Succinyl-CoA:3-ketoacid CoA transferase
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Xiang-Qian Song; Toshiyuki Fukao; Hiroh Watanabe; Haruo Shintaku; Ken Hirayama;
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Article
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1998
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John Wiley and Sons
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English
⚖ 412 KB
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Succinyl-CoA:3-ketoacid CoA transferase (SCOT; EC 2.8.3.5; locus symbol OXCT) is the key enzyme of ketone body utilization. Hereditary SCOT deficiency (MIM 245050) causes episodes of severe ketoacidosis. We developed a transient expression system for mutant SCOT cDNAs, using immortalized SCOT-defici