Chuang, D T <br/><br/>
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] 

 -  Proceedings of the National Academy of Sciences of the United States of America  May 1982 
 - 3300-4 p.  digital 
<br/><br/> Publication Type: Journal Article; Research Support, U.S. Gov't, P.H.S. 
<br/><br/><label>ISSN: </label>0027-8424 
<br/><br/><label>Standard No.: </label>10.1073/pnas.79.10.3300  doi 
<br/><br/><label>Subjects--Topical Terms: </label><br/>3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)<br/>Cells, Cultured<br/>Hemiterpenes<br/>Humans<br/>Keto Acids<br/>Ketone Oxidoreductases--metabolism<br/>Kinetics<br/>Maple Syrup Urine Disease--drug therapy<br/>Multienzyme Complexes--metabolism<br/>Pyruvate Dehydrogenase Complex--metabolism<br/>Substrate Specificity<br/>Thiamine--therapeutic use<br/>Thiamine Pyrophosphate--pharmacology