Normal 2‐aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase

Pyruvate Dehydrogenase, Substrate Specificity and Product Inhibition

1969 • 161 citations

MAPLE SYRUP DISEASE

1959 • 141 citations

The quantitative extraction and gas-liquid chromatographic determination of organic acids in urine

1972 • 127 citations

Enzyme activity in classical and variant forms of maple syrup urine disease

1972 • 102 citations

Mammalian α-Keto Acid Dehydrogenase Complexes

1969 • 98 citations

Fatal Lactic Acidosis in a Newborn Attributable to a Congenital Defect of Pyruvate Dehydrogenase

1976 • 86 citations

Branched Chain α-Keto Acid Metabolism

1968 • 83 citations

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

1980 • 73 citations

PYRUVIC AND α-KETOGLUTARIC OXIDASE OF ANIMAL TISSUE

1947 • 69 citations

Mammalian α-Keto Acid Dehydrogenase Complexes

1969 • 67 citations

Hypervalinemia. A defect in valine transamination.

1967 • 47 citations

HYPERVALINEMIA

1967 • 41 citations

Aspects of the molecular biology of lipoamide dehydrogenase.

1978 • 38 citations

Chapter 16 A Simple Biochemical Technique for the Detection of Mycoplasma Contamination of Cultured Cells

1975 • 30 citations

Branched chain amino acids as activators of branched chain ketoacid dehydrogenase

1978 • 20 citations

Metabolic studies on normal and pyruvate dehydrogenase deficient cultured human fibroblasts

1977 • 5 citations

Metabolic studies on normal and pyruvate dehydrogenase deficient cultured human fibroblasts

1977 • 4 citations

Deleted Work

1955 • 0 citations