In August 1979 and January 1982, two infants died of carnitine deficiency at the age of 3 months. The same family, at Seattle Children's Hospital,Ronald Scott Rare Disease Specialist.Systemic primary carnitine deficiency (SPCD)[1] is an inborn error of fatty acid transport caused by a defect in the

As an example of normal synthesis, a 70 kg (150 lb) person would produce 11-34 mg of carnitine per day. [1] Adults eating a diet mixed with red meat and other animal products consume about 60-180 mg of carnitine per day, while vegans consume about 10-12 mg per day. [3] The majority (54–86%) of carni

Free-floating fatty acids released from adipose tissue into the blood bind to a carrier protein molecule called serum albumin, which transports the fatty acids into the cytoplasm of target cells such as heart, skeletal muscle, and other tissue cells, where they for fuel. But before fatty acids can b

Carnitine is a quaternary ammonium compound involved in the metabolism of most mammals, plants and some bacteria. [1][2][3][4] To support energy metabolism, carnitine transports long-chain fatty acids to the mitochondria to be oxidized for energy production and participates in the removal of metabol

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