Primary human fibroblasts from a maple syrup urine disease patient undergo apoptosis following exposure to physiological concentrations of branched chain amino acids

Annals of the New York Academy of Sciences

Volumn 926, Issue , 2000, Pages 116-121

  Jouvet, Philippe ^a   Kozma, Mary ^a   Mehmet, Huseyin ^a  

^a HAMMERSMITH HOSPITAL   (United Kingdom)

Author keywords

Apoptosis; Maple syrup urine disease; ketoacids

Indexed keywords

2 OXOACID; 2 OXOACID DEHYDROGENASE; BRANCHED CHAIN AMINO ACID; HEXANOIC ACID DERIVATIVE; ISOLEUCINE; LEUCINE; VALERIC ACID DERIVATIVE; VALINE;

APOPTOSIS; CELL DEATH; CONFERENCE PAPER; CONTROLLED STUDY; ENZYME DEFICIENCY; FIBROBLAST; HUMAN; HUMAN CELL; MAPLE SYRUP URINE DISEASE; METABOLITE; NERVE DEGENERATION;

ACER;

EID: 0034512868     PISSN: 00778923     EISSN: None     Source Type: Book Series    
DOI: 10.1111/j.1749-6632.2000.tb05604.x     Document Type: Conference Paper

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15. Effect of diets on the activity of enzymes involved in branched chain α-keto acid metabolism