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Two siblings with episodic ketoacidosis and decreased activity of succinyl-CoA:3-ketoacid CoA-transferase in cultured fibroblasts

โœ Scribed by C. J. Pretorius; G. G. Loy Son; F. Bonnici; E. H. Harley


Publisher
Springer
Year
1996
Tongue
English
Weight
325 KB
Volume
19
Category
Article
ISSN
0141-8955

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Succinyl-CoA:3-ketoacid CoA transferase
โœ Xiang-Qian Song; Toshiyuki Fukao; Hiroh Watanabe; Haruo Shintaku; Ken Hirayama; ๐Ÿ“‚ Article ๐Ÿ“… 1998 ๐Ÿ› John Wiley and Sons ๐ŸŒ English โš– 412 KB ๐Ÿ‘ 1 views

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