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Decreased Succinyl-CoA 3-ketoacid CoA transferase activity

MedGen UID:
1841916
•Concept ID:
C5826892
•
Finding
Synonym: SCOT activity low in fibroblasts
 
HPO: HP:4000162

Definition

Decreased activity of the enzyme succinyl-CoA 3-ketoacid CoA transferase. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDecreased Succinyl-CoA 3-ketoacid CoA transferase activity

Recent clinical studies

Diagnosis

Low ketolytic enzyme levels in tumors predict ketogenic diet responses in cancer cell lines in vitro and in vivo.
Zhang J, Jia PP, Liu QL, Cong MH, Gao Y, Shi HP, Yu WN, Miao MY
J Lipid Res 2018 Apr;59(4):625-634. Epub 2018 Feb 5 doi: 10.1194/jlr.M082040. PMID: 29414764Free PMC Article
Identification and characterization of a temperature-sensitive R268H mutation in the human succinyl-CoA:3-ketoacid CoA transferase (SCOT) gene.
Fukao T, Kursula P, Owen EP, Kondo N
Mol Genet Metab 2007 Nov;92(3):216-21. Epub 2007 Aug 13 doi: 10.1016/j.ymgme.2007.07.005. PMID: 17706444
Practical assay method of cytosolic acetoacetyl-CoA thiolase by rapid release of cytosolic enzymes from cultured lymphocytes using digitonin.
Watanabe H, Yamaguchi S, Kimura M, Wakazono A, Song XQ, Fukao T, Orii T, Hashimoto T
Tohoku J Exp Med 1998 Jan;184(1):29-38. doi: 10.1620/tjem.184.29. PMID: 9607396

Prognosis

Low ketolytic enzyme levels in tumors predict ketogenic diet responses in cancer cell lines in vitro and in vivo.
Zhang J, Jia PP, Liu QL, Cong MH, Gao Y, Shi HP, Yu WN, Miao MY
J Lipid Res 2018 Apr;59(4):625-634. Epub 2018 Feb 5 doi: 10.1194/jlr.M082040. PMID: 29414764Free PMC Article

Clinical prediction guides

Low ketolytic enzyme levels in tumors predict ketogenic diet responses in cancer cell lines in vitro and in vivo.
Zhang J, Jia PP, Liu QL, Cong MH, Gao Y, Shi HP, Yu WN, Miao MY
J Lipid Res 2018 Apr;59(4):625-634. Epub 2018 Feb 5 doi: 10.1194/jlr.M082040. PMID: 29414764Free PMC Article

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