Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: A severe fatty acid oxidation disorder

Prophylaxis of early ventricular fibrillation by inhibition of acylcarnitine accumulation.

1989 • 207 citations

Long-Chain Acyl Coenzyme A Dehydrogenase Deficiency: An Inherited Cause of Nonketotic Hypoglycemia

1985 • 203 citations

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

1983 • 202 citations

Fatty acid oxidation disorders: A new class of metabolic diseases

1992 • 178 citations

Human trifunctional protein deficiency: A new disorder of mitochondrial fatty acid β-oridation

1992 • 154 citations

Human liver long-chain 3-hydroxyacyl-coenzyme a dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria

1992 • 145 citations

Deficiency of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase: A Cause of Lethal Myopathy and Cardiomyopathy in Early Childhood

1990 • 122 citations

Long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency: Identification of a new inborn error of mitochondrial fatty acidβ‐oxidation

1990 • 121 citations

Identification of Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency in Three Patients Previously Diagnosed with Long-Chain Acyl-CoA Dehydrogenase Deficiency

1993 • 119 citations

Medium-chain and long-chain acyl CoA dehydrogenase deficiency: Clinical, pathologic and ultrastructural differentiation from Reye's syndrome

1986 • 111 citations

Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency

1991 • 110 citations

Fatty acid oxidation: Clinical, biochemical, and molecular aspects

1991 • 97 citations

3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment

1991 • 76 citations

Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

1991 • 70 citations

3-hydroxydicarboxylic aciduria—a fatty acid oxidation defect with severe prognosis

1990 • 70 citations

Medium- and Long-Chain Dicarboxylic Aciduria in Patients with Zellweger Syndrome and Neonatal Adrenoleukodystrophy

1986 • 66 citations

Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency

1992 • 61 citations

The L-3-hydroxyacyl-CoA dehydrogenase deficiency.

1990 • 52 citations

A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria

1993 • 48 citations

3-hydroxydecanedioic acid and related homologues: urinary metabolites in ketoacidosis.

1980 • 44 citations

Differential diagnosis of hydroxydicarboxylic aciduria based on release of³H₂O from [9,10‐³H]myristic and [9,10‐³H]palmitic acids by intact cultured fibroblasts

1992 • 44 citations

3-hydroxydecanedioic acid and related homologues: urinary metabolites in ketoacidosis.

1980 • 44 citations

Familial hypoketotic hypoglycaemia associated with peripheral neuropathy, pigmentary retinopathy and C₆–C₁₄ hydroxydicarboxylic aciduria. A new defect in fatty acid oxidation?

1988 • 39 citations

3‐Hydroxydicarboxylic aciduria: A distinctive type of intermittent dicarboxylic aciduria of possible diagnostic significance

1987 • 34 citations

Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency—diagnosis, plasma carnitine fractions and management in a further patient

1993 • 27 citations

Long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency: Different clinical expression in three unrelated patients

1991 • 23 citations

Clinical and biochemical presentations in 20 cases of hydroxydicarboxylic aciduria.

1990 • 22 citations

Fatty acid β-oxidation in leukocytes from control subjects and medium-chain acyl-CoA dehydrogenase deficient patients

1992 • 15 citations

Fatal outcome in a patient with long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency

1992 • 15 citations

The Existence of Two Different h-3-Hydroxyacyl-Coenzyme A Dehydrogenases in Rat Tissues

1979 • 11 citations

4-Hydroxycyclohexanecarboxylic acid: a rare compound in urinary organic acid analysis

1991 • 9 citations

Long‐chain 3‐hydroxyacyl‐CoA dehydrogenase in leukocytes and chorionic villus fibroblasts: Potential for pre‐ and postnatal diagnosis

1992 • 8 citations

Pathophysiological approach to carnitine palmitoyltransferase II deficiencies.

1992 • 7 citations

Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: Possible implications for human inborn disorders of mitochondrialβ‐oxidation

1988 • 5 citations

Plasma and mitochondrial membrane carnitine transport defects.

1992 • 4 citations