A new variant of maple syrup urine disease (branched chain ketoaciduria)

Automatic Recording Apparatus for Use in Chromatography of Amino Acids

1958 • 9,612 citations

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

1954 • 408 citations

Amino Acid Metabolism and Genetic Variation.

1968 • 263 citations

MAPLE SYRUP URINE DISEASE, WITH PARTICULAR REFERENCE TO DIETOTHERAPY

1964 • 229 citations

"Maple Syrup Urine Disease"

1959 • 227 citations

Effect of Sodium Lactate Infusion on Urate Clearance in Man.

1957 • 164 citations

MAPLE SYRUP DISEASE

1959 • 141 citations

Methylmalonic Aciduria

1968 • 129 citations

Renal Retention of Uric Acid Induced by Infusion of Beta-Hydroxybutyrate and Acetoacetate

1965 • 126 citations

"Maple Syrup Urine Disease"

1959 • 121 citations

Intermittent Branched-Chain Ketonuria

1967 • 113 citations

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

1960 • 103 citations

INTERMITTENT CEREBELLAR ATAXIA ASSOCIATED WITH HYPERPYRUVIC ACIDEMIA, HYPERALANINEMIA, AND HYPERALANINURIA

1969 • 95 citations

The effects of ACTH and cortisone on the renal tubular transport of uric acid, phosphorus, and electrolytes in patients with normal renal and adrenal function.

1951 • 86 citations

Hartnup Disease

1965 • 80 citations

THE DIAGNOSIS OF MAPLE SYRUP URINE DISEASE (BRANCHED- CHAIN KETOACIDURIA) BY THE IN VITRO STUDY OF THE PERIPHERAL LEUKOCYTE.

1963 • 76 citations

Influence of Dietary Protein Levels and Hydrocortisone Administration on the Branched-chain Amino Acid Transaminase Activity in Rat Tissues

1968 • 63 citations

Branched Chain α-Keto Acid Metabolism

1968 • 62 citations

Maple Syrup Urine Disease

1961 • 60 citations

THE DIAGNOSIS OF MAPLE SYRUP URINE DISEASE (Branched-chain Ketoaciduria)

1963 • 59 citations

The metabolism of leucine in tissue culture of skin fibroblasts of maple-syrup-urine disease

1963 • 53 citations

Late Manifesting Variant of Branched‐Chain Ketoaciduria (Maple Syrup Urine Disease)

1964 • 50 citations

Vitamin B6 Deficiency and Dependency in Man

1967 • 46 citations

Enzyme defect in skin fibroblasts in intermittent branched-chain detonuria and in maple syrup urine disease

1969 • 37 citations

Detection of the heterozygote in maple syrup urine disease

1965 • 31 citations

Dietary Treatment of Maple Syrup Urine Disease

1967 • 22 citations

Biochemical studies on branched-chain oxoacid oxidases

1967 • 21 citations

Late-onset branched-chain ketoaciduria: (maple syrup urine disease).

1966 • 20 citations

EAR COMPLICATIONS OF INFLUENZA

1932 • 20 citations

Alteration in the magnitude of induction of tyrosine transaminase by glucocorticoids. The effects of phenobarbital, o,p′DDD and β-diethylaminoethyl diphenylpropylacetate (SKF 525A)

1968 • 20 citations

Alteration in the magnitude of induction of tyrosine transaminase by glucocorticoids

1969 • 19 citations

Maple Syrup Urine Disease

2009 • 17 citations

THE ENZYME DEFECT IN MAPLE SYRUP URINE DISEASE

1967 • 14 citations

Ein weiterer Heterozygoten-Test f�r die Ahornsirupkrankheit (maple syrup urine disease)

1963 • 14 citations

Detection of heterozygotes in maple syrup urin disease: Role of lymphocyte count

1968 • 13 citations

Untersuchungen zur Ahornsirupkrankheit an zwei Familien

1964 • 10 citations

Cystathioninuria: Management

1967 • 10 citations

Heterozygotentest f�r die Ahorn-Sirup-Krankheit

1963 • 10 citations

WALSALL COTTAGE HOSPITAL.

1865 • 2 citations

Deleted Work

1955 • 0 citations