Octanoic acidemia and octanoylcarnitine excretion with dicarboxylic aciduria due to defective oxidation of medium-chain fatty acids

The Cerebrohepatorenal (Zellweger) Syndrome

1984 • 268 citations

Systemic Carnitine Deficiency — A Treatable Inherited Lipid-Storage Disease Presenting as Reye's Syndrome

1980 • 249 citations

Adrenoleukodystrophy: Impaired Oxidation of Very Long Chain Fatty Acids in White Blood Cells, Cultured Skin Fibroblasts, and Amniocytes

1984 • 223 citations

Jamaican Vomiting Sickness

1976 • 208 citations

Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Children with Non-Ketotic Hypoglycemia and Low Carnitine Levels

1983 • 202 citations

Fasting hypoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency

1981 • 173 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

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

1982 • 168 citations

Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria

1976 • 134 citations

Hyperinsulinism in Infancy: Diagnosis by Demonstration of Abnormal Response to Fasting Hypoglycemia

1976 • 133 citations

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

1983 • 112 citations

General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases

1983 • 93 citations

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

1982 • 91 citations

Lethal hypoglycemia in a child with a deficiency of 3-hydroxy-3-methylglutarylcoenzyme A lyase

1979 • 58 citations

Carnitine and Acylcarnitines

1974 • 54 citations

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

1979 • 52 citations

Purification and properties of acyl coenzyme A dehydrogenases from bovine liver. Formation of 2-trans,4-cis-decadienoyl coenzyme A.

1984 • 46 citations

Exercise-induced asthma after walking, running and cycling

1982 • 40 citations

Gas chromatography of urinary N-phenylacetylglutamine

1979 • 33 citations

Regional Cerebral Na+K+ ATPase Activity following Octanoate Administration

1980 • 31 citations

Effects of octanoate on rat brain and liver mitochondria

1983 • 27 citations

Episodic Hypoglycemia with Ψ-Hydroxy Fatty Acid Excretion

1983 • 25 citations

C6-C10-Dicarboxylic aciduria: Biochemical considerations in relation to diagnosis of β-oxidation defects

1982 • 24 citations

A method for serum octanoate in hepatic cirrhosis and hepatic encephalopathy.

1977 • 18 citations

The analysis of plasma and urinary organic acids during prolonged fasting differentiates between systemic carnitine deficiency and a defect of fatty acid oxidation

1983 • 17 citations

Mechanism of free fatty acid-induced arrhythmias

1982 • 15 citations

Long-Chain Acyl-CoA Dehydrogenase Deficiency

2009 • 3 citations