Böhles H, Michalk D, Brandl U, Fekl W, Börresen H C, Stehr K
J Nutr. 1984 Apr;114(4):671-6. doi: 10.1093/jn/114.4.671.
Miniature piglets underwent total parenteral nutrition (TPN) with and without L-carnitine supplementation during a 7-day period. Thereafter the tissue amino acid concentrations of liver, heart, skeletal muscle and brain were determined and compared to those of orally fed animals. The altered tissue amino acid concentrations during TPN without carnitine returned to normal when L-carnitine was supplemented. The most striking changes of tissue concentrations showed taurine in liver, muscle and brain and ethanolamine in heart and brain. In muscle the branched-chain amino acids were increased when L-carnitine was added to the TPN regime. Ethanolamine changes were discussed with respect to the position of this amino acid in the synthesis of phospholipids. The marked decrease of brain taurine concentrations after carnitine-free TPN was accompanied by reduced values for GABA. Both the substances function as inhibitory transmitters in the brain and should be considered when seizure activity in patients with systemic carnitine deficiency is discussed.
小型仔猪在7天时间内接受了有无L-肉碱补充的全胃肠外营养（TPN）。此后，测定了肝脏、心脏、骨骼肌和大脑的组织氨基酸浓度，并与经口喂养动物的浓度进行比较。在无肉碱的TPN期间改变的组织氨基酸浓度在补充L-肉碱后恢复正常。组织浓度最显著的变化表现为肝脏、肌肉和大脑中的牛磺酸以及心脏和大脑中的乙醇胺。在TPN方案中添加L-肉碱时，肌肉中的支链氨基酸增加。就该氨基酸在磷脂合成中的位置对乙醇胺的变化进行了讨论。无肉碱TPN后脑牛磺酸浓度的显著降低伴随着GABA值的降低。这两种物质在大脑中均作为抑制性神经递质，在讨论全身性肉碱缺乏患者的癫痫活动时应予以考虑。
