Lessons from genetic disorders of branched-chain amino acid metabolism

Journal of Nutrition

Volumn 136, Issue 1, 2006, Pages

  Chuang, David T ^a   Chuang, Jacinta L ^a   Wynn, R Max ^a  

^a UNIVERSITY OF TEXAS AT DALLAS   (United States)

Author keywords

Branched chain amino acid metabolism; Branched chain ketoacid dehydrogenase; E2 deficiency; Maple Syrup Urine Disease; Thiamin diphosphate; Thiamin supplementation; Thiamin responsive MSUD

Indexed keywords

2 OXOACID; 2 OXOACID DEHYDROGENASE; 4 AMINOBUTYRIC ACID; BRANCHED CHAIN AMINO ACID; CARBOXYLYASE; COCARBOXYLASE; GLUTAMIC ACID; GLUTAMINE; GLYCOPROTEIN E2; OXIDOREDUCTASE; THIAMINE;

ACIDURIA; AMINO ACID BLOOD LEVEL; AMINO ACID METABOLISM; BRAIN CORTEX; BRAIN DISEASE; CONFERENCE PAPER; CORRELATION ANALYSIS; DECARBOXYLATION; DIET SUPPLEMENTATION; DIETARY INTAKE; DISEASE SEVERITY; ENZYME ASSAY; GENE LOCUS; GENETIC DISORDER; HUMAN; LIVER TRANSPLANTATION; MAPLE SYRUP URINE DISEASE; PHENOTYPE;

ACER;

EID: 31544455515     PISSN: 00223166     EISSN: 15416100     Source Type: Journal    
DOI: 10.1093/jn/136.1.243s     Document Type: Conference Paper

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