Pessayre Dominique, Mansouri Abdellah, Berson Alain, Fromenty Bernard
Dominique Pessayre, INSERM U773, 16, rue Henri Huchard, 75018, Paris, France.
Handb Exp Pharmacol. 2010(196):311-65. doi: 10.1007/978-3-642-00663-0_11.
Mitochondrial dysfunction is a major mechanism of liver injury. A parent drug or its reactive metabolite can trigger outer mitochondrial membrane permeabilization or rupture due to mitochondrial permeability transition. The latter can severely deplete ATP and cause liver cell necrosis, or it can instead lead to apoptosis by releasing cytochrome c, which activates caspases in the cytosol. Necrosis and apoptosis can trigger cytolytic hepatitis resulting in lethal fulminant hepatitis in some patients. Other drugs severely inhibit mitochondrial function and trigger extensive microvesicular steatosis, hypoglycaemia, coma, and death. Milder and more prolonged forms of drug-induced mitochondrial dysfunction can also cause macrovacuolar steatosis. Although this is a benign liver lesion in the short-term, it can progress to steatohepatitis and then to cirrhosis. Patient susceptibility to drug-induced mitochondrial dysfunction and liver injury can sometimes be explained by genetic or acquired variations in drug metabolism and/or elimination that increase the concentration of the toxic species (parent drug or metabolite). Susceptibility may also be increased by the presence of another condition, which also impairs mitochondrial function, such as an inborn mitochondrial cytopathy, beta-oxidation defect, certain viral infections, pregnancy, or the obesity-associated metabolic syndrome. Liver injury due to mitochondrial dysfunction can have important consequences for pharmaceutical companies. It has led to the interruption of clinical trials, the recall of several drugs after marketing, or the introduction of severe black box warnings by drug agencies. Pharmaceutical companies should systematically investigate mitochondrial effects during lead selection or preclinical safety studies.
线粒体功能障碍是肝损伤的主要机制。母体药物或其活性代谢产物可因线粒体通透性转换而引发线粒体外膜通透性增加或破裂。后者可严重消耗三磷酸腺苷(ATP)并导致肝细胞坏死,或者通过释放细胞色素c导致细胞凋亡,细胞色素c可激活细胞质中的半胱天冬酶。坏死和凋亡可引发细胞溶解性肝炎,在一些患者中导致致命的暴发性肝炎。其他药物会严重抑制线粒体功能并引发广泛的微泡性脂肪变性、低血糖、昏迷和死亡。较轻且持续时间较长的药物性线粒体功能障碍形式也可导致大泡性脂肪变性。虽然这在短期内是一种良性肝脏病变,但可进展为脂肪性肝炎,进而发展为肝硬化。患者对药物性线粒体功能障碍和肝损伤的易感性有时可通过药物代谢和/或消除方面的遗传或获得性变异来解释,这些变异会增加有毒物质(母体药物或代谢产物)的浓度。另一种损害线粒体功能的疾病的存在也可能增加易感性,如先天性线粒体细胞病、β氧化缺陷、某些病毒感染、妊娠或肥胖相关代谢综合征。线粒体功能障碍导致的肝损伤对制药公司可能产生重要影响。它已导致临床试验中断、数种药物上市后召回,或药物监管机构发布严重的黑框警告。制药公司应在先导化合物筛选或临床前安全性研究期间系统地研究线粒体效应。