Metabolism of branched-chain amino acids in maple syrup urine disease

European Journal of Pediatrics, Supplement

Volumn 156, Issue 1, 1997, Pages

  Wendel, U ^a  

^a HEINRICH HEINE UNIVERSITY   (Germany)

Author keywords

Branched chain amino acids; Maple syrup urine disease; Metabolism; Protein turnover; Stable isotopes

Indexed keywords

BRANCHED CHAIN AMINO ACID; CARBON 13; CARBON DIOXIDE; ISOTOPE; LEUCINE;

AMINO ACID METABOLISM; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; BREATH ANALYSIS; CARBON DIOXIDE MEASUREMENT; CATABOLISM; CONTROLLED STUDY; HUMAN; INTRAVENOUS DRUG ADMINISTRATION; ISOTOPE LABELING; MAPLE SYRUP URINE DISEASE; METABOLIC DISORDER; ORAL DRUG ADMINISTRATION; OXIDATION; PRIORITY JOURNAL; PROTEIN METABOLISM;

AMINO ACIDS, BRANCHED-CHAIN; HUMANS; ISOTOPE LABELING; LEUCINE; MAPLE SYRUP URINE DISEASE; OXIDATION-REDUCTION; SEVERITY OF ILLNESS INDEX;

EID: 31344482641     PISSN: 09439676     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (2)

1. Collins JE, Umpleby AM, Boroujerdi MA, Leonard JV, Sonksen PH (1987) Effect of insulin on leucine kinetics in maple syrup urine disease. Pediatric Res 21: 10-13
2. Dancis J, Hutzler J, Levitz M (1960) Metabolism of the white blood cells in maple syrup urine disease. Biochim Biophys Acta 43: 342-343