由于肉碱转运蛋白 OCTN2 缺陷和胎盘肉碱缺乏导致的小鼠胚胎致死。
Embryonic lethality in mice due to carnitine transporter OCTN2 defect and placental carnitine deficiency.
机构信息
Department of Pediatrics, Division of Neonatology, MetroHealth Medical Center, Case Western Reserve University, Cleveland, OH 44109, USA.
Department of Mathematics, Sciences and Technology, Paine College, Augusta, GA 30901, USA.
出版信息
Placenta. 2018 Sep;69:71-73. doi: 10.1016/j.placenta.2018.06.312. Epub 2018 Jun 30.
l-Carnitine plays a crucial role in uptake and subsequent β-oxidation of long-chain fatty acids in the mitochondria. Placental trophoblast cells oxidize long-chain fatty acids for energy production. Here we present data showing that l-carnitine deficiency due to a defect in the carnitine transporter OCTN2 (SLC22A5) in a mouse model leads to embryonic lethality. Placental levels of l-carnitine are reduced to <10% of normal and deficiency of l-carnitine is associated with markedly reduced expression of several growth factors and transforming growth factor β (TGF-β) genes. This report links for the first time reduced l-carnitine levels in the placenta to embryonic lethality.
左旋肉碱在将长链脂肪酸摄入线粒体和随后的β氧化中起着关键作用。胎盘滋养层细胞氧化长链脂肪酸以产生能量。在这里，我们提供的数据表明，由于肉碱转运蛋白 OCTN2（SLC22A5）缺陷导致的 l-肉碱缺乏在小鼠模型中导致胚胎致死。胎盘 l-肉碱水平降低至正常水平的 <10%，并且 l-肉碱缺乏与几种生长因子和转化生长因子β（TGF-β）基因的表达明显减少有关。本报告首次将胎盘 l-肉碱水平降低与胚胎致死联系起来。
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