated in the protein phosphatase-1-binding motif in the skeletal muscle glycogen-targeting subunit. 
  • Natural Progression of Canine Glycogen Storage Disease Type IIIa. 
  • Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice. 
  • Organizing glucose disposal: emerging roles of the glycogen targeting subunits of protein phosphatase-1. 
  • Overexpression of protein targeting to glycogen in cultured human muscle cells stimulates glycogen synthesis independent of glycogen and glucose 6-phosphate levels. 
  • Overexpression of the P46 (T1) translocase component of the glucose-6-phosphatase complex in hepatocytes impairs glycogen accumulation via hydrolysis of glucose 1-phosphate. 
  • Ovine placental lactogen inhibits glucagon-induced glycogenolysis in fetal rat hepatocytes. 
  • Pathogenesis of growth failure and partial reversal with gene therapy in murine and canine Glycogen Storage Disease type Ia. 
  • Peripheral effects of endurance training in young and old subjects. 
  • Physiologic importance of protein phosphatase inhibitors. 
  • Polysomnographic findings in infantile Pompe disease. 
  • Pompe disease gene therapy. 
  • Pyruvate incubation enhances glycogen stores and sustains neuronal function during subsequent glucose deprivation. 
  • Quantitative computed tomography for enzyme replacement therapy in Pompe disease. 
  • Recombinant human acid alpha-glucosidase corrects acid alpha-glucosidase-deficient human fibroblasts, quail fibroblasts, and quail myoblasts. 
  • Recombinant human acid alpha-glucosidase enzyme therapy for infantile glycogen storage disease type II: results of a phase I/II clinical trial. 
  • Regulation and function of the muscle glycogen-targeting subunit of protein phosphatase 1 (GM) in human muscle cells depends on the COOH-terminal region and glycogen content. 
  • Regulation of cerebral glucose metabolism. 
  • Regulation of skeletal muscle oxidative capacity and insulin signaling by the mitochondrial rhomboid protease PARL. 
  • Relationship between fat-to-fat-free mass ratio and decrements in leg strength after downhill running. 
  • Reversal of diet-induced glucose intolerance by hepatic expression of a variant glycogen-targeting subunit of protein phosphatase-1. 
  • Salmeterol enhances the cardiac response to gene therapy in Pompe disease. 
  • Skeletal muscle adaptation to chronic low-frequency motor nerve stimulation. 
  • Sparing effect of leptin on liver glycogen stores in rats during the fed-to-fasted transition. 
  • Starch Binding Domain-containing Protein 1 Plays a Dominant Role in Glycogen Transport to Lysosomes in Liver. 
  • Stbd1 is highly elevated in skeletal muscle of Pompe disease mice but suppression of its expression does not affect lysosomal glycogen accumulation. 
  • Substrate availability and transcriptional regulation of metabolic genes in human skeletal muscle during recovery from exercise. 
  • Suppression of pullulanase-induced cytotoxic T cell response with a dual promoter in GSD IIIa mice. 
  • Sustained correction of motoneuron histopathology following intramuscular delivery of AAV in pompe mice. 
  • Synergistic Efficacy from Gene Therapy with Coreceptor Blockade and a β2-Agonist in Murine Pompe Disease. 
  • Synergistic effects of oPL and insulin on glycogen metabolism in fetal rat hepatocytes. 
  • Systemic Correction of Murine Glycogen Storage Disease Type IV by an AAV-Mediated Gene Therapy. 
  • Systemic Delivery of AAVB1-GAA Clears Glycogen and Prolongs Survival in a Mouse Model of Pompe Disease. 
  • Systemic correction of the muscle disorder glycogen storage disease type II after hepatic targeting of a modified adenovirus vector encoding human acid-alpha-glucosidase. 
  • The Effect of sex steroid hormones on substrate oxidation during prolonged submaximal exercise in women. 
  • The effects of local progesterone on stilbestrol-associated vaginal adenosis. 
  • The embryologic development of the human vagina. 
  • The glycogenic effects of placental lactogen and growth hormone in ovine fetal liver are mediated through binding to specific fetal ovine placental lactogen receptors. 
  • The new era of Pompe disease: advances in the detection, understanding of the phenotypic spectrum, pathophysiology, and management. 
  • The pathway mediating insulin's effects on pyruvate dehydrogenase bypasses the insulin receptor tyrosine kinase. 
  • Therapeutic Benefit of Autophagy Modulation in Pompe Disease. 
  • Three-dimensional tissue-engineered human skeletal muscle model of Pompe disease. 
  • Tor and cyclic AMP-protein kinase A: two parallel pathways regulating expression of genes required for cell growth. 
  • Transcriptional activation of the IL-6 gene in human contracting skeletal muscle: influence of muscle glycogen content. 
  • Transcriptomic and Proteomic Analysis of Clear Cell Foci (CCF) in the Human Non-Cirrhotic Liver Identifies Several Differentially Expressed Genes and Proteins with Functions in Cancer Cell Biology and Glycogen Metabolism. 
  • Tumor necrosis factor increases mitochondrial oxidant production and induces expression of uncoupling protein-2 in the regenerating mice [correction of rat] liver. 
  • Type III glycogen storage disease: an adult case with mild disease but complete absence of debrancher protein. 
  • Ultrastructure of the eye in fetal type II glycogenosis (Pompe's disease). 
  • What's new and what's next for gene therapy in Pompe disease? 
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  Keywords of People

  • Case, Laura Elizabeth, Associate Professor in Orthopaedic Surgery, Pediatrics, Medical Genetics
  • Freemark, Michael Scott, Robert C. Atkins, M.D. and Veronica Atkins Distinguished Professor of Pediatrics, in the School of Medicine, Pediatrics, Endocrinology
  • Turner, Dennis Alan, Professor of Neurosurgery, Biomedical Engineering