Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate. [electronic resource]
Producer: 19820917Description: 3300-4 p. digitalISSN:- 0027-8424
- 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
- Cells, Cultured
- Hemiterpenes
- Humans
- Keto Acids
- Ketone Oxidoreductases -- metabolism
- Kinetics
- Maple Syrup Urine Disease -- drug therapy
- Multienzyme Complexes -- metabolism
- Pyruvate Dehydrogenase Complex -- metabolism
- Substrate Specificity
- Thiamine -- therapeutic use
- Thiamine Pyrophosphate -- pharmacology
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Publication Type: Journal Article; Research Support, U.S. Gov't, P.H.S.
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