Vankoningsloo Sébastien, Piens Marie, Lecocq Christophe, Gilson Audrey, De Pauw Aurélia, Renard Patricia, Demazy Catherine, Houbion Andrée, Raes Martine, Arnould Thierry
Laboratory of Biochemistry and Cellular Biology, University of Namur, 5000 Namur, Belgium.
J Lipid Res. 2005 Jun;46(6):1133-49. doi: 10.1194/jlr.M400464-JLR200. Epub 2005 Mar 1.
Mitochondrial cytopathy has been associated with modifications of lipid metabolism in various situations, such as the acquisition of an abnormal adipocyte phenotype observed in multiple symmetrical lipomatosis or triglyceride (TG) accumulation in muscles associated with the myoclonic epilepsy with ragged red fibers syndrome. However, the molecular signaling leading to fat metabolism dysregulation in cells with impaired mitochondrial activity is still poorly understood. Here, we found that preadipocytes incubated with inhibitors of mitochondrial respiration such as antimycin A (AA) accumulate TG vesicles but do not acquire specific markers of adipocytes. Although the uptake of TG precursors is not stimulated in 3T3-L1 cells with impaired mitochondrial activity, we found a strong stimulation of glucose uptake in AA-treated cells mediated by calcium and phosphatidylinositol 3-kinase/Akt1/glycogen synthase kinase 3beta, a pathway known to trigger the translocation of glucose transporter 4 to the plasma membrane in response to insulin. TG accumulation in AA-treated cells is mediated by a reduced peroxisome proliferator-activated receptor gamma activity that downregulates muscle carnitine palmitoyl transferase-1 expression and fatty acid beta-oxidation, and by a direct conversion of glucose into TGs accompanied by the activation of carbohydrate-responsive element binding protein, a lipogenic transcription factor. Taken together, these results could explain how mitochondrial impairment leads to the multivesicular phenotype found in some mitochondria-originating diseases associated with a dysfunction in fat metabolism.
线粒体细胞病变在多种情况下与脂质代谢改变相关,比如在多发性对称性脂肪瘤病中观察到的异常脂肪细胞表型的获得,或与肌阵挛性癫痫伴破碎红纤维综合征相关的肌肉中甘油三酯(TG)积累。然而,导致线粒体活性受损的细胞中脂肪代谢失调的分子信号传导仍知之甚少。在此,我们发现用线粒体呼吸抑制剂如抗霉素A(AA)处理的前脂肪细胞会积累TG囊泡,但不会获得脂肪细胞的特异性标志物。尽管线粒体活性受损的3T3-L1细胞中TG前体的摄取未受到刺激,但我们发现AA处理的细胞中葡萄糖摄取受到强烈刺激,这是由钙和磷脂酰肌醇3激酶/Akt1/糖原合酶激酶3β介导的,该信号通路已知可在胰岛素作用下触发葡萄糖转运蛋白4向质膜的转位。AA处理的细胞中TG积累是由过氧化物酶体增殖物激活受体γ活性降低介导的,该活性下调肌肉肉碱棕榈酰转移酶-1的表达和脂肪酸β氧化,并且通过葡萄糖直接转化为TG并伴随碳水化合物反应元件结合蛋白(一种生脂转录因子)的激活。综上所述,这些结果可以解释线粒体损伤如何导致在一些与脂肪代谢功能障碍相关的线粒体起源疾病中发现的多囊泡表型。