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This reaction requires -ketoglutarate as a necessary cofactor

Carnitine palmitoyl transferase 1 (CPT-1) deficiency: is a fatty acid oxidation defect that results in accumulation of toxic long chain substrates and impedes mitochondrial -oxidation

Diabetes Res 27: 1525, 1994 [PubMed] [Google Scholar] 38.Celi FS, Zemskova M, Linderman JD, Smith S, Drinkard B, Sachdev V, Skarulis MC, Kozlosky M, Csako G, Costello R, Pucino F

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