The inhibition of pyruvate decarboxylation in rat brain by α-ketoisocaproic acid

The Determination of Enzyme Dissociation Constants

Hans Lineweaver, Dean Burk · Journal of the American Chemical Society · 1934 · 12.1K citations

Life Sciences, Biochemistry, Chemistry +13

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

John H. Menkes, Peter L. Hurst, John M. Craig · PEDIATRICS · 1954 · 411 citations

Medicine, Neurology, Pediatrics +8

THE METABOLIC BASIS OF INHERITED DISEASE

John B. Stanbury, James Β. Wyngaarden, Donald S. Fredrickson · The American Journal of the Medical Sciences · 1966 · 245 citations

Life Sciences, Genetics, Molecular Genetics +13

MAPLE SYRUP URINE DISEASE, WITH PARTICULAR REFERENCE TO DIETOTHERAPY

Selma E. Snyderman, Patricia M. Norton, Ellen Roitman et al. · PEDIATRICS · 1964 · 226 citations

Agriculture, Biochemistry, Nutrition +15

Intermittent Branched-Chain Ketonuria

Joseph Dancis, Joel Hutzler, T Rokkones · New England Journal of Medicine · 1967 · 112 citations

Life Sciences, Biochemistry, Nutrition +13