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

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

Rat liver peroxisomes catalyze the beta oxidation of fatty acids.

1978 • 608 citations

Studies on glucosaminidase. 4. The fluorimetric assay of N-acetyl-β-glucosaminidase

1961 • 458 citations

Isovaleric acidemia: a new genetic defect of leucine metabolism.

1966 • 348 citations

Isovaleric Acidemia

1967 • 110 citations

Methylmalonicacidemia: biochemical heterogeneity in defects of 5'-deoxyadenosylcobalamin synthesis.

1975 • 83 citations

Identification of β-hydroxyisovaleric acid in the urine of a patient with isovaleric acidemia

1968 • 77 citations

Mechanism of action of coenzyme B12. Hydrogen transfer in the isomerization of methylmalonyl coenzyme A to succinyl coenzyme A

1969 • 64 citations

SUBCELLULAR DISTRIBUTION OF ISOCITRATE DEHYDROGENASES IN NEONATAL AND ADULT MOUSE BRAIN

1973 • 37 citations

Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects.

1976 • 34 citations

[50] Acyl-CoA dehydrogenases and electron-transferring flavoprotein

1978 • 33 citations

Oxidative phosphorylation in mitochondria isolated from human fibroblasts

1973 • 26 citations

In Vivo and in Vitro Response of Human Branched Chain α-Ketoacid Dehydrogenase to Thiamine and Thiamine Pyrophosphate

1978 • 24 citations

The stability of some enzymes in cultured cells

1969 • 17 citations

Possibility of inborn defect in isovalericacidaemia involving altered enzyme specificity rather than total inactivity

1974 • 11 citations