Type I glycogen storage disease: a metabolic basis for advances in treatment.

[1]  J. Coleman.  Metabolic interrelationships between carbohydrates lipids and proteins , 1980 .

[2]  H. Greene,et al.  ATP depletion, a possible role in the pathogenesis of hyperuricemia in glycogen storage disease type I. , 1978, The Journal of clinical investigation.

[3]  C. Stanley,et al.  831 LONG TERM TREATMENT OF GLYCOGEN STORAGE DISEASE TYPE I (GSD I): CLINICAL IMPROVEMENT BUT PERSISTENT ABNORMALITIES OF LACTATE & TRIGLYCERIDE CONCENTRATION , 1978, Pediatric Research.

[4]  H. Greene,et al.  894 DIFFERING FOOD COMPOSITION FOR NOCTURNAL INTRAGASTRIC THERAPY IN TYPES I AND III GLYCOGEN STORAGE DISEASE (GSD) , 1978, Pediatric Research.

[5]  R. A. Cooper.  Abnormalities of cell-membrane fluidity in the pathogenesis of disease. , 1977, The New England journal of medicine.

[6]  M. Kogut,et al.  The Pathogenesis of Hyperuricemia in Glycogen Storage Disease, Type I , 1977, Pediatric Research.

[7]  V. Esmann,et al.  Glycogen metabolism: the integrated cellular response to a bi-directional metabolic stimulus. , 1977, Biochemical and biophysical research communications.

[8]  P. Saugmann.  Glycogen synthase- "R": the occurrence and significance of a previously unknown form of GS, found in metabolically active human leucocytes. , 1977, Biochemical and biophysical research communications.

[9]  J. Folkman,et al.  Glycogen storage disease: new approaches to therapy. , 1977, Ciba Foundation symposium.

[10]  T. Starzl,et al.  Portacaval shunt for glycogen storage disease and hyperlipidaemia. , 1977, Ciba Foundation symposium.

[11]  R. Stevenson,et al.  Hepatic adenomata with type 1 glycogen storage disease. , 1976, JAMA.

[12]  A. de Vries,et al.  De novo synthesis of purine nucleotides in human peripheral blood leukocytes. Excessive activity of the pathway in hypoxanthine-guanine phosphoribosyltransferase deficiency. , 1976, The Journal of clinical investigation.

[13]  A. Sadeghi‐Nejad,et al.  The glycogenoses and other inherited disorders of carbohydrate metabolism. , 1976, Clinics in perinatology.

[14]  H. Greene,et al.  Continuous nocturnal intragastric feeding for management of type 1 glycogen-storage disease. , 1976, The New England journal of medicine.

[15]  R. Knuppel,et al.  Pregnancy and von Gierke's disease. , 1976, Obstetrics and gynecology.

[16]  H. Greene,et al.  Comparison of the effects of total parenteral nutrition, continuous intragastric feeding, and portacaval shunt on a patient with type I glycogen storage disease. , 1974, The Journal of pediatrics.

[17]  J. Fernandes.  THE EFFECT OF DISACCHARIDES ON THE HYPERLACTACIDAEMIA OF GLUCOSE‐6‐PHOSPHATASE‐DEFICIENT CHILDREN , 1974, Acta paediatrica Scandinavica.

[18]  K. Kim,et al.  Regulation of rat liver glycogen synthetase D. Role of glucose 6-phosphate and enzyme sulfhydryl groups in activity and glycogen binding. , 1974, The Journal of biological chemistry.

[19]  H. Greene,et al.  Impaired platelet function in glucose-6-phosphatase deficiency. , 1974, The Journal of pediatrics.

[20]  A. Sadeghi‐Nejad,et al.  Studies in type I glycogenosis of the liver. The genesis and disposition of lactate. , 1974, The Journal of pediatrics.

[21]  J. Fernandes,et al.  Triglyceride Clearing in Glycogen Storage Disease , 1974, Pediatric Research.

[22]  T. Starzl,et al.  Portacaval shunt in hyperlipidemia , 1974 .

[23]  A. Binkiewicz,et al.  Decreased ketogenesis in von Gierke's disease (type I glycogenosis). , 1973, The Journal of pediatrics.

[24]  O. Zelder,et al.  Depletion of Liver Adenosine Phosphates and Metabolic Effects of Intravenous Infusion of Fructose or Sorbitol in Man and in the Rat *, ** , 1973, European journal of clinical investigation.

[25]  T. Starzl,et al.  Portal Diversion for the Treatment of Glycogen Storage Disease in Humans , 1973, Annals of surgery.

[26]  K. Raivio,et al.  Role of glutamine in purine synthesis and in guanine nucleotide formation in normal fibroblasts and in fibroblasts deficient in hypoxanthine phosphoribosyltransferase activity. , 1973, Biochimica et biophysica acta.

[27]  J. Folkman,et al.  Portacaval shunt for glycogen storage disease: value of prolonged intravenous hyperalimentation before surgery. , 1972, Surgery.

[28]  T. Shimazu.  Regulation of glycogen metabolism in liver by the autonomic nervous system. V. Activation of glycogen synthetase by vagal stimulation. , 1971, Biochimica et biophysica acta.

[29]  J. Fernandes,et al.  Glucose-6-phosphatase deficiency and hyperlipaemia. , 1970, Clinica chimica acta; international journal of clinical chemistry.

[30]  H. Krebs,et al.  The cause of hepatic accumulation of fructose 1-phosphate on fructose loading. , 1970, The Biochemical journal.

[31]  L. Birnbaumer,et al.  Glucagon-Sensitive Adenyl Cylase in Plasma Membrane of Hepatic Parenchymal Cells , 1969, Science.

[32]  R. Mahler.  Glycogen storage diseases , 1969 .

[33]  R. Howell.  Hyperuricemia in childhood. , 1968, Federation proceedings.

[34]  O. Rennert,et al.  Diazoxide in von Gierke's disease. , 1968, Archives of disease in childhood.

[35]  S. Jakovcic,et al.  Studies of uric acid metabolism in glycogen storage disease associated with gouty arthritis. , 1967, Arthritis and rheumatism.

[36]  J. Larner,et al.  Rapid activation-inactivation of liver uridine diphosphate glucose-glycogen transferase and phosphorylase by insulin and glucagon in vivo. , 1967, The Journal of biological chemistry.

[37]  R. Howell,et al.  Relationships between glycogen storage disease and tophaceous gout. , 1967, The American journal of medicine.

[38]  G. Donnell,et al.  Hyperuricemia in glycogen-storage disease type 1. , 1966, American journal of diseases of children.

[39]  A. Riddell,et al.  Portacaval transposition in the treatment of glycogen-storage disease. , 1966, Lancet.

[40]  A. Khachadurian,et al.  The hyperlipidemia in glycogen storage disease. , 1966, The Journal of laboratory and clinical medicine.

[41]  P. Öckerman.  In vitro studies of adipose tissue metabolism of glucose, glycerol and free fatty acids in glycogen storage disease type 1. , 1965 .

[42]  P. Öckerman.  Glucose, glycerol and free fatty acids in glycogen storage disease type 1. Blood levels in the fasting and non-fasting state. Effect of glucose and adrenalin administration. , 1965 .

[43]  R. Howell.  The interrelationship of glycogen storage disease and gout. , 1965, Arthritis and rheumatism.

[44]  L. Mosovich,et al.  THE PARADOXICAL EFFECT OF ALCOHOL ON CARBOHYDRATE METABOLISM IN FOUR PATIENTS WITH LIVER GLYCOGEN DISEASE. , 1965, Pediatrics.

[45]  T. Tanaka,et al.  PHOSPHOFRUCTOKINASE DEFICIENCY IN SKELETAL MUSCLE. A NEW TYPE OF GLYCOGENOSIS. , 1965, Biochemical and biophysical research communications.

[46]  T. Starzl,et al.  THE EFFECT OF PORTACAVAL TRANSPOSITION ON CARBOHYDRATE METABOLISM: EXPERIMENTAL AND CLINICAL OBSERVATIONS. , 1965, Surgery.

[47]  J. F. Henderson,et al.  SYNTHESIS OF 5-PHOSPHORIBOSYL 1-PYROPHOSPHATE FROM GLUCOSE IN EHRLICH ASCITES TUMOR CELLS IN VITRO. , 1965, The Journal of biological chemistry.

[48]  J. Sidbury.  The genetics of the glycogen storage diseases. , 1965, Progress in medical genetics.

[49]  T. Starzl,et al.  LIVER DEGLYCOGENATION AFTER PORTACAVAL TRANSPOSITION. , 1964, Surgical forum.

[50]  J. Wyngaarden,et al.  GLUCOSE-6-PHOSPHATASE DEFICIENCY GLYCOGEN STORAGE DISEASE , 1962, Pediatrics.

[51]  W. Schubert,et al.  Menghini needle biopsy of the liver. , 1960, American journal of diseases of children.

[52]  E. Sutherland,et al.  Immunological differences of phosphorylases. , 1957, The Journal of biological chemistry.

[53]  D. Pickering,et al.  Glycogen-Storage Disease: A Study on the Effect of Sodium/-Thyroxine and Glucagon , 1956 .

[54]  E. Sutherland,et al.  The relationship of epinephrine and glucagon to liver phosphorylase. I. Liver phosphorylase; preparation and properties. , 1956, The Journal of biological chemistry.

[55]  E. Sutherland,et al.  The relationship of epinephrine and glucagon to liver phosphorylase. II. Enzymatic inactivation of liver phosphorylase. , 1956, The Journal of biological chemistry.