Glycogen-Storage Diseases**This review is dedicated to Carl F. Cori on the occasion of his seventieth birthday.: Types I, III, IV, V, VII and Unclassified Glycogenoses

α-Glucosidase deficiency in generalized glycogen-storage disease (Pompe's disease)

1963 • 847 citations

MYOPATHY DUE TO A DEFECT IN MUSCLE GLYCOGEN BREAKDOWN

1951 • 593 citations

Glucose-6-Phosphatase of the Liver in Glycogen Storage Disease

1952 • 574 citations

McARDLE'S SYNDROME

1962 • 391 citations

Phosphofructokinase deficiency in skeletal muscle. A new type of glycogenosis

1965 • 362 citations

Glycogen Storage Disease

1964 • 352 citations

CHRONIC PROGRESSIVE MYOPATHY WITH MYOGLOBINURIA: DEMONSTRATION OF A GLYCOGENOLYTIC DEFECT IN THE MUSCLE*

1959 • 308 citations

THE METABOLIC BASIS OF INHERITED DISEASE

1966 • 249 citations

Evidence for the Common Identity of Glucose 6-Phosphatase, Inorganic Pyrophosphatase, and Pyrophosphate- Glucose Phosphotransferase

1964 • 238 citations

The Role of Hepatic Glucose-6-phosphatase in the Regulation of Carbohydrate Metabolism

1959 • 233 citations

A FUNCTIONAL DISORDER OF MUSCLE ASSOCIATED WITH THE ABSENCE OF PHOSPHORYLASE

1959 • 221 citations

A metabolic myopathy due to absence of muscle phosphorylase

1961 • 208 citations

ACTION OF AMYLO-1,6-GLUCOSIDASE AND PHOSPHORYLASE ON GLYCOGEN AND AMYLOPECTIN

1951 • 186 citations

Glucose-6-phosphatase deficiency glycogen storage disease. Studies on the interrelationships of carbohydrate, lipid, and purine abnormalities.

1962 • 183 citations

GLYCOGEN SYNTHESIS IN MUSCLE LACKING PHOSPHORYLASE

1959 • 171 citations

Familial cirrhosis of the liver with storage of abnormal glycogen.

1956 • 158 citations

THE DISTRIBUTION OF GLUCOSE-6-PHOSPHATASE IN THE LIVER AND KIDNEY OF THE MOUSE

1953 • 155 citations

Structure of Glycogens and Amylopectins

1952 • 143 citations

GLUCOSE-6-PHOSPHATASE DEFICIENCY GLYCOGEN STORAGE DISEASE

1962 • 143 citations

The mechanism of carbohydrase action. 8. Structures of the muscle-phosphorylase limit dextrins of glycogen and amylopectin

1960 • 142 citations

Lack of an alpha-1,4-glucan: alpha-1,4-glucan 6-glycosyl transferase in a case of type IV glycogenosis.

1966 • 139 citations

Glycogen structure and enzyme deficiencies in glycogen storage disease.

1952 • 138 citations

Metabolism of Inorganic Pyrophosphate

1964 • 129 citations

AMYLO-1, 6-GLUCOSIDASE IN MUSCLE TISSUE IN GENERALIZED GLYCOGEN STORAGE DISEASE

1956 • 117 citations

Metabolism of Inorganic Pyrophosphate

1964 • 112 citations

Hereditary Absence of Muscle Phosphorylase (McArdle's Syndrome)

1961 • 99 citations

Late-Onset Type of Skeletal-Muscle Phosphorylase Deficiency

1963 • 96 citations

On the conversion of fructose to glucose by guinea pig intestine

1960 • 91 citations

Glycogen storage disease

1953 • 88 citations

GLYCOGEN IN ERYTHROCYTES OF PATIENTS WITH GLYCOGEN STORAGE DISEASE

1961 • 86 citations

Liver Microsomal Glucose 6-Phosphatase, Inorganic Pyrophosphatase, and Pyrophosphate-Glucose Phosphotransferase

1965 • 85 citations

THE ACTION OF BRANCHING ENZYMES ON OUTER CHAINS OF GLYCOGEN

1953 • 85 citations

Skeletal muscle glycogenosis: an investigation of two dissimilar cases

1963 • 81 citations

McArdle's Disease

1963 • 76 citations

FURTHER STUDIES ON THE HISTOCHEMISTRY OF GLUCOSE-6-PHOSPHATASE

1955 • 73 citations

STUDIES OF GLYCOGEN METABOLISM IN LIVER GLYCOGEN DISEASE (VON GIERKE'S DISEASE): SIX CASES WITH SIMILAR METABOLIC ABNORMALITIES AND RESPONSES TO GLUCAGON*

1961 • 72 citations

Glycogen disease of the liver (von Gierke's disease) with hepatomata; case report with metabolic studies.

1955 • 71 citations

GALACTOSE TOLERANCE IN GLYCOGEN STORAGE DISEASE

1957 • 66 citations

GLYCOGEN DISEASE OF THE LIVER (VON GIERKE'S DISEASE) WITH HEPATOMATA

1955 • 64 citations

LEUKOCYTE DEBRANCHING ENZYME IN GLYCOGEN STORAGE DISEASE

1963 • 64 citations

Phosphoric Esters of Biological Importance. VI. The Synthesis of D-Glucosamine 6-Phosphate and N-Acetyl-D-glucosamine 6-Phosphate¹

1956 • 64 citations

Cardiovascular and Metabolic Responses to Exercise in a Patient with McArdle's Syndrome

1966 • 59 citations

Studies on carbohydrate-metabolizing enzymes. 9. The action of isoamylase on amylose

1963 • 56 citations

Liver glucose-6-phosphatase and pyrophosphate-glucose phosphotransferase: Effects of fasting

1965 • 55 citations

Studies in Glycogen Storage Diseases. I. Intestinal Glucose-6-Phosphatase Activity in Patients with Von Gierke's Disease and Their Parents*

1965 • 55 citations

A new substrate for the detection of amylo-1,6-glucosidase, and its application to the study of glycogen-storage disease Type III

1966 • 52 citations

Gout and Glycogen Storage Disease

1963 • 51 citations

Glycogen Storage Disease

1961 • 51 citations

THE PROPERTIES OF AN OLIGO-1,4 → 1,4-GLUCANTRANSFERASE FROM ANIMAL TISSUES

1962 • 50 citations

Type IV glycogenosis.

1966 • 50 citations

ENZYMES IN A GLYCOGEN STORAGE MYOPATHY

1959 • 48 citations

Type II Glycogenosis

1966 • 47 citations

Glycogen storage disease

1965 • 47 citations

Amylo-1,6-glucosidase activity in normal leucocytes and in leucocytes of patients with glycogen-storage disease

1964 • 46 citations

a-1,4-glucan: a-1,4-glucan 6-glycosyltransferase from liver

1962 • 46 citations

The substrate specificity of liver glucose-6-phosphatase

1955 • 43 citations

A study of the reaction catalysed by the liver branching enzyme

1966 • 42 citations

α-1,4-Glucosidase Activity in Leucocytes from the Family of Two Brothers Who Lack this Enzyme in Muscle

1966 • 39 citations

Carbohydrate Metabolism

1952 • 38 citations

Type III glycogenosis

1966 • 37 citations

Glucagon tolerance test in glycogen storage disease

1962 • 36 citations

Cori's Disease (Amylo-1.6-Glucosidase Deficiency)

1963 • 36 citations

THE EFFECTS OF GLUCAGON IN THREE FORMS OF GLYCOGEN STORAGE DISEASE*

1962 • 34 citations

Chronische hepatogene Hypoglykämie im Kindesalter

1932 • 34 citations

A Family Study of Phosphorylase Deficiency in Muscle

1965 • 33 citations

The hyperlipidemia in glycogen storage disease.

1966 • 33 citations

Glucose-6-phosphatase in human jejunal mucosa lack of activity in glycogenosis of cori's type 1

1964 • 32 citations

ACTION OF AMYLO-1,6-GLUCOSIDASE ON LOW MOLECULAR WEIGHT SUBSTRATES AND THE ASSAY OF THIS ENZYME IN GLYCOGEN STORAGE DISEASE

1962 • 31 citations

Glucose-6-phosphatase in human jejunal mucosa properties demonstrating the specific character of the enzyme activity

1965 • 31 citations

Amylo-1,6-glucosidase activity in leucocytes from patients with glycogen storage disease

1963 • 30 citations

Glucose-6-phosphatase and pyrophosphatase activities of homogenates of livers from patients with glycogen storage disease

1965 • 27 citations

886. α-1,4-Glucosans. Part XV. Structural analysis of glycogens on a milligram scale

1962 • 26 citations

Liver glycogen disease

1964 • 26 citations

Biochemical investigation of a case of glycogen-storage disease (von Gierke's disease)

1960 • 24 citations

Blood Lactic Acid in Liver Glycogen Disease

1943 • 24 citations

Clinical Course of Glycogen Disease

1963 • 23 citations

ENZYME INDUCTION IN A CASE OF GLYCOGEN STORAGE DISEASE

1966 • 22 citations

"Double Barrel" Glucagon Test

1965 • 22 citations

Detection of Heterozygous Carriers in Glycogen Storage Disease of the Liver (von Gierke's Disease)

1959 • 20 citations

Differential diagnosis of the type of glycogen disease in two adult patients with long history of glycogenosis

1964 • 20 citations

"DOUBLE BARREL" GLUCAGON TEST. CORRELATION WITH ENZYME ASSAYS IN LIMIT DEXTRINOSIS.

1965 • 19 citations

Intestinal Glucose-6-Phosphatase in Control Subjects and Relatives of Patient with Glycogen Storage Disease

1963 • 19 citations

Action of a muscle branching enzyme on polysaccharides enlarged from UDP[14C]glucose

1966 • 19 citations

Heterogeneous glycogen storage disease in siblings and favorable response to synthetic androgen administration

1962 • 19 citations

Hexose monophosphate, pyruvate and lactate In the peripheral blood in glycogen-storage disease Type I

1962 • 17 citations

An inborn error of lipid metabolism.

1959 • 13 citations

Phosphorylated glycolytic intermediates in liver and blood cells in members of a family with glycogenosis type I

1963 • 12 citations

α-1 : 4-Glucosans. Part II. The molecular structure of the liver glycogen from a case of von Gierke's disease

1954 • 11 citations

AN INBORN ERROR OF LIPID METABOLISM

1959 • 11 citations

Glucose-6-Phosphate Levels and Liver Glycogen Disease

1962 • 4 citations