Studies including the cohort of six patients with the myopathic form of CPT-2 deficiency showed that six-month-long treatment with BF (200 mg three times a day) markedly upregulated CPT-2, increased oxidation rates of the long-chain fatty acids, decreased muscle pain and increased physical activity in all BF-treated patients [112]. This evidence concerns the gene CPT2 and hyperinsulinemic hypoglycemia, familial, 4.