The Clinical and Biochemical Implications of Pyruvate Carboxylase Deficiency

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

Immunoassay of Insulin: Two Antibody System: Plasma Insulin Levels of Normal, Subdiabetic and Diabetic Rats

1963 • 2,254 citations

Enzymic determination of <scp>d</scp>(−)-β-hydroxybutyric acid and acetoacetic acid in blood

1962 • 1,407 citations

Hormone-fuel interrelationships during fasting.

1966 • 1,032 citations

A Sensitive Double Antibody Immunoassay for Human Growth Hormone in Plasma

1964 • 645 citations

COENZYME A FUNCTION IN AND ACETYL TRANSFER BY THE PHOSPHOTRANSACETYLASE SYSTEM

1951 • 513 citations

Pyruvate Carboxylase

1963 • 439 citations

Effect of ketone infusions on amino acid and nitrogen metabolism in man.

1975 • 331 citations

Pyruvate Carboxylase

1963 • 324 citations

Active and inactive forms of pyruvate dehydrogenase in rat heart and kidney: Effect of diabetes, fasting, and refeeding on pyruvate dehydrogenase interconversion

1971 • 231 citations

Leigh's encephalomyelopathy: an inborn error of gluconeogenesis.

1968 • 201 citations

Rat Liver Pyruvate Carboxylase

1971 • 169 citations

Control of Hepatic Mitochondrial CO2 Fixation by Glucagon, Epinephrine, and Cortisol

1969 • 163 citations

The metabolic events of starvation

1976 • 152 citations

Subacute Necrotizing Encephalomyelopathy (Leigh's Disease): a Consideration of Clinical Features and Etiology

1972 • 145 citations

The Citrate Enzymes: Their Structures, Mechanisms, and Biological Functions

1972 • 123 citations

Ketonic Diet in the Management of Pyruvate Dehydrogenase Deficiency

1976 • 111 citations

Hepatic fructose-1,6-diphosphatase deficiency

1972 • 110 citations

Rat Liver Pyruvate Carboxylase

1971 • 96 citations

A microfluorometric enzymatic assay for the determination of alanine and pyruvate in plasma and tissues.

1972 • 94 citations

Rat Liver Pyruvate Carboxylase

1971 • 93 citations

Neonatal congential lactic acidosis with pyruvte carboxylase deficiency in two siblings.

1976 • 89 citations

THIAMINE-RESPONSIVE LACTIC ACIDOSIS IN A PATIENT WITH DEFICIENT LOW-KM PYRUVATE CARBOXYLASE ACTIVITY IN LIVER

1972 • 89 citations

The activity of pyruvate dehydrogenase in rat brain during postnatal development

1974 • 88 citations

Ketotic Hypoglycemia: An Amino Acid Substrate Limited Disorder*

1974 • 85 citations

Uncontrolled diabetes mellitus and hyperglucagonemia associated with an islet cell carcinoma

1975 • 77 citations

Brain glutamate decarboxylase: changes in the developing rat brain¹

1970 • 76 citations

[21] Extramitochondrial l-malate dehydrogenase of beef heart

1969 • 75 citations

Regulation of the Redox State of the Pyridine Nucleotides in Rat Liver

1970 • 71 citations

Biochemical studies and therapy in subacute necrotizing encephalomyelopathy (Leigh's syndrome)

1972 • 68 citations

TWO CASES OF PHOSPHOENOLPYRUVATE CARBOXYKINASE DEFICIENCY

1976 • 66 citations

Comparison of competitive protein binding radioassay of cortisol to double isotope dilution and porter silber methods

1970 • 63 citations

Immunochemical studies with soluble and mitochondrial pyruvate carboxylase activities from rat tissues

1970 • 60 citations

Pathogenesis of Leigh's encephalomyelopathy

1972 • 58 citations

REGULATION OF PALMITIC ACID SYNTHESIS IN CULTURED GLIAL CELLS: EFFECTS OF LIPID ON FATTY ACID SYNTHETASE, ACETYL‐CoA CARBOXYLASE, FATTY ACID AND STEROL SYNTHESIS¹

1975 • 56 citations

Rat Liver Pyruvate Carboxylase

1971 • 54 citations

Lactic Acidosis in Childhood

1975 • 47 citations

A comparison of the regulation of pyruvate dehydrogenase in mitochondria from rat brain and liver

1975 • 42 citations

Methods for Determination of Coenzyme A

1955 • 39 citations

Specificity of the urine inhibitor test for Leigh's disease

1974 • 39 citations

Purification and properties of human liver pyruvate carboxylase

1974 • 36 citations

Hyperalaninemia with Pyruvicemia due to Pyruvate Carboxylase Deficiency of the Liver

1969 • 36 citations

BIOCHEMICAL ABNORMALITIES IN LEIGH'S DISEASE

1976 • 31 citations

Hypoglycemia and Maple Syrup Urine Disease: Defective Gluconeogenesis

1973 • 29 citations

The incidence of lipid droplets in human skeletal muscle in neuromuscular disorders: A histochemical, electron‐microscopic and freeze‐etch study

1972 • 25 citations

Preferential labeling of brain cholesterol by {3‐¹⁴C}D(−)‐3‐hydroxybutyrate

1973 • 24 citations

Subcellular distribution of ketone body metabolizing enzymes in the rat brain¹

1976 • 16 citations

Pyruvate carboxylase in human liver

1974 • 9 citations

Purification and Properties of Pyruvate Carboxylase from Rat Liver

1971 • 7 citations

Relative importance of acetate, acetoacetate and D-β-OH-butyrate in the transport of acetyl CoA from the mitochondria to the cytoplasm for fatty acid synthesis in mice

1976 • 6 citations