Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders

Molecular Genetics and Metabolism

Volumn 113, Issue 1, 2014, Pages 131-135

  Burrage, Lindsay C ^a   Jain, Mahim ^a   Gandolfo, Laura ^b   Lee, Brendan ^c   Batshaw, Mark L ^c   Tuchman, Mendel ^c   Summar, Marshall L ^c   Baumgartner, Matthias R ^c   Berry, Susan A ^c   Cederbaum, Stephen ^c   Diaz, George A ^c   Feigenbaum, Annette ^c   Gallagher, Renata C ^c   Harding, Cary O ^c   Hoffmann, Georg ^c   Kerr, Douglas S ^c   Lichter Konecki, Uta ^c   McCandless, Shawn E ^c   Merritt, J Lawrence ^c   Schulze, Andreas ^c   Seashore, Margretta R ^c   Stricker, Tamar ^c   Waisbren, Susan ^c   Wong, Derek ^c   Yudkoff, Mark ^c   Nagamani, Sandesh C S ^a   more..

^a BAYLOR COLLEGE OF MEDICINE   (United States)

^b UNIVERSITY OF SOUTH FLORIDA   (United States)

^c HOWARD HUGHES MEDICAL INSTITUTE   (United States)

Author keywords

Branched chain amino acids; Sodium phenylbutyrate; Urea cycle disorder

Indexed keywords

4 PHENYLBUTYRIC ACID; BRANCHED CHAIN AMINO ACID; ESSENTIAL AMINO ACID; ARYLBUTYRIC ACID DERIVATIVE;

ADOLESCENT; ADULT; AGED; AMINO ACID DEFICIENCY; AMINO ACID METABOLISM; ARTICLE; BRANCHED CHAIN AMINO ACID DEFICIENCY; CLINICAL EVALUATION; COHORT ANALYSIS; CONFOUNDING VARIABLE; CONTROLLED STUDY; CORRELATION ANALYSIS; FEMALE; HUMAN; HYPERAMMONEMIA; INFANT; LONGITUDINAL STUDY; MAJOR CLINICAL STUDY; MALE; MAPLE SYRUP URINE DISEASE; NEWBORN; SIDE EFFECT; UREA CYCLE DISORDER; BLOOD; CHILD; DEFICIENCY; MIDDLE AGED; PRESCHOOL CHILD; UREA CYCLE DISORDERS, INBORN; YOUNG ADULT;

ADOLESCENT; ADULT; AGED; AMINO ACIDS, BRANCHED-CHAIN; AMINO ACIDS, ESSENTIAL; CHILD; CHILD, PRESCHOOL; FEMALE; HUMANS; HYPERAMMONEMIA; INFANT; INFANT, NEWBORN; MALE; MIDDLE AGED; PHENYLBUTYRATES; UREA CYCLE DISORDERS, INBORN; YOUNG ADULT;

EID: 84908550679     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2014.06.005     Document Type: Article

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