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Since renal medullary cells, whose role is to concentrate urine, are constantly exposed to extremely high levels of sodium chloride and urea, the accumulation of betaine in renal medullary cells helps balance the hypertonicity in interstitial fluid (Kempson et al., 2014)

Methylcobalamin (vitamin B12) regulates body functions like cell growth, blood formation, and protein synthesis

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Given these functions, carnitine is concentrated in tissues that utilize fatty acids as fuel, like skeletal and cardiac muscles

Due to lifelong persistent chylomicronemia, patients with FCS have an increased likelihood of developing acute pancreatitis (62)

Grattagliano et al., 2019)
