Heterogeneity in maple syrup urine disease: Aspects of cofactor requirement and complementation in cultured fibroblasts

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

A NEW SYNDROME: PROGRESSIVE FAMILIAL INFANTILE CEREBRAL DYSFUNCTION ASSOCIATED WITH AN UNUSUAL URINARY SUBSTANCE

1954 • 408 citations

THIAMINE-RESPONSIVE MAPLE-SYRUP-URINE DISEASE

1971 • 170 citations

A simplified method for the preparation of 14C-labelled branched-chain α-oxo acids

1972 • 153 citations

Metabolism of the white blood cells in maple-syrup-urine disease

1960 • 103 citations

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

1972 • 102 citations

Branched Chain α-Keto Acid Metabolism

1968 • 83 citations

Pyruvate Dehydrogenase Complex

1969 • 59 citations

Coenzyme A- and Nicotinamide Adenine Dinucleotide-dependent Branched Chain α-Keto Acid Dehydrogenase

1969 • 59 citations

Complementation Analysis of Maple Syrup Urine Disease in Heterokaryons derived from Cultured Human Fibroblasts

1973 • 53 citations

Maple Syrup Urine Disease: Coenzyme function and prenatal monitoring

1974 • 48 citations

Treatment of inborn errors of metabolism

1972 • 48 citations

Branched-chain alpha-keto acids isolated as oxime derivatives: Relationship to the corresponding hydroxy acids and amino acids in Maple Syrup Urine Disease

1974 • 45 citations

Rapid diagnosis of maple syrup urine disease (branched chain ketoaciduria) by micro-enzyme assay in leukocytes and fibroblasts

1973 • 36 citations

Classical Maple Syrup Urine Disease: Cofactor resistance

1972 • 25 citations

Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease

1972 • 22 citations

Biochemical studies on branched-chain oxoacid oxidases

1967 • 21 citations

5-Ethyl-2?-deoxyuridine: Absence of effects on the chromosomes of human lymphocytes and fibroblasts in culture

1974 • 20 citations

Viral‐induced fusion of human cells. I. Quantitative studies on the fusion of human diploid fibroblasts induced by sendai virus

1971 • 19 citations

Untersuchungen zur Ahornsirupkrankheit an zwei Familien

1964 • 10 citations