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