Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia

Biochemistry

Volumn 37, Issue 28, 1998, Pages 10325-10335

  Mohsen, Al Walid A ^a   Anderson, Bambi D ^a   Volchenboum, Samuel L ^b   Battaile, Kevin P ^b,c   Tiffany, Karen ^d   Roberts, David ^d   Kim, Jung Ja P ^d   Vockley, Jerry ^a,b  

^a MAYO CLINIC   (United States)

^b MAYO GRADUATE SCHOOL   (United States)

^c OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

^d MEDICAL COLLEGE OF WISCONSIN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ISOVALERIC ACID; ISOVALERYL COENZYME DEHYDROGENASE; UNCLASSIFIED DRUG;

ACIDEMIA; AMINO ACID SUBSTITUTION; ARTICLE; CELL CULTURE; DNA DETERMINATION; HUMAN; HUMAN CELL; IMMUNOBLOTTING; MUTATION; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DETERMINATION; PROTEIN STABILITY;

AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; CELLS, CULTURED; CRYSTALLOGRAPHY, X-RAY; DNA MUTATIONAL ANALYSIS; DNA, COMPLEMENTARY; ENZYME STABILITY; ESCHERICHIA COLI; FIBROBLASTS; HUMANS; ISOVALERYL-COA DEHYDROGENASE; KINETICS; MALE; MITOCHONDRIA, LIVER; OXIDOREDUCTASES; OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS; RATS; RATS, SPRAGUE-DAWLEY; RECOMBINANT PROTEINS; SPECTROPHOTOMETRY;

EID: 0032516429     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi973096r     Document Type: Article

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