Correlations between branched-chain amino acids and branched-chain α-keto acids in blood in maple syrup urine disease

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

1954 • 408 citations

MAPLE SYRUP URINE DISEASE, WITH PARTICULAR REFERENCE TO DIETOTHERAPY

1964 • 229 citations

A New Design of a Thermionic Nitrogen and Phosphorus Detector for GC

1974 • 134 citations

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

1960 • 103 citations

STUDIES ON d- AND l-α-KETO-β-METHYLVALERIC ACIDS

1951 • 72 citations

Quantitative gas chromatography and single-ion detection of aliphatic α-keto acids from urine as their o-trimethylsilylquinoxalinol derivatives

1977 • 63 citations

Thermionic detectors in gas chromatography

1970 • 62 citations

Effects of Branched-chain Amino Acid Antagonism in the Rat on Tissue Amino Acid and Keto Acid Concentrations

1977 • 61 citations

Determination of α-keto acids as silylated oximes in urine and serum by combined gas chromatography-mass spectrometry

1973 • 53 citations

Quantitative analysis of branched-chain α-keto acids as their trimethylsilylated oximes

1973 • 52 citations

Urinary organic acids in man. III. Quantitative ranges and patterns of excretion in a normal population.

1976 • 49 citations

Investigation of the metabolic pattern in maple syrup urine disease by means of glass capillary gas chromatography and mass spectrometry

1977 • 49 citations

Derivatives for the identification and quantitative determination of some keto and aldo-carboxylic acids by gas-liquid chromatography

1972 • 46 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

Accelerated chromatography of amino acids associated with phenylketonuria, leucinosis (maple syrup urine disease), and other inborn errors of metabolism

1967 • 38 citations

Statistische auswertungsmethoden

1971 • 32 citations

Quinoxalinol derivatives of aliphatic 2-oxocarboxylic acids. Infrared and mass spectra of theO-trimethylsilylated compounds

1977 • 31 citations

Pathogenesis of maple-syrup-urine disease: observations during dietary management and treatment of coma by peritoneal dialysis

1969 • 28 citations

Maple syrup urine disease: branched-chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids.

1977 • 23 citations

O-trimethylsilylquinoxalinol derivatives of aromatic α-keto acids

1978 • 22 citations

Maple Syrup Urine Disease: Analysis of Branched Chain Ketoacid Decarboxylation in Cultured Fibroblasts

1975 • 22 citations

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

1972 • 22 citations

The configuration of the alloisoleucine present in maple syrup urine disease plasma

1970 • 18 citations

Gas chromatography—mass spectrometry studies of hipuric acid derivatives

1973 • 10 citations

Quantitative gas chromatography of α-keto acids with a nitrogen-selective detector

1978 • 8 citations

Determination of urinary phenylpyruvic acid in phenylketonurics by enol-borate method

1974 • 3 citations

Dietary treatment of maple sirup urine disease (branched-chain ketoaciduria)

1976 • 3 citations