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Detailed stages of the cycle: CPT-1 (outer mitochondrial membrane): Acyl-CoA + L-carnitine Acyl-carnitine + CoA-SH Rate limiting enzyme Inhibited by malonyl-CoA Isoforms: CPT-1A (liver), CPT-1B (muscle), CPT-1C (brain) CACT (translocase): Transport of acylcarnitine into the matrix Antiport with free carnitine The only transporter for acylcarnitines CPT-2 (inner mitochondrial membrane): Acyl-carnitine + CoA-SH Acyl-CoA + L-carnitine Regeneration of acyl-CoA in the matrix Not regulated by malonyl-CoA -oxidation (matrix): Cyclic shortening of the acyl chain Products: acetyl-CoA, FADH2, NADH CPT-1 regulation Research on Cell Cultures L-carnitine tested on various cell types: Cardiomyocytes: main source of energy from -oxidation Myotubes (C2C12): skeletal muscle metabolism Hepatocytes: ketogenesis and gluconeogenesis Adipocytes: lipolysis and lipid metabolism Cancer cells: Warburg metabolism vs

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In their review of 58 cases, Cook and colleagues 2 found 62% had extensive facial freckling