The multiple acyl-coenzyme A dehydrogenation disorders, glutaric aciduria type II and ethylmalonic-adipic aciduria. Mitochondrial fatty acid oxidation, acyl-coenzyme A dehydrogenase, and electron transfer flavoprotein activities in fibroblasts.

The Natural History of the Inherited Methylmalonic Acidemias

1983 • 317 citations

Glutaric aciduria type II: Report on a previously undescribed metabolic disorder

1976 • 229 citations

Glutaric aciduria Type II

1980 • 214 citations

Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase.

1983 • 171 citations

Deficiency of electron transfer flavoprotein or electron transfer flavoprotein:ubiquinone oxidoreductase in glutaric acidemia type II fibroblasts.

1985 • 152 citations

Purification and Properties of Rat Liver Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein1

1981 • 125 citations

C6—C10-Dicarboxylic Aciduria: Investigations of a Patient with Riboflavin Responsive Multiple Acyl-CoA Dehydrogenation Defects

1982 • 121 citations

Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria.

1983 • 117 citations

Recurrent Hypoglycemia Associated with Glutaric Aciduria Type II in an Adult

1979 • 116 citations

The purification and some properties of electron transfer flavoprotein and general fatty acyl coenzyme A dehydrogenase from pig liver mitochondria.

1975 • 112 citations

Dicarboxylic Aciduria: Deficient [1- ¹⁴ C]Octanoate Oxidation and Medium-Chain Acyl-CoA Dehydrogenase in Fibroblasts

1983 • 112 citations

Ethylmalonic-Adipic Aciduria

1979 • 110 citations

Purification and characterization of 2-methyl-branched chain acyl coenzyme A dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria.

1983 • 109 citations

Multiple Acyl-CoA Dehydrogenase Deficiency (Glutaric Aciduria Type II) with Transient Hypersarcosinemia and Sarcosinuria; Possible Inherited Deficiency of an Electron Transfer Flavoprotein

1980 • 97 citations

Electron transfer flavoprotein-ubiquinone oxidoreductase from pig liver: purification and molecular, redox, and catalytic properties

1985 • 95 citations

Separation, properties, and regulation of acyl coenzyme A dehydrogenases from bovine heart and liver

1982 • 85 citations

Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two brothers

1982 • 83 citations

Multiple Acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two newborn brothers

1982 • 82 citations

Glutaric acidaemia type II (multiple Acyl‐Coa dehydrogenation deficiency)

1984 • 77 citations

Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.

1980 • 73 citations

Biochemical studies in a patient with defects in the metabolism of acyl‐CoA and sarcosine: Another possible case of glutaric aciduria type II

1980 • 63 citations

Glutaric acidemia type II: Clinical, biochemical, and morphologic considerations

1982 • 63 citations

Purification and properties of electron-transferring flavoprotein from pig kidney

1982 • 63 citations

Reaction of electron-transfer flavoprotein with electron transfer flavoprotein-ubiquinone oxidoreductase

1985 • 56 citations

The effect of tetrahydrofolate on the reduction of electron transfer flavoprotein by sarcosine and dimethylglycine dehydrogenases

1982 • 50 citations

Inhibition of general acyl CoA dehydrogenase by electron transfer flavoprotein semiquinone

1981 • 47 citations

Multiple acyl-CoA dehydrogenase deficiency occurring in pregnancy and caused by a defect in riboflavin metabolism in the mother

1983 • 44 citations

Glutaric aciduria; Presence of glutaconic and β-hydroxyglutaric acids in urine

1975 • 43 citations

Riboflavin deficiency and β‐oxidation systems in rat liver

1982 • 42 citations

The acyl-CoA dehydrogenation deficiencies. Recent advances in the enzymic characterization and understanding of the metabolic and pathophysiological disturbances in patients with acyl-CoA dehydrogenation deficiencies.

1985 • 40 citations

Riboflavin‐responsive ethylmalonic—adipic aciduria

1984 • 38 citations

Glutaric Aciduria Type II. In Vitro Studies on Substrate Oxidation, Acyl-CoA Dehydrogenases, and Electron-Transferring Flavoprotein in Cultured Skin Fibroblasts

1980 • 38 citations

Neonatal glutaric aciduria type II: An X-linked recessive inherited disorder

1981 • 38 citations

Glutaric aciduria type II: Biochemical investigation and treatment of a child diagnosed prenatally

1983 • 36 citations

Novel tritium release assays for isovaleryl-CoA and butyryl-CoA dehydrogenases.

1981 • 35 citations

Multiple acyl-Co A dehydrogenation deficiency (MADD) in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy. Detection of N-isovalerylglutamic acid and its monoamide.

1983 • 34 citations

An essential cysteine residue located in the vicinity of the FAD-binding site in short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria.

1985 • 33 citations

Glutaric Aciduria Type II: Evidence for a Defect Related to the Electron Transfer Flavoprotein or Its Dehydrogenase

1984 • 32 citations

Induction of Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein and Their Roles in Fatty Acid Oxidation in Rat Liver Mitochondria1

1981 • 29 citations

Glutaric aciduria type II: treatment with riboflavine, carnitine and insulin

1984 • 26 citations

Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients.

1985 • 19 citations

The urinary excretion of ethylmalonic acid: What level requires further attention?

1983 • 17 citations

Electron-transferring Flavoprotein Deficiency in the Multiple Acyl-CoA Dehydrogenation Disorders, Glutaric Aciduria Type II and Ethylmalonic—adipic Aciduria

1984 • 11 citations

Glutaryl-CoA Dehydrogenase Activity Determined with Intact Electron-transport Chain: Application to Glutaric Aciduria Type II

1984 • 5 citations

Glutaric Acidaemia Type II (Multiple Acyl-CoA Dehydrogenation Deficiency)

1984 • 4 citations

Chemical Characterization of Two Folate-Binding Proteins from Mitochondria: Dimethylglycine Dehydrogenase and Sarcosine Dehydrogenase

1983 • 1 citations