Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients

Urea cycle disorders (UCDs) are a group of inborn errors of hepatic metabolism caused by the loss of enzymatic activities that mediate the transfer of nitrogen from ammonia to urea. These disorders often result in life-threatening hyperammonemia and hyperglutaminemia. A combination of sodium phenylb...

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Bibliographic Details
Published in:	Molecular genetics and metabolism Vol. 81; pp. 79 - 85
Main Authors:	Scaglia, Fernando, Carter, Susan, O’Brien, William E, Lee, Brendan
Format:	Journal Article
Language:	English
Published:	United States Elsevier Inc 01-04-2004
Subjects:	Adolescent Adult Amino Acid Metabolism, Inborn Errors - drug therapy Amino Acids, Branched-Chain - metabolism Branched chain amino acids Citrullinemia - drug therapy Drug Therapy, Combination Female Humans Male Ornithine Carbamoyltransferase Deficiency Disease - drug therapy Phenylacetates - pharmacokinetics Phenylacetates - therapeutic use Phenylbutyrates - pharmacokinetics Phenylbutyrates - therapeutic use Retrospective Studies Sodium Benzoate - pharmacokinetics Sodium Benzoate - therapeutic use Sodium phenylacetate Sodium phenylbutyrate Time Factors Urea - metabolism Urea cycle disorders Sodium phenylbutyrate Urea cycle disorders Branched chain amino acids Sodium phenylacetate
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