DICARBOXYLIC ACIDURIA DUE TO MEDIUM CHAIN ACYL CoA DEHYDROGENASE DEFECT

The syndrome of systemic carnitine deficiency

1975 • 397 citations

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

1976 • 229 citations

Glutaric aciduria Type II

1980 • 214 citations

Jamaican Vomiting Sickness

1976 • 208 citations

An enzymatic fluorimetric micromethod for the determination of acetoacetate, β-Hydroxybutyrate, pyruvate and lactate

1971 • 184 citations

The mechanism of fatty acid uptake by heart mitochondria: An acylcarnitine‐carnitine exchange

1975 • 174 citations

In vitro fibroblast studies in a patient with C6-C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase

1982 • 168 citations

The occurrence of adipic and suberic acid in urine from ketotic patients

1972 • 162 citations

Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria

1976 • 134 citations

Recurrent Hypoglycemia Associated with Glutaric Aciduria Type II in an Adult

1979 • 116 citations

Researches on fat metabolism. II

1934 • 114 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

Oxidation of Fatty Acids in Cultured Fibroblasts: a Model System for the Detection and Study of Defects in Oxidation

1982 • 91 citations

Studies on fatty acid ω-oxidation antiketogenic effect and gluconeogenicity of dicarboxylic acids

1977 • 69 citations

The occurrence of C6–C10-dicarboxylic acids in urine from patients and rats treated with dipropylacetate

1980 • 52 citations

Intermittent non‐ketotic dicarboxylic aciduria in two siblings with hypoglycaemia: An apparent defect in β‐oxidation of fatty acids

1979 • 52 citations

Non-ketotic C6-C10-dicarboxylic aciduria: Biochemical investigations of two cases

1980 • 49 citations

Dicarboxylic aciduria: The response to fasting

1979 • 48 citations

Twin Siblings with a Reye's-Like Syndrome Associated with an Abnormal Organic Aciduria, Hypoglycemia, Diarrhea, and Vomiting with Close Similarities to Jamaican Vomiting Sickness

1980 • 35 citations

Medium-chain triglyceride medication as a pitfall in the diagnosis of non-ketotic C6-C10-dicarboxylic acidurias

1980 • 33 citations

Urinary excretion of C6–C10 dicarboxylic acids in glycogen storage disease types I and III

1974 • 30 citations

Profiles in altered metabolism. III—(Ω-1)-hydroxyacid excretion in a case of episodic hypoglycemia

1980 • 26 citations

Urinary organic acid profiles of reye's syndrome patients

1977 • 24 citations

3‐Hydroxy‐3‐methylglutaric aciduria combined with 3‐methylglutaconic aciduria: A new case

1981 • 17 citations

THE EXCRETION OF C₆–C₁₀‐DICARBOXYLIC ACIDS IN THE URINE OF NEWBORN INFANTS DURING STARVATION

1979 • 8 citations

Methods for measurement of fatty acid β‐oxidation and acyl‐CoA dehydrogenase activity in cultured fibroblasts

1982 • 8 citations

DICARBOXYLIC ACIDURIA DURING KETOTIC PHASES IN VARIOUS TYPES OF GLYCOGEN STORAGE DISEASE

1981 • 3 citations