左卡尼汀 (Carnitor)

概览

Levocarnitine is a quaternary ammonium compound used to treat carnitine deficiency or to stimulate gastric and pancreatic secretions in hyperlipoproteinemia.

作用机制

Levocarnitine can be synthesised within the body from the amino acids lysine or methionine. Vitamin C (ascorbic acid) is essential to the synthesis of carnitine. Levocarnitine is a carrier molecule in the transport of long chain fatty acids across the inner mitochondrial membrane. It also exports acyl groups from subcellular organelles and from cells to urine before they accumulate to toxic concentrations. Only the L isomer of carnitine (sometimes called vitamin BT) affects lipid metabolism. Levocarnitine is handled by several proteins in different pathways including carnitine transporters, carnitine translocases, carnitine acetyltransferases and carnitine palmitoyltransferases.

适应症

⑴用于继发性肉碱缺乏症。主要用于慢性肾衰长期血透患者因肉碱缺乏产生的一系列并发症状，临床表现如心肌病、骨骼肌病、心律失常、高脂血症，以及低血压和透析中肌痉挛等。 ⑵用于罕见病β-酮硫解酶缺乏症（BKD）和原发性肉碱缺乏症（PCD）。 ⑶用于罕见病戊二酸Ⅰ型。 ⑷用于维持罕见病同型半胱氨酸血症引起的血液游离肉碱的正常水平。

• Carnitine Deficiency
• Congenital carnitine deficiency
• Secondary Carnitine deficiency