ubiquinone and Liver-Failure--Acute

To study whether CO-Q10 can protect liver injury caused by acute on chronic liver failure (ACLF) by autophagy.. Rats were separated into three groups: control group, acute on chronic liver failure (ACLF) and intervenient group, liver tissues were observed by optical microscopy and electron microscopy. The levels of Beclin-1 expression were determined by real-time PCR. And Western Blot.. Areas of necrosis detected in intervenient group were alleviated than in ACLF significantly. Most mitochondrias had been degradated in ACLF group while alive in intervenient group. Real-time PCR and Western Blot revealed level of beclin-1 in ACLF was lower than control and intervenient group.. Intervenient group may ameliorate rat liver injury by promoting autophagy.

Topics: Animals; Apoptosis Regulatory Proteins; Autophagy; Beclin-1; Humans; Liver Failure, Acute; Male; Mitochondria, Liver; Rats; Rats, Sprague-Dawley; Ubiquinone

The effect of rooibos tea (Aspalathus linearis) on liver antioxidant status and oxidative stress was investigated in rat model of carbon tetrachloride-induced liver damage. Synthetic antioxidant N-acetyl-L-cysteine (NAC) was used for comparison. Administration of carbon tetrachloride (CCl4) for 10 weeks decreased liver concentrations of reduced and oxidized forms of coenzyme Q9 (CoQ9H2 and CoQ9), reduced -tocopherol content and simultaneously increased the formation of malondialdehyde (MDA) as indicator of lipid peroxidation. Rooibos tea and NAC administered to CCl4-damaged rats restored liver concentrations of CoQ9H2 and alpha-tocopherol and inhibited the formation of MDA, all to the values comparable with healthy animals. Rooibos tea did not counteract the decrease in CoQ9, whereas NAC was able to do it. Improved regeneration of coenzyme Q9 redox state and inhibition of oxidative stress in CCl4-damaged livers may explain the beneficial effect of antioxidant therapy. Therefore, the consumption of rooibos tea as a rich source of natural antioxidants could be recommended as a market available, safe and effective hepatoprotector in patients with liver diseases.

Topics: Acetylcysteine; Animals; Antioxidants; Aspalathus; Beverages; Carbon Tetrachloride; Liver Failure, Acute; Liver Regeneration; Male; Oxidation-Reduction; Oxidative Stress; Phytotherapy; Plant Extracts; Rats; Rats, Wistar; Treatment Outcome; Ubiquinone

CoQ transfers electrons from complexes I and II of the mitochondrial respiratory chain to complex III. There are very few reports on human CoQ deficiency. The clinical presentation is usually characterized by: epilepsy, muscle weakness, ataxia, cerebellar atrophy, migraine, myogloblinuria and developmental delay. We describe a patient who presented with neonatal liver and pancreatic insufficiency, tyrosinemia and hyperammonemia and later developed sensorineural hearing loss and Leigh syndrome. Liver biopsy revealed markedly reduced complex I+III and II+III. Addition of CoQ to the liver homogenate restored the activities, suggesting CoQ depletion. Histological staining showed prominent bridging; septal fibrosis and widening of portal spaces with prominent mixed inflammatory infiltrate, associated with interface hepatitis, bile duct proliferation with numerous bile plugs. Electron microscopy revealed a large number of mitochondria, which were altered in shape and size, widened and disordered intercristal spaces. This may be the first case of Leigh syndrome with liver and pancreas insufficiency, possibly caused by CoQ responsive oxphos deficiency.

Topics: Biopsy; Electron Transport Complex I; Electron Transport Complex II; Electron Transport Complex III; Hearing Loss, Sensorineural; Humans; Hyperammonemia; Infant; Leigh Disease; Liver; Liver Failure, Acute; Male; Metabolism, Inborn Errors; Mitochondria, Liver; Mitochondrial Diseases; Oxidative Phosphorylation; Pancreas; Ubiquinone